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Acetylcholine receptor antibodies in myasthenia gravis are associated with greater risk of diabetes and thyroid disease.
Toth C, McDonald D, Oger J, Brownell K.
Department of Clinical Neurosciences, University of Calgary and the Calgary Health Region, Calgary, Alberta, Canada.
Myasthenia
gravis (MG) may be associated with the presence of acetylcholine
receptor antibodies (AChRAb) [seropositive MG (SPMG)] or their absence
[seronegative MG (SNMG)]. Along with features of MG, the presence of
the AChRAb may relate to the existence of other immune-mediated
diseases. We sought to determine the association of SPMG with other
potential autoimmune diseases. A retrospective evaluation of
prospectively identified MG patients at a tertiary care center was
performed, with patients separated into SPMG and SNMG. Prevalence of
other immune-mediated disorders, as well as the epidemiology,
sensitivity of diagnostic testing, and thymic pathology, was contrasted
between both patient groups. Of the 109 MG patients identified, 66%
were SPMG. SPMG was associated with a greater likelihood of significant
repetitive stimulation decrement, the presence of either thymoma or
thymic hyperplasia, and the presence of thyroid disease. In addition,
all patients with a diagnosis of diabetes, concurrent with MG, were
found to be SPMG. AChRAb and SPMG impart not only a distinctive
clinical and electrophysiological phenotype of MG, but are also
associated with the heightened presence of endocrinological disease.
PMID: 16867036 [PubMed - in process]
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Myasthenia gravis in children: a longitudinal study.
Ashraf VV, Taly AB, Veerendrakumar M, Rao S.
Department of Neurology, Malabar Institute of Medical Sciences, Calicut, Kerala, India.
Juvenile
myasthenia gravis (JMG) is an uncommon disease. Unlike adults, clinical
characteristics and outcomes of myasthenia gravis (MG) are not well
studied in children. Case records of 77 patients with MG who were 15
years of age or less at disease onset, evaluated over a period of 34
years at the National Institute of Mental Health and Neurosciences,
Bangalore, India, were reviewed. Their clinical characteristics and
response to therapy was compared with 290 patients with MG onset after
15 years of age. Median age at onset was 8 years and mean period of
follow-up was 6.2 years (range 6 months to 25 years). At presentation,
30% of patients had ocular myasthenia and the rest had generalized
disease. Twenty-one patients (27%) had disease confined to ocular
muscles throughout the course and three had limb girdle myasthenia.
Familial myasthenia was more common than adult onset disease, 10
patients had positive family history. Unlike adults, none of the
patients had associated autoimmune disease. Fifty-two patients (67%)
received corticosteroids, and azathioprine was added in five patients.
Thymectomy was performed in 11 patients, six below the age of 15 years.
Thymic histology was normal in one and showed hyperplasia in eight and
thymoma in one. Four patients had crisis. At the end of follow-up, 25
patients were asymptomatic, 28 had partial improvement, and nine
remained unchanged or worsened and two died. Ten patients achieved
complete stable remission. This study shows some distinctive
characteristics of JMG, such as higher frequency of ocular myasthenia,
benign course, better long-term outcome and lack of association of
thymoma and other autoimmune disorders.
PMID: 16867035 [PubMed - in process]
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Carcinoma
Showing Thymus-like Differentiation of the Thyroid (CASTLE): A
Comparative Study: Evidence of Thymic Differentiation and Solid Cell
Nest Origin.
Reimann JD, Dorfman DM, Nose V.
Brigham and Women's Hospital and Harvard Medical School, Boston, MA.
Carcinoma
showing thymus-like differentiation (CASTLE) is a rare intrathyroidal
neoplasm, a member of a tumor family probably arising from ectopic
thymus or branchial pouch remnants. Thyroid solid cell nests (SCNs) may
also be derived from branchial pouch remnants. SCNs express p63,
carcinoembryonic antigen (CEA), and high molecular weight keratin
(HMWK). To determine whether CASTLE and SCNs derive from similar
embryologic origins/lines of differentiation, and to better
differentiate CASTLE from other thyroid neoplasms, we compared p63,
CD5, HMWK, and CEA staining of CASTLE and SCNs with other thyroid and
thymic lesions. Seven CASTLE, 11 SCNs, 10 thymic carcinoma, 11 invasive
thymoma, 12 thymoma, 28 papillary thyroid carcinoma, 4 thyroid squamous
cell carcinoma, 2 childhood sclerosing carcinoma, 4 follicular adenoma,
6 follicular carcinoma, 4 poorly differentiated carcinoma, and 20
lymphocytic thyroiditis cases were analyzed. In normal thyroid, only
SCNs stained for p63, HMWK, and CEA. The only CD5-positive cells in
normal thyroid were T cells. Thymomas and normal thymus stained
similarly to SCNs. All CASTLE and thymic carcinomas exhibited diffuse
p63 and HMWK staining and all CASTLE cases and the majority of thymic
carcinomas were positive for CEA and CD5. In contrast, none of the
other thyroid neoplasms examined exhibited consistent staining for all
4 markers studied. These findings provide further evidence that CASTLE
is distinct from other thyroid neoplasms, is probably of thymic origin,
and may arise from branchial pouch remnants, the thyroid SCNs. Moreover
CD5, HMWK, CEA and p63 can be used to help distinguish CASTLE from
other thyroid neoplasms.
PMID: 16861971 [PubMed - as supplied by publisher]
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Recurrent infections in a patient with thymoma: Good's syndrome.
Perciaccante A, Anzivino C, Fiorentini A.
Department
of Clinical Medicine, Institute of III Clinical Medicine, University
"La Sapienza", Viale dell'Universita, 37, 00185 Rome, Italy.
Publication Types:
PMID: 16864022 [PubMed - in process]
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Primary Solitary Mediastinal Mass Lesions: A Review of 37 Cases.
Alizzi AM, Hemli JM, Diqer AM, Bidstrup B.
Department of Cardiothoracic Surgery, The Townsville Hospital, Townsville, Queensland, Australia.
BACKGROUND:
Primary solitary mass lesions of the mediastinum, although relatively
uncommon, encompass an interesting spectrum of pathologies. METHODS: A
comprehensive retrospective review was undertaken of all cases of
mediastinal lesions that presented to the two major thoracic surgical
centres in North Queensland, Australia, over a 7-year period. RESULTS:
Thirty-seven mediastinal mass lesions were managed over the period of
the review. Over one-quarter of all cases were clinically silent, the
pathology having been discovered incidentally during investigation for
other reasons. Malignant thymoma was the single most common pathology,
being present in 13 (35.1%) cases. A variety of other pathologies were
encountered, including thymic cyst, bronchogenic cyst, neurofibroma,
parathyroid adenoma, and lymphoma. Expeditious surgical resection of
the lesions, once discovered, afforded good medium-term survival, even
for those patients with malignant pathology. CONCLUSIONS: Prompt
thoracic surgical referral with view to aggressive, early resection
optimizes clinical outcome in the short and medium-term for patients
presenting with mass lesions of the mediastinum.
PMID: 16860602 [PubMed - as supplied by publisher]
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Thymoma
followed by paroxysmal nocturnal hemoglobinuria: A unique clinical
association in the context of multiorgan autoimmunity with a potential
role for CD8+ T lymphocytes.
Palmieri G, Selleri C, Montella L, Bulgarelli G, Vitiello L, Merkabaoui G, Ricci P, Del Vecchio L, Masci A, Racioppi L, Rotoli B.
Department of Molecular and Clinical Endocrinology & Oncology, "Federico II" University, Naples, Italy.
Seven
years after a surgically resected thymoma, a female patient affected by
myasthenia gravis and Good's syndrome presented with paroxysmal
nocturnal hemoglobinuria (PNH). Co-culture experiments and
spectratyping analyses indicated that CD8+ lymphocytes were involved in
damaging hematopoietic precursors. While PNH clones have been
identified in various hematological disorders, the sequential
association of thymoma and PNH has been unreported so far. Am. J.
Hematol., 2006. (c) 2006 Wiley-Liss, Inc.
PMID: 16838334 [PubMed - as supplied by publisher]
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COX-2 upregulation in thymomas and thymic carcinomas.
Rieker RJ, Joos S, Mechtersheimer G, Blaeker H, Schnabel PA, Morresi-Hauf A, Hecker E, Thomas M, Dienemann H, Schirmacher P, Kern MA.
Department of General Pathology, University Hospital, Heidelberg, Germany.
The
treatment of advanced stage thymomas and thymic carcinomas is a
multimodal therapy. New therapeutic targets are currently under
investigation, including the epidermal growth factor receptor (EGFR) as
well as KIT. A number of studies have shown protumorigenic potential of
Cyclooxygenase-2 (COX-2) in a variety of human malignancies, but so far
it is unknown whether COX-2 is expressed in primary malignancies of the
thymus. Using tissue microarrays, the expression of COX-2,
microsomal-PGES-1 and -PGES-2 (mPGES-1 and mPGES-2), as well as EGFR
was evaluated in different subtypes of thymoma and thymic carcinomas.
COX-2 was expressed in all subtypes as determined by
immunohistochemistry. Some cases of type B2 and thymic carcinomas had
COX-2 staining levels classified as mild to moderate. However, when
measuring the optical color intensity, no significant differences could
be detected. Concerning the expression levels, a weak correlation
between the expression of COX-2, mPGES-1 and mPGES-2 as well as EGFR
was found. Furthermore, additional cases of thymomas and thymic
carcinomas were analyzed by COX-2 Western immunoblot analysis and were
compared to normal thymi. The analysis showed that thymomas and thymic
carcinomas had a significantly stronger COX-2 expression than that of
the normal thymi (p < 0.04). In summary, COX-2 is expressed in all
subtypes of thymomas and thymic carcinomas and thus represents, in
addition to EGFR and KIT, a potential therapeutic target. Further
studies are needed in order to determine whether a combined therapy
using COX-2 inhibitors in addition to the evolving anti-EGFR antibody
therapy may be considered as a treatment option. (c) 2006 Wiley-Liss,
Inc.
PMID: 16823844 [PubMed - as supplied by publisher]
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Management of superior vena cava syndrome by internal jugular to femoral vein bypass.
Dhaliwal RS, Das D, Luthra S, Singh J, Mehta S, Singh H.
Department
of Cardiovascular and Thoracic Surgery, Postgraduate Institute of
Medical Education and Research, Chandigarh, PIN, India.
We
report a 30-year-old man with superior vena cava syndrome due to
fibrosis from a previously irradiated malignant thymoma. The patient
presented 4 years after the initial treatment, after having been lost
to follow-up. Investigations revealed total obstruction of the superior
vena cava, and right subclavian and right internal jugular vein. The
patient underwent an extra-anatomic bypass (ringed
polytetrafluoroethylene graft 10-mm diameter) between the left internal
jugular vein and the left femoral vein brought in a subcutaneous tunnel
over the anterior chest and abdominal wall. Entry to the thoracic
cavity was avoided due to extensive fibrotic changes visualized in the
computed tomographic chest scan. Follow-up Doppler at 2 months, 6
months, 1 year, and 3 years showed a patent graft. An internal jugular
vein to the femoral vein bypass is a simple method for palliation of
superior vena cava syndrome.
PMID: 16798237 [PubMed - in process]
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Tumor growth impedes natural-killer-cell maturation in the bone marrow.
Richards JO, Chang X, Blaser BW, Caligiuri MA, Zheng P, Liu Y.
Division
of Cancer Immunology, Department of Pathology, The Ohio State
University Medical Center and Comprehensive Cancer Center, Columbus, OH
43210, USA.
Natural-killer (NK)-cell dysfunction and IFN-gamma
deficiencies have been associated with increased incidence of both
malignancy and infection. The immunologic basis of NK-cell defects in
cancer-bearing hosts has not been extensively studied. Here, we
demonstrate that multiple lineages of tumors, including thymoma, breast
cancer, colon cancer, and melanoma cell lines, interrupt functional
maturation during NK-cell development in the bone marrow. The immature
NK cells in the periphery of tumor-bearing mice had impaired IFN-gamma
production but seemingly normal cytotoxicity. T cells are not involved
in this NK maturation arrest, because T-cell depletion did not restore
NK-cell development. Moreover, the extent of tumor-cell infiltration
into the bone marrow does not correlate with defective NK maturation.
Interestingly, the defect was associated with a significant reduction
in the IL-15Ralpha+ cells in the non-T, non-NK compartment of bone
marrow cells and restored by overexpression of IL-15. Our data
demonstrate that tumor growth can impede functional maturation of NK
cells, most likely by interrupting the requisite IL-15 signaling
pathway.
PMID: 16556890 [PubMed - in process]
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Serial right ventricular endomyocardial biopsy in rapid-onset severe heart failure due to giant cell myocarditis.
van Haelst PL, Brugemann J, Diercks GF, Suurmeijer A, van Veldhuisen DJ.
Department
of Cardiology, University Medical Center Groningen, NL-9700RB
Groningen, The Netherlands; Department of Pathology, University Medical
Center Groningen, NL-9700RB Groningen, The Netherlands.
Giant
cell myocarditis (GCM) is a serious condition that warrants immediate
diagnosis and treatment. It often presents as rapidly progressive heart
failure and/or malignant ventricular arrhythmias. Here, we describe a
34-year-old patient with myasthenia gravis who presented with GCM 2
weeks after resection of a thymoma. A cardiac biopsy confirming the
diagnosis was done within 3 days after admission. After institution of
an aggressive immunosuppressive drug regimen, implantation of an
implantable cardioverter defibrillator, and intensive cardiac
rehabilitation, the patient recovered dramatically. In control biopsies
after 4 weeks and 6 months, no more giant cells were found. We conclude
that, in the case of nonischemic acute heart failure in young patients,
a biopsy should be performed as soon as possible to prevent an
unfavourable outcome of this often fatal disease.
PMID: 16844555 [PubMed - in process]
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Guidelines for the treatment of autoimmune neuromuscular transmission disorders.
Skeie GO, Apostolski S, Evoli A, Gilhus NE, Hart IK, Harms L, Hilton-Jones D, Melms A, Verschuuren J, Horge HW.
Department of Neurology, University of Bergen, Bergen, Norway. geir.olve.skeie@helse-bergen.no
Important
progress has been made in our understanding of the cellular and
molecular processes underlying the autoimmune neuromuscular
transmission (NMT) disorders; myasthenia gravis (MG), Lambert-Eaton
myasthenic syndrome (LEMS) and neuromyotonia (peripheral nerve
hyperexcitability; Isaacs syndrome). To prepare consensus guidelines
for the treatment of the autoimmune NMT disorders. References retrieved
from MEDLINE, EMBASE and the Cochrane Library were considered and
statements prepared and agreed on by disease experts and a patient
representative. The proposed practical treatment guidelines are agreed
upon by the Task Force: (i) Anticholinesterase drugs should be the
first drug to be given in the management of MG (good practice point).
(ii) Plasma exchange is recommended as a short-term treatment in MG,
especially in severe cases to induce remission and in preparation for
surgery (level B recommendation). (iii) Intravenous immunoglobulin
(IvIg) and plasma exchange are equally effective for the treatment of
MG exacerbations (level A Recommendation). (iv) For patients with
non-thymomatous autoimmune MG, thymectomy (TE) is recommended as an
option to increase the probability of remission or improvement (level B
recommendation). (v) Once thymoma is diagnosed TE is indicated
irrespective of the severity of MG (level A recommendation). (vi) Oral
corticosteroids is a first choice drug when immunosuppressive drugs are
necessary in MG (good practice point). (vii) In patients where
long-term immunosuppression is necessary, azathioprine is recommended
together with steroids to allow tapering the steroids to the lowest
possible dose whilst maintaining azathioprine (level A recommendation).
(viii) 3,4-diaminopyridine is recommended as symptomatic treatment and
IvIg has a positive short-term effect in LEMS (good practice point).
(ix) All neuromyotonia patients should be treated symptomatically with
an anti-epileptic drug that reduces peripheral nerve hyperexcitability
(good practice point). (x) Definitive management of paraneoplastic
neuromyotonia and LEMS is treatment of the underlying tumour (good
practice point). (xi) For immunosuppressive treatment of LEMS and NMT
it is reasonable to adopt treatment procedures by analogy with MG (good
practice point).
PMID: 16834699 [PubMed - in process]
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Neuromyotonia and myasthenia gravis in the absence of thymoma.
Antelli A, Spengos K, Zambelis T, Pandis D, Tsivgoulis G, Zis V.
Publication Types:
PMID: 16834693 [PubMed - in process]
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Mediastinal large B-cell lymphoma with rosette formation mimicking thymoma and thymic carcinoid.
Tsai HW, Yen YS, Chang KC.
Department of Pathology, National Cheng Kung University Hospital, Tainan, Taiwan.
PMID: 16842255 [PubMed - in process]
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Pure
red cell aplasia developing after treatment of pleural recurrence of
thymoma, successfully treated with cyclosporin A but not with
tacrolimus.
Fukushima K, Sato T, Mitsuhashi S, Gono T, Kaneko K, Yazaki M, Mastuda M, Ikeda S.
Department of Internal Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto.
PMID: 16679708 [PubMed - in process]
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Thymectomy for paraneoplastic stiff-person syndrome associated with invasive thymoma.
Iwata T, Inoue K, Mizuguchi S, Morita R, Tsukioka T, Suehiro S.
Department of Thoracic Surgery, Osaka City University Hospital, Osaka, Japan. taiwata@med.osaka-cu.ac.jp
Publication Types:
PMID: 16798340 [PubMed - indexed for MEDLINE]
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[Primary thymic lymphomas.]
[Article in Spanish]
Rios A, Torres J, Roca MJ, Galindo PJ, Alonso JL, Parrilla P.
Departamento de Cirugia. Servicio de Cirugia Toracica. Hospital Universitario Virgen de la Arrixaca. El Palmar. Murcia. Espana.
INTRODUCTION.
Primary thymic lymphomas (PTLs) are uncommon, and their prognosis is
linked with early treatment. A review is carried out of this disease in
our hospital in order to determine the best diagnostic-therapeutic
management for these patients. MATERIAL AND METHODS. Ten LPTs - four
Hodgkin's and six non-Hodgkin's (4 primary mediastinal B lymphomas
[PMBLs] and 2 lymphoblastic T lymphomas [LTLs] - were reviewed. Most of
the patients were females, with a mean age of 23 +/- 10 years. RESULTS.
The initial diagnostic suspicion in the Hodgkin's lymphomas was thymoma
in two cases and lymphoma in the other 2. All of them underwent
surgery, including an intra-operative biopsy, which was completed with
a thymectomy in the two in which thymoma was reported. They were
treated with radio and chemotherapy. The response was partial in two
cases, and treatment was completed with a bone marrow transplant (BMT)
(one died and the other had active disease). The non-Hodgkin's
lymphomas had large tumors and short evolution. All of them received
surgery, with an intra-operative biopsy in four and a thymectomy in
two. They were treated with chemotherapy, with associated radiotherapy
in two. The response was total in three, with two recurring, who are in
complete remission after a BMT. In the other three the response was
partial. CONCLUSIONS. In a patient with thymic tumour with a
preoperative or intraoperative study suspected of having a lymphoma, it
is necessary to do a biopsy and not resective surgery, to avoid
unnecessary resections and morbidity. PTLs are uncommon but aggressive,
principally the non-Hodgkin's lymphomas. The main treatment is radio
and chemotherapy, with associated bone marrow transplantation in
selected cases.
PMID: 16831379 [PubMed - in process]
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Aspergillus fumigatus extract differentially regulates antigen-specific CD4+ and CD8+ T cell responses to promote host immunity.
Tao J, Segal BH, Eppolito C, Li Q, Dennis CG, Youn R, Shrikant PA.
Departments of *Immunology and Medicine, Roswell Park Cancer Institute, Buffalo, New York.
Invasive
aspergillosis is a major cause of morbidity and mortality in the
severely immunocompromised. The paucity of information about the
mechanisms by which Aspergillus-derived factors regulate
antigen-specific T cell responses in vivo poses a significant hurdle
for devising effective immunization strategies to treat or prevent
aspergillosis. By monitoring adoptively transferred T cell receptor
transgenic, naive CD4+ (OT-II) and CD8+ (OT-I) T cells specific for
distinct peptides of a nominal antigen, chicken ovalbumin (OVA), we
demonstrate that sensitization with Aspergillus fumigatus (Af) extract
plus OVA protein considerably enhances OT-I and OT-II T cell
activation, which results in clonal expansion, primarily as a result of
increased proliferation. The sensitization provided by Af extract
promotes OT-I expansion accompanied by differentiation into
interferon-gamma-producing cytotoxic cells. It is surprising that no
effector differentiation of the induced OT-II response was observed.
Moreover, the Af extract-induced OT-I and OT-II T cell expansion was
transient, as considerable contraction in the numbers of detectable
OT-I and OT-II T cells was evidenced by Day 10. In agreement with these
observations, sensitization with Af extract plus OVA marginally
promoted host immunity against an OVA-expressing thymoma (E.G7)
challenge, and the protection was enhanced by resensitization with Af
extract and OVA. Our results demonstrate the ability of Af extract to
differentially regulate antigen-specific CD4+ and CD8+ T cell
responses, resulting in limited augmentation of host immunity. This
information suggests that strategies to target CD4+ T cell effector
maturation may promote host immunity to Aspergillus and unexpectedly
demonstrates the use for Af extract as a CD8+ T cell adjuvant.
PMID: 16793916 [PubMed - as supplied by publisher]
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Thymoma associated with keratoconjunctivitis, lichen planus, hypogammaglobinemia, and absent circulating B cells.
Hon C, Chui WH, Cheng LC, Shek TW, Jones BM, Au WY.
Department of Ophthalmology, Prince of Wales Hospital, Queen Mary Hospital, Shatin, China.
Publication Types:
PMID: 16782934 [PubMed - indexed for MEDLINE]
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CD95 ligand mediates T-cell receptor-induced apoptosis of a CD4(+) CD8(+) double positive thymic lymphoma.
Schmitz I, Meyer C, Schulze-Osthoff K.
1Institute of Molecular Medicine, University of Dusseldorf, Dusseldorf, Germany.
Tumors
in the thymus can be of different cellular origin. Among the most
common tumors are thymoma and lymphoma, which are derived from
transformed thymic epithelial cells and transformed lymphocytes,
respectively. Thymic lymphoma and their response to apoptotic stimuli
are poorly characterized. Here, we analyse apoptosis events in the
thymic lymphoma cell line Thy278, which expresses cell surface antigens
characteristic of immature double positive thymocytes. Upon T-cell
receptor (TCR)/CD3 stimulation, Thy278 cells die by apoptosis, similar
as primary thymocytes during negative selection. Caspases are crucial
for deletion of both Thy278 cells and normal thymocytes. Moreover, we
show that deletion of primary thymocytes and Thy278 cells upon CD3
stimulation is considerably impaired by neutralizing CD95L antibody.
Thus, our results not only demonstrate that TCR-induced apoptosis is
still functional in transformed thymocytes, but also suggest that
Thy278 cells are a helpful model for the molecular analysis of negative
selection.Oncogene (2006) advance online publication, 12 June 2006;
doi:10.1038/sj.onc.1209741.
PMID: 16767155 [PubMed - as supplied by publisher]
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Anesthetic management of a patient with stiff-person syndrome and thymoma: a case report.
Qin X, Wang DX, Wu XM.
Department of Anesthesiology, Peking University First Hospital, Beijing 100034, China.
Publication Types:
PMID: 16780779 [PubMed - indexed for MEDLINE]
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Clinical outcomes following extended thymectomy for myasthenia gravis: report of 17 cases.
Hase R, Sugiura H, Fukunaga A, Takahashi H, Simozawa E, Kondo S.
Department of Surgery, Hakodate Medical Association Hospital, Japan.
PURPOSE:
This retrospective study was undertaken to assess the changes in the
clinical status of patients with generalized myasthenia gravis (MG)
treated with extended thymectomy and to identify prognostic variables
that may be of significance in optimizing patient selection. PATIENTS
AND METHODS: We reviewed the clinical outcomes of 17 patients who
underwent extended thymectomy for MG. Main factors influencing the
outcome are changes in clinical stage and medication requirement before
and after thymectomy, age, sex, duration of disease, stage of disease,
antibody status, histological characteristics of the thymus, and
duration of follow-up. RESULTS: There was remission in 4 patients
(23.5%), improvement in 9 patients (53%), and no change in 4 patients
(23.5%). Patients in Osserman stage IIB and with a higher rate of
decrease in acetylcholine receptor (AchR) antibody ratio showed a
greater degree of postoperative improvement. Age of the patient, sex,
presence or absence of thymoma, and time elapsed between diagnosis and
operations were not found to be significant prognostic factors.
CONCLUSION: The present study demonstrated that extended thymectomy for
MG is an effective therapy with no great morbidity or mortality.
Patients in preoperative stage IIB and with higher rate of change in
the AchR antibody titer showed the greatest degree of postoperative
improvement.
PMID: 16823336 [PubMed - in process]
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Middle mediastinal thymoma of unusual pathologic type.
Kuzdzal J, Zielinski M, Papla B, Szlubowski A.
Department of Thoracic Surgery, Pulmonary Hospital Zakopane, Poland.
Among
the many sites of ectopic thymoma development, the middle mediastinum
is the rarest in the English language literature we found only two case
reports. We present a case of 69-year-old woman with slow-growing,
ectopic middle mediastinum thymoma of an very unusual histological
type, neither classified in the Muller-Hermelink nor the World Health
Organization (WHO) classification.
PMID: 16823335 [PubMed - in process]
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Clinical value of the WHO classification system of thymoma.
Detterbeck FC.
Division of Thoracic Surgery, Yale University, New Haven, Connecticut 06520-8062, USA. frank.detterbeck@yale.edu
Since
the World Health Organization (WHO) histologic classification system
for thymoma was introduced in 1999, several centers have published
results using this system. This review of the published experience with
the WHO system examines whether the classification is reproducible,
whether the WHO system defines clinically distinct patient groups,
assesses the independent prognostic value of the WHO type by
multivariate analysis, and discusses the impact of the WHO system on
clinical management decisions.
PMID: 16731193 [PubMed - in process]
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Veterinary diagnostic imaging.Thymoma.
Hylands R.
North Town Veterinary Hospital, 496 Main Street North, Brampton, Ontario L6V 1P9.
PMID: 16808236 [PubMed - in process]
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Metastatic basal cell carcinoma: complete response to chemotherapy and associated pure red cell aplasia.
Carneiro BA, Watkin WG, Mehta UK, Brockstein BE.
Department of Medicine, Evanston Northwestern Healthcare, Illinois, USA.
Basal
cell carcinoma (BCC) is usually a benign and indolent cancer cured in
greater than 95 percent of cases. Nevertheless, it can be locally
destructive or occasionally metastasize to distant organs. We report a
case of BCC metastatic to the lungs, occurring 17 years after the
primary BCC was noticed, that responded to carboplatin and paclitaxel
on 3 occasions. The patient also developed pure red cell aplasia
(PRCA). Work-up did not reveal underlying thymoma or infectious,
rheumatologic, or lymphoproliferative disorders. Parvovirus serologies
were negative, and antibodies against erythropoetin were not detected.
There was no history of exposure to drugs associated with PRCA. Bone
marrow biopsy on 2 different occasions did not show evidence of
myelodysplasia. PRCA may represent an unusual paraneoplastic syndrome
associated with BCC as reported with other carcinomas. This is the
first report of PRCA associated with metastatic BCC or the drugs
carboplatin and paclitaxel, which were used to treat it. The literature
on chemotherapy for metastatic BCC is reviewed.
PMID: 16777692 [PubMed - in process]
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Does perioperative high-dose prednisolone have clinical benefits for generalized myasthenia gravis?
Sekine Y, Kawaguchi N, Hamada C, Sekiguchi H, Yasufuku K, Iyoda A, Shibuya K, Fujisawa T.
Department
of Thoracic Surgery, Graduate School of Medicine, Chiba University,
1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan. sekine@faculty.chiba-u.jp
OBJECTIVE:
The purpose of this study was to clarify the clinical benefits of
perioperative administration of high-dose prednisolone (PSL) combined
with extended thymectomy on the long-term outcomes of 116 consecutive
patients with generalized myasthenia gravis (MG). METHODS: A
retrospective review was conducted on 116 patients diagnosed with
generalized MG who received alternate-day oral administration of
high-dose PSL (100 mg/alternate days) and had undergone transsternal
extended thymectomy. Incidences of postoperative myasthenic crisis,
adverse effects of steroid, long-term outcomes, such as complete stable
remission (CSR), pharmacologic remission (PR) or improvement (Imp), and
disease recurrence after CSR were evaluated. RESULTS: Six patients
(5.2%) experienced post-thymectomy myasthenic crisis. Crude cumulative
CSR and PR + CSR rates were 44.8 and 62.7%, respectively. Life table
analysis showed that 41.8, 52.8 and 63.4% of the patients were in CSR
at 3, 5 and 10 years, respectively. Multivariate analysis revealed that
age and pretreatment classification according to the Myasthenia Gravis
Foundation of America (MGFA) criteria tended to be independent
predictors of CSR. There were 6.9% with compressive vertebral fracture,
13.8% with cataract, and 5.2% with steroid-induced diabetes. Life table
analysis revealed that recurrence rates after CSR were 36.8 and 46.0%
at 3 and 5 years, respectively. Patients with thymoma had a
significantly higher rate of recurrence than those without thymoma (p =
0.001). CONCLUSIONS: Alternate-day administration of high-dose
prednisolone reduced the risk of post-thymectomy myasthenic crisis.
Presence of thymoma was a risk factor for MG recurrence after CSR.
PMID: 16675232 [PubMed - in process]
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A case of non-Hodgkin's lymphoma and invasive thymoma treated with R-CHOP therapy.
Yamato H, Tsutsumi Y, Ehira N, Kanamori H, Shimoyama N, Tanaka J, Imamura M, Asaka M, Hasegawa T, Masauzi N.
Publication Types:
PMID: 16787882 [PubMed - in process]
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Elevation of IL-12 p40 and its antibody in myasthenia gravis with thymoma.
Yoshikawa H, Sato K, Edahiro S, Furukawa Y, Maruta T, Iwasa K, Watanabe H, Takaoka S, Suzuki Y, Takamori M, Yamada M.
Department
of Neurology and Neurobiology of Aging, Graduate School of Medicine
Kanazawa University, Japan. @kenroku.kanazawa-u.ac.jp
We
examined the serum levels of cytokines, interferon (IFN)-alpha,
IFN-gamma, interleukin (IL)-4, IL-12 p40, and IL-12 p70; those that
affect the T helper 1 and 2 balance in patients with myasthenia gravis
(MG). Among the cytokines tested, only IL-12 p40, together with the
serum titer of anti-IL-12 p40 antibody, was significantly elevated in
MG with thymoma. Their elevation was independent of the histopathology
of thymoma. Thymectomy decreased the levels of IL-12 p40 accompanied by
the anti-acetylcholine receptor antibody, but not anti-IL-12 p40
antibodies. These data strongly suggest the association of IL-12 p40
and its autoantibody with the immunopathology of MG with thymoma.
PMID: 16574246 [PubMed - indexed for MEDLINE]
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Expression and mutation statuses of epidermal growth factor receptor in thymic epithelial tumors.
Suzuki E, Sasaki H, Kawano O, Endo K, Haneda H, Yukiue H, Kobayashi Y, Yano M, Fujii Y.
Department
of Surgery II, Nagoya City University Medical School, Kawasumi 1,
Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan.
hisasaki@med.nagoya-cu.ac.jp.
BACKGROUND: Epidermal growth
factor receptor (EGFR) gene mutations have been reported to correlate
with the sensitivity to the tyrosine kinase inhibitor treatment for
advanced lung cancers. Since several reports have shown that invasive
thymoma overexpress the EGFR protein, we examined the EGFR expression
and mutation statuses in thymoma and thymic carcinoma tissues. METHODS:
EGFR mutation statuses from 99 thymic epithelial tumor samples were
evaluated by a rapid and sensitive TaqMan assay using Applied
Biosysytems 7500 real-time PCR system. Probes were designed according
to the 13 different EGFR mutations reported previously in lung cancers.
A total of 38 thymoma samples were directly sequenced for the EGFR
gene. Protein expressions were evaluated for 56 thymic epithelial
tumors by immunohistochemistry. RESULTS: EGFR gene mutations were not
detected in any of the thymoma and thymic cancer samples using TaqMan
PCR assay. Of the 38 samples 3 showed a heterozygous silent mutation
without changes in the protein, a G to A transition at the nucleotide
2361 in exon 18. EGFR expression was significantly higher in invasive
thymomas (stages III-IV, 15/19 were positive) than in early stage
thymomas (stages I-II, 7/33 were positive) (P < 0.0001). All four
carcinomas and all seven B3 thymomas showed EGFR positive staining.
CONCLUSIONS: Although EGFR mutation at the tyrosine kinase domain is
unlikely to be a therapeutic target for thymoma, the information about
EGFR expression would contribute to the further identification of the
therapeutic target for advanced thymomas.
PMID: 16762968 [PubMed - in process]
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Large cell neuroendocrine thymic carcinoma coexisting within large WHO type AB thymoma.
Nagata Y, Ohno K, Utsumi T, Sasaki Y, Suzuki Y.
Department of Surgery, Osaka Kosei-Nenkin Hospital, Japan.
Large
cell neuroendocrine carcinoma (LCNEC) is a rare type of thymic
epithelial tumor. It is recognized as a different entity from other
thymic tumors on account of it having a more aggressive biologic
behavior and poor prognosis. We report an extremely rare case of a very
small, "large cell neuroendocrine thymic carcinoma" coexisting within a
large thymoma that could not be detected by usual biopsy. Surgery as
the initial treatment has the significance of definitive diagnosis and
curative treatment for LCNEC of the thymus. To make a successful
differential diagnosis, application of detailed immunohistochemical
stains may be of aid, since thymic epithelial tumor is not always
morphologically homogenous.
PMID: 16813109 [PubMed - in process]
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Differential lineage-specific regulation of murine CD45 transcription by Oct-1 and PU.1.
Kwon UK, Yen PH, Collins T, Wells RA.
Molecular
and Cellular Biology, Sunnybrook and Women's Research Institute,
Department of Medical Oncology, Toronto Sunnybrook Regional Cancer
Centre, Toronto, Ont., Canada M4N 2M5.
Although it has been
established that CD45 expression is regulated at the transcriptional
level, neither the regulatory elements that are responsible for its
unique expression pattern nor the relevance of its three distinct
transcriptional start sites (P1a, P1b, and P2) has been fully
characterized. We studied the contribution of the three start sites to
CD45 mRNA production in haematopoietic cell lines and primary
haematopoietic cells. In myeloid and lymphoid cells and cell lines most
CD45 transcripts originate from P1b with the exception of the
thymoma-derived T cell line EL4, in which approximately 90% of CD45
transcripts originate from P1a. The degree of contribution of P1a is
highest in lymphoid cells and increases in T cells following mitogen
stimulation. In vitro evaluation of sequence upstream of the start
sites shows that the P2 start site is sufficient for CD45 expression in
lymphoid but not in myeloid cells, confirms the presence of a
PU.1-binding site essential for myeloid expression of CD45, and reveals
an Octamer-binding site that interacts with both Oct-1 and Oct-2 and
activates CD45 transcription in lymphoid and myeloid cells. These
findings are the first evidence that Octamer-binding factors are
involved in the control of CD45 expression.
PMID: 16616894 [PubMed - indexed for MEDLINE]
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Safer video-assisted thoracoscopic thymectomy after location of thymic veins with multidetector computed tomography.
Shiono H, Inoue A, Tomiyama N, Shigemura N, Ideguchi K, Inoue M, Minami M, Okumura M.
Division
of General Thoracic Surgery, Department of Surgery, Osaka University
Graduate School of Medicine, E1, 2-2 Yamadaoka, Suita, Osaka, 565-0871,
Japan, hishiro@surg1.med.osaka-u.ac.jp.
BACKGROUND:
Video-assisted thoracoscopic (VATS) thymectomy has been applied as a
surgical option for autoimmune myasthenia gravis. Prior identification
and fine division of the thymic veins are critical to the prevention of
unexpected severe bleeding that may require conversion to open surgery.
Until recently, such bleeding could be avoided only by meticulous
dissection of thymic fat tissue away from the left brachiocephalic vein
(LBV). With recent advances in computed tomography (CT),
multidetector-row computed tomography (MDCT) can readily be obtained
and provides three-dimensional (3D) images. This study explored its
value for preoperative identification of the thymic veins draining into
the LBV, and thus for prevention of injury to these veins during
endoscopic thymectomy. METHODS: Five patients with myasthenia gravis,
thymoma, or both underwent enhanced MDCT preoperatively. The thymic
veins draining into the LBV were visualized using both horizontal and
sagittal/coronal CT images. Then 3D images were reconstructed to enable
operators to simulate endoscopic views. During each VATS extended
thymectomy, the numbers and branching patterns of the thymic veins were
compared with the preoperative MDCT images. RESULTS: The thymic veins
draining into the LBV were clearly identified with MDCT in all five
patients examined. Reconstructed 3D images clearly located their
courses in the thymic/fat tissue and their entry routes into the LBV,
thus simulating the actual intraoperative endoscopic views. All
tributaries divided during surgery were identified preoperatively with
MDCT. CONCLUSIONS: Location of thymic veins with MDCT can provide
precise preoperative information about thymic venous anatomy. This easy
and less invasive examination has the potential to make VATS thymectomy
easier and safer.
PMID: 16736308 [PubMed - as supplied by publisher]
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Debulking surgery for advanced thymoma.
Liu HC, Chen YJ, Tzen CY, Huang CJ, Chang CC, Huang WC.
Division
of Thoracic Surgery, Department of Surgery, Mackay Memorial Hospital,
No. 92, Sector 2, Chung-San North Road, Taipei, Taiwan; Department of
Medicine, Taipei Medical University, Taipei, Taiwan; Mackay Junior
College of Nursing, Taipei, Taiwan.
AIMS: This study was
conducted to evaluate the efficacy of debulking surgery in the
treatment of locally advanced but operable malignant thymoma. METHODS:
We reviewed 43 cases with incompletely resected stage III and IVa
malignant thymoma managed between January 1987 and December 2002.
RESULTS: Twenty-two had stage III and 21 had stage IVa disease. Maximal
debulking was performed in 15 patients, nine with stage III and six
with stage IVa disease. Nine patients also had myasthenia gravis (MG).
Using univariate Kaplan-Meier analysis, we found that maximal debulking
surgery, RT, and with the presence of MG were associated with better
survival. Debulking resulted in a better outcome than non-debulking
surgery (mean survival: 106 months vs 57.2 months). After adjustment
with multivariate analysis, RT and MG were both associated with better
survival. CT did not appear to be beneficial for advanced thymoma.
CONCLUSIONS: RT is independently associated with a better outcome in
locally advanced thymoma. Debulking surgery apparently allows for a
better response to RT.
PMID: 16697547 [PubMed - as supplied by publisher]
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My approach to the diagnosis of thymoma.
Suster S.
Ohio State University, United States.
The
diagnosis of thymic epithelial neoplasm has been a topic of controversy
for many years. Reasons for this include the lack of predictive value
associated with the morphology of these tumors and the multiplicity of
classification schemes and terminologies proposed over the years.
Recently, a new classification schema was introduced by the World
Health Organization (WHO) in an attempt to standardize nomenclature and
facilitate the diagnosis of primary thymic epithelial neoplasms. This
schema, although not originally intended as a new histologic
classification but rather as a means for translating equivalent terms
from the various existing classifications, has represented a major step
forward in the field. However, problems still exist with the WHO
schema, particularly with some of the criteria for the various
histologic subtypes as well as with issues of inter-observer
reproducibility. For this reason, we favor utilizing a much more
simplified approach to the morphologic classification of thymic
epithelial neoplasms. Herein I will detail my personal approach to the
morphologic diagnosis of thymoma, with a brief explanation for the
rationale for simplifying the existing diagnostic categories.
PMID: 16679354 [PubMed - as supplied by publisher]
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Atypical thymoma (WHO B3) with neuroendocrine differentiation: report of a case.
Shiraishi J, Nomori H, Orikasa H, Mori T, Yamazaki K.
Department
of Pathology, Japanese Red Cross Medical Center, 4-1-22 Hiroo,
Shibuya-ku, Tokyo, 150-8935, Japan, jshira@chive.ocn.ne.jp.
We
report a case of type-B3 thymoma manifesting neuroendocrine
differentiation. The patient was a 42-year-old woman who complained of
shoulder pain but had no symptoms of myasthenia gravis or anemia. The
tumor was located in the anterior mediastinum and had directly invaded
the pericardium and left lung. Histological examination revealed that
the tumor was lobulated by bands of fibrous tissue, perivascular spaces
were scattered throughout the tumor, and there were a few
intraepithelial lymphocytes. The vast majority of lymphocytes in the
perivascular spaces and in the lobulated tumor were
immunohistochemically positive for TdT, MIC2, and CD1a. The majority of
tumor cells were polygonal and medium or large in size. The tumor cells
were weakly positive for synaptophysin, chromogranin A, CD56, and NSE.
Small nests of small, relatively uniform polygonal cells were observed
facing the fibrous bands. These cells resembled the cells of carcinoid
tumors and were strongly positive for NSE, synaptophysin, chromogranin
A, and CD56. Ultrastructurally, sparse dense-core granules were
observed in the cytoplasm of a few tumor cells. This is a unique case
of thymoma with neuroendocrine differentiation, and to the best of our
knowledge this is the first such case ever reported.
PMID: 16673117 [PubMed - as supplied by publisher]
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Anterior mediastinal mass in a patient with Graves' disease.
Yamanaka K, Nakayama H, Watanabe K, Kameda Y.
Division of Thoracic Surgery, Kanagawa Cancer Center, Yokohama, Japan. kaz-genesis@mvb.biglobe.ne.jp
Thymic
hyperplasia is a common feature in Graves' disease. However, in most
cases, thymic enlargement is minimal, and radiologically detectable
massive enlargement of the thymus is infrequently reported. Half of
them undergo thymectomy due to the concern about a thymoma. We report a
28-year-old woman with untreated Graves' disease. She had an anterior
mediastinal mass that was diagnosed as true thymic hyperplasia by
biopsy and disappeared after treatment of the hyperthyroid state.
Recognizing the association between thymic hyperplasia and
Graves'disease, and existence of the benign course after treatment of
the hyperthyroidism may be useful for avoiding unnecessary surgical
procedure.
Publication Types:
PMID: 16631707 [PubMed - indexed for MEDLINE]
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Preoperative steroid pulse therapy for invasive thymoma: clinical experience and mechanism of action.
Kobayashi Y, Fujii Y, Yano M, Sasaki H, Yukiue H, Haneda H, Suzuki E, Endo K, Kawano O.
Department of Surgery II, Nagoya City University Medical School, Nagoya, Japan. shogen@med.nagoya-cu.ac.jp
BACKGROUND:
Glucocorticoid was used in thymomas. The purpose of the study was to
evaluate the efficacy of intravenous high-dose glucocorticoid (steroid
pulse) therapy in patients with previously untreated advanced thymoma.
Causes were also sought for a possible underlying mechanism of the
effect of steroid on thymoma. METHODS: Seventeen patients with invasive
thymoma who had not received previous chemotherapy or radiation therapy
were enrolled in the study. All cases were treated with 2 courses of
glucocorticoid therapy before surgery. Tumor response was assessed by
computed tomography (CT) scan 1 week after the steroid pulse therapy.
Lymphocytes associated with thymoma were analyzed for their CD4/CD8
phenotype and glucocorticoid receptor (GR). TdT-mediated dUTP-biotin
nick-end labeling (TUNEL) staining was used to analyze the apoptotic
lymphocytes and epithelial cells. RESULTS: The overall response rate to
the steroid pulse therapy was 47.1% (8 of 17). The reduction in tumor
size was most prominent in type B1 thymomas; there were significant
differences between type AB and type B1 thymomas (P = .0234) and type
B1 and type B3 thymomas (P = .0068). The reduction in tumor size was
accompanied with a marked reduction in the CD4+8+ double-positive
immature thymocytes that expressed higher levels of glucocorticoid
receptor. Apoptotic changes were observed in both neoplastic epithelial
cell and lymphocyte components after glucocorticoid therapy.
CONCLUSIONS: The efficiency of preoperative steroid pulse therapy in
type B1 thymoma was most prominent, which is probably related to the
specific effect on GR-rich CD4+8+ double-positive immature lymphocytes,
which are abundant in this type of thymoma.
PMID: 16598701 [PubMed - indexed for MEDLINE]
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Experience with starting tacrolimus postoperatively after transsternal extended thymectomy in patients with myasthenia gravis.
Ponseti JM, Azem J, Fort JM, Lopez-Cano M, Vilallonga R, Gamez J, Armengol M.
Unit
of Myasthenia Gravis, Department of Surgery, Hospital General
Universitari Vall d'Hebron, Autonomous University of Barcelona, Spain.
jmponseti@vhebron.net
BACKGROUND: Thymectomy is a standard
treatment of myasthenia gravis (MG). Immunomodulating agents are
frequently given during the post-thymectomy latency period until
complete remission is fully consolidated, but serious side effects is a
relevant clinical problem for patients on long-term immunomodulating
treatment. OBJECTIVE: To assess the effectiveness of starting
tacrolimus in the immediate postoperative period in MG patients
undergoing transsternal extended thymectomy, with complete stable
remission (CSR) as the primary outcome of the study. METHODS:
Forty-eight MG patients received tacrolimus, 0.1 mg/kg per day b.i.d.
(started 24 h after thymectomy) and prednisone 1.5 mg/kg/day.
Histologically, 34 patients had hyperplasia, 20 thymic involution, and
14 thymoma. Of the 48 patients, 40 completed 1 year of tacrolimus
therapy, 38 completed 2 years, 27 completed 3 years, 21 completed 4
years, and 9 more than 5 years. Mean dose of tacrolimus was 4.9 mg/day
(range 2-8 mg/day) with a mean plasma drug concentration of 7.6 ng/mL
(range 7-9 ng/mL). Prednisone could be withdrawn after the first year
in 93.7% of patients and at 2 years in 100%. RESULTS: The mean
follow-up was 24.4 months, SD 17.3 (range 6-60 months). Improvement of
muscular strength and decrease of anti-AChR antibodies were
statistically significant (p < 0.001) shortly after operation. CSR
was obtained in 33.4% of patients, pharmacological remission in 62.6%;
4% of patients had minimal symptoms. None of the patients with thymoma
achieved CSR. The estimated median follow-up to obtain a CSR was 37.9
months (95% confidence interval [CI] 26.4-49.5 months). The overall
crude CSR rate was 33.4%, with 47% for non-thymoma patients. The
probability to achieve CSR at 3 years was 67% for the non-thymomatous
group. CONCLUSIONS: Long-term immune-directed treatment with tacrolimus
to improve the effectiveness of thymectomy in MG is feasible and was
associated with a high rate of CSR in patients without thymoma.
PMID: 16709310 [PubMed - in process]
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Recurrent malignant thymoma diagnosed by EUS-guided Trucut biopsy.
Larghi A, Rodriguez-Wulff E, Noffsinger A, Dye CE.
Unita Operativa di Endoscopia Digestiva, Policlinico A. Gemelli, Rome, Italy.
PMID: 16650555 [PubMed - in process]
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Ectopic hamartomatous thymoma: a case report with immunohistochemical study and review of the literature.
Kushida Y, Haba R, Kobayashi S, Ishikawa M, Doi T, Kadota K.
Department of diagnostic pathology, Kagawa University, Japan. ykushida@med.kagawa-u.ac.jp
Ectopic
hamartomatous thymoma (EHT) is a rare benign tumor. We present a case
of EHT, which was seen as subcutaneous mass on the left supraclavicular
area in a 19-year-old man. The tumor consisted of spindle cells,
epithelial cells, adipose cells, and a small amount of lymphocytes, as
described previously. Immunohistochemically, spindle cells were
positive for keratin, a-smooth muscle actin, CD34 and vimentin, but
negative for desmin and S-100 protein. Lymphocytes were positive for
CD45RO but negative for CD20, CD1a, and CD99. Approximately, 5% of
cells were positive for MIB-1 and no cells stained for p53 and bcl-2.
Recognition of EHT is important and needs to be differentiated from
high-grade sarcomas such as synovial sarcoma or glandular malignant
peripheral nerve sheath tumor.
Publication Types:
PMID: 16640545 [PubMed - indexed for MEDLINE]
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Expression of the onconeural CV2/CRMP5 antigen in thymus and thymoma.
Camdessanche JP, Lassabliere F, Meyronnet D, Ferraud K, Absi L, Honnorat J, Antoine JC.
Equipe d'Accueil 3062, Universite Jean-Monnet, Saint-Etienne, France.
Anti-CV2
antibodies (AB) react with the developmentally regulated neural
proteins CRMPs and particularly with CRMP5. They occur with small cell
lung cancer (SCLC) and thymoma. SCLCs universally express CRMP5. We
investigated the expression of CRMPs in thymoma and thymus. In thymoma,
none of the CRMPs were detected by immunohistochemistry in tumorous
epithelial cells with specific antibodies including CRMP5 but an
antibody reacting with a peptide common to the CRMPs labeled a 66-kDa
protein in Western blot of rat brain, thymus, and thymoma extracts.
Thus, the normal CRMP5 is probably not expressed by tumorous epithelial
cells. These results indicate that the mechanisms leading to CRMP5
autoimmunization are different in SCLC and thymoma.
PMID: 16519949 [PubMed - indexed for MEDLINE]
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Nourishing vascularization of a thymoma issued from a left internal thoracic artery graft.
Thomas PA, Collart F, Doddoli C, Gariboldi V, Moulin G.
Department of Thoracic Surgery, Sainte Marguerite-CHU Sud, Marseille, France. Pascal-alexandre.Thomas@mail.ap-hm.fr
Publication Types:
PMID: 16678622 [PubMed - indexed for MEDLINE]
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Cavernous hemangioma of the anterior mediastinum: case report and 50-year review of Japanese cases.
Yamazaki A, Miyamoto H, Saito Y, Matsuzawa H, Sakao Y, Anami Y.
Department of General Thoracic Surgery, Juntendo University School of Medicine, Tokyo, Japan.
Hemangioma
is a rare tumor of the mediastinum. We report a case of cavernous
hemangioma of the anterior mediastinum that was incidentally detected
by chest radiography during a routine health check. A mass lesion was
seen in the anterior mediastinum on computed tomography and magnetic
resonance imaging of the chest. Because a thymoma with cystic
degeneration was suspected preoperatively, extended thymectomy was
performed. The tumor was confined to the left lobe of the thymus
without invasion into the adjacent structures. It measured 42x32x17 mm,
and was completely resected without any major bleeding. Pathological
examination led to the diagnosis of cavernous hemangioma. A total of 61
cases of mediastinal hemangioma, including our case, reported in Japan
over the past 50 years are reviewed.
PMID: 16764313 [PubMed - in process]
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Non-small-cell lung cancer associated with non-thymomatous myasthenia gravis.
Sakamaki Y, Yoon HE, Oda N.
Department of General Thoracic Surgery, Rinku General Medical Center, Izumisano, Osaka, Japan.
A
non-small-cell lung cancer without distant metastases was incidentally
found in a 77-year-old man who had suffered from myasthenia gravis (MG)
without thymoma. The patient's condition was stabilized by oral
pyridostigmine bromide which he had taken during the past 6 years. He
simultaneously underwent thymectomy and left lower lobectomy with
regional lymph node dissection. Although postoperative myasthenic
crisis occurred, mechanical ventilation and intravenous steroid pulse
relieved the patient and the symptoms improved thereafter. Cases of
operable lung cancer with non-thymomatous MG have rarely been reported
and the appropriate therapeutic strategy for such cases remains to be
debated. Their causal association remains to be identified, whereas
some studies have implied that immune disorder due to the abnormal
thymus might possibly enhance the oncogenesis of extrathymic
malignancies. Myasthenic crisis should also be taken into account in
postoperative management of MG patients who simultaneously undergo
thymectomy and lobectomy for synchronous lung cancer.
PMID: 16764310 [PubMed - in process]
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[A resected case of sclerosing thymoma]
[Article in Japanese]
Kim YH, Ishii G, Naito Y, Goto K, Nagai K, Nishiwaki Y.
Division of Thoracic Oncology, National Cancer Center Hospital East.
An
asymptomatic 47-year-old man was found to have an anterior mediastinal
nodule on an annual chest X-ray. Chest CT showed a 2cm nodule in the
left anterior mediastinum. Because thymoma was suspected, total
thymectomy was performed. Histologically, the greater part of the
nodule was composed of hyalinized fibrous tissues and there was a
scattered, small aggregation of spindle to oval cells, showing positive
for AE 1/3. Therefore this nodule was diagnosed as sclerosing thymoma,
which is a subtype of thymoma first reported in 1994. Since few reports
of this disease have been made so far, needless to say its clinical
features or pathogenesis, even its very existence is not well known.
Sclerosing thymoma should be taken into account, when only fibrous
tissues are obtained by biopsy in patients with anterior mediastinal
tumors.
Publication Types:
PMID: 16780103 [PubMed - indexed for MEDLINE]
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[Clinicopathologic study of metaplastic thymoma]
[Article in Chinese]
Jin M, Liu B, Wang L, Xu JY.
Department of Pathology, Sir Run Run Shaw Hospital, Zhejiang University, Hangzhou 310016, China. srrshjinmei@163.com
OBJECTIVE:
To study the clinicopathologic characteristics of metaplastic thymoma.
METHODS: Resection specimens of 3 cases of metaplastic thymoma were
studied by light microscopy, immunohistochemistry and electron
microscopy. RESULTS: All the 3 patients were females and aged 33, 58
and 45 years respectively. Histologically, a biphasic growth pattern,
consisting of intimate admixture of epithelial cells and spindle cells,
was noted. The epithelial cells showed mild cytologic atypia, sometimes
nuclear grooves and pseudonuclear cytoplasmic inclusions. These cells
were arranged in nests and anastomosing cords. Mitotic figures were
rarely seen. On the other hand, the spindle cells were bland-looking,
mitotically inactive and arranged in fascicles. Immunohistochemical
study showed that the epithelial cells strongly expressed cytokeratin
(AE1/AE3) but not vimentin or CD5. The proliferation index, as
demonstrated by Ki-67 immunostaining, was about 3% to 5%. In contrast,
the spindle cells were diffusely positive for vimentin and epithelial
membrane antigen. Staining for CD5 and CD20 was negative. The
background lymphocytes were positive for CD3, but not for TdT and CD99.
Ultrastructurally, well-formed desmosomes or hemidesmosomes were
identified in the epithelial element. They were not detected within the
spindle cells. CONCLUSION: Metaplastic thymoma is a rarely encountered
indolent or low-grade thymic tumor and may represent a distinct
clinicopathologic entity.
PMID: 16777000 [PubMed - in process]
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Long-term therapeutic efficacy and safety of low-dose tacrolimus (FK506) for myasthenia gravis.
Tada M, Shimohata T, Tada M, Oyake M, Igarashi S, Onodera O, Naruse S, Tanaka K, Tsuji S, Nishizawa M.
Department
of Neurology, Resource Branch for Brain Disease Research, Brain
Research Institute, Niigata University, 1-757 Asahi-machi-dori Niigata,
Niigata 951-8585, Japan.
OBJECTIVE: To elucidate the long-term
therapeutic efficacy and safety of low-dose FK506 (tacrolimus) in
patients with myasthenia gravis (MG). PATIENTS AND METHODS: We treated
nine patients with MG (all women: age range: 35-83years (mean:
51.1years); MGFA classification: 4 type IIa, 4 type IIb, and 1 type IVb
patients) with FK506 for more than 24months (observation period:
24-46months). All the patients had undergone extended thymectomy before
FK506 treatment; two patients (22.2%) had noninvasive thymoma and six
(66.7%) had thymic hyperplasia. We evaluated total Quantitative MG
(Q-MG) score, anti-acetylcholine receptor (AChR) antibody titer in the
blood, interleukin 2 (IL-2) production in peripheral blood mononuclear
cells (PBMCs), administration dosage of prednisolone (PSL), and adverse
effects of FK506. RESULTS: A reduction in steroid dosage of 50% without
worsening of the symptoms was observed 1year after FK506 administration
in three out of six steroid-dependent MG patients (50.0%). The total
Q-MG scores (range: 0-39 points) at 6months and 1year after FK506
administration improved by 3 points or more in six (66.7%) and seven
(77.8%) out of nine patients, respectively. The efficacy of FK506 was
maintained for more than 2years. Although adverse effects were observed
in three patients (33.3%), these were not serious. CONCLUSIONS: Our
study indicates that low-dose FK506 treatment may be efficacious not
only in controlling intractable myasthenic symptoms, but also in
reducing steroid dosage, and that FK506 is safe as an adjunctive drug
to PSL for MG treatment for a maximum of 3years.
PMID: 16631797 [PubMed - as supplied by publisher]
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Inhibition
of interleukin-4 production in activated T cells via the downregulation
of AP-1/NF-AT activation by N-lauroyl-D-erythro-sphingosine and
N-lauroyl-D-erythro-C20-sphingosine.
Park J, Kim SH, Li Q, Chang YT, Kim TS.
School of Life Sciences and Biotechnology, Korea University, Seoul, Republic of Korea.
Allergic
diseases are hypersensitivity disorders that are associated with the
generation of specific immunoglobulin E (IgE) in response to
environmental allergens. Interleukin (IL)-4, which is primarily
produced by the CD4(+) T cells, is an important stimulus for the
switching of the antibody isotype to IgE in both mice and humans. In a
previous study, we demonstrated that ceramide derivatives coupled with
a lauroyl group exerted strong inhibitory effects on IL-4 production in
T cells. In this study, we attempted to characterize the mechanisms
underlying the inhibition of IL-4 production in T cells. Two ceramide
derivatives, N-lauroyl-D-erythro-sphingosine and
N-lauroyl-D-erythro-C(20)-sphingosine (hereafter abbreviated as LES and
LECS, respectively), were shown to significantly inhibit the production
of IL-4 in both primary CD4(+) T cells and EL4 T thymoma cells in a
dose-dependent manner. LES and LECS also inhibited the activity of the
IL-4 gene promoter in EL4 cells transiently transfected with IL-4 gene
promoter constructs, but this effect was impaired in EL4 cells that had
been transfected with an IL-4 promoter construct deleted of a P4 site
harboring the AP-1 and NF-AT binding sites. Furthermore, LES and LECS
inhibited the DNA binding activities of both AP-1 and NF-AT
transcription factors. In addition, LES and LECS were demonstrated to
suppress PMA-stimulated PKC activity, although they exerted no
significant effects on the protein levels of the conventional PKCs.
These results indicate that the ceramides, LES and LECS, may inhibit
the production of IL-4 in the activated T cells, via the downregulation
of AP-1/NF-AT activation and PKC activity.
PMID: 16448625 [PubMed - indexed for MEDLINE]
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Noninvasive cystic thymoma in an adolescent boy.
Verhey PT, Hopkins KL, Primack SL, Radovich N, Zigman A.
Department of Radiology, Oregon Health & Science University, 3181 S.W. Sam Jackson Park Rd., Portland, OR 97239, USA.
Publication Types:
PMID: 16554600 [PubMed - indexed for MEDLINE]
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Malignant
thymoma associated with fatal myocarditis and polymyositis in a
32-year-old woman with a history of hairy cell leukemia.
Gidron A, Quadrini M, Dimov N, Argiris A.
Division
of Hematology-Oncology, Department of Medicine, Northwestern University
Feinberg School of Medicine and the Robert H. Lurie Comprehensive
Cancer Center of Northwestern University, Chicago, Illinois, USA.
Publication Types:
PMID: 16601447 [PubMed - indexed for MEDLINE]
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Thymoma classification: current status and future trends.
Suster S, Moran CA.
Department of Pathology, Division of Anatomic, Pathology, the Ohio State University, Columbus 43231, USA.
The
classification of thymic epithelial neoplasms has been a controversial
topic for many years. Recent advances in diagnostic methods and renewed
interest in the biology of these tumors has led to efforts by
investigators to shed new light on their biologic behavior and to offer
novel perspectives on these unusual neoplasms. Several new
classification schemes have been proposed, including the new World
Health Organization schema for the histologic typing of tumors of the
thymus. We review the current status of thymoma classification and
comment on problem areas and future trends that may offer a more
pragmatic approach to these tumors.
Publication Types:
PMID: 16627265 [PubMed - indexed for MEDLINE]
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A 75-year-old man with an asymptomatic pleural-based mass discovered on routine chest radiographs. Primary pleural thymoma.
Qing G, Ionescu DN, Colby TV, Leslie KO.
Department of Pathology, University of Manitoba, Winnipeg, Canada. gqing@hsc.mb.ca
Publication Types:
PMID: 16594763 [PubMed - indexed for MEDLINE]
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Intradermal
vaccination of dendritic cell-derived exosomes is superior to a
subcutaneous one in the induction of antitumor immunity.
Hao S, Ye Z, Yang J, Bai O, Xiang J.
Research
Unit, Division of Health Research, Saskatchewan Cancer Agency,
Department of Oncology, College of Medicine, University of
Saskatchewan, Saskatoon, Saskatchewan, Canada.
Because dendritic
cell (DC)-derived exosomes (EXO) harbor many important DC molecules
involved in inducing immune responses, EXO-based vaccines have been
extensively used to induce antitumor immunity in different animal tumor
models. However, it is not clear which route of EXO administration can
induce more efficient antitumor immune responses. In this study, we
compared the antitumor immunity derived from EXO vaccine by way of the
two common administration routes, the subcutaneous (s.c.) and the
intradermal (i.d.) administrations. Our data showed that the i.d. EXO
administration resulted in more EXO-absorbed DC migrating into the
T-cell areas of draining lymph nodes than the s.c. administration.
Interestingly, the i.d. EXO administration also resulted in an enhanced
ovalbumin (OVA)-specific CD8(+) T-cell proliferation and CD8(+) CTL
effector responses in vivo, compared to the s.c. administration.
Similarly, compared to the s.c. vaccination, the i.d. vaccination
induced stronger antitumor immunity in the animal tumor model.
Therefore, the i.d. EXO vaccination is superior to the s.c. one and
should be considered when EXO-based vaccine is designed.
PMID: 16706635 [PubMed - indexed for MEDLINE]
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A rare case of intracardiac thymoma.
Kazemi S, Kress DC, Gal RA, Gupta A.
Aurora
Sinai/St. Luke's Medical Centers, University of Wisconsin School of
Medicine and Public Health-Milwaukee Clinical Campus, Milwaukee,
Wisconsin 53233, USA.
PMID: 16640719 [PubMed - in process]
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The role of radiation therapy in thoracic tumors.
Kong FM, Zhao L, Hayman JA.
Department
of Radiation Therapy, University of Michigan, 1500 East Medical Center
Drive, Ann Arbor, MI 48109, USA. fengkong@med.umich.edu
Radiation
plays an important role in the treatment of thoracic tumors. During the
last 10 years there have been several major advances in thoracic RT
including the incorporation of concurrent chemotherapy and the
application of con-formal radiation-delivery techniques (eg,
stereotactic RT, three-dimensional conformal RT, and
intensity-modulated RT) that allow radiation dose escalation. Radiation
as a local measure remains the definitive treatment of medically
inoperable or surgically unresectable disease in NSCLC and part of a
multimodality regimen for locally advanced NSCLC, limited stage SCLC,
esophageal cancer, thymoma, and mesothelioma.
PMID: 16730299 [PubMed - in process]
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HLA-DQ polymorphism in Turkish patients with myasthenia gravis.
Saruhan-Direskeneli G, Kilic A, Parman Y, Serdaroglu P, Deymeer F.
Department of Physiology, I.U. Istanbul Medical Faculty, Istanbul, Turkey. gsaruhan@istanbul.edu.tr
Genetic
susceptibility to myasthenia gravis (MG) is reported frequently and
varies depending on the clinical presentation of the patients. HLA-DQ
genotyping was performed in 132 patients using polymerase chain
reaction and sequence-specific oligonucleotide hybridizations in the
Turkish population for the first time in this study. Antibody
positivities against acetylcholine receptor and titin were 81 and 27%,
respectively. Sixty-five percent of the patients had disease onset
before 40 years of age (EOMG). Overall distribution of DQA1*0103 (odds
ratio (OR): 0.5) and DQB1*0502 (OR: 1.9) alleles was different in
patients and an ethnically matched healthy control group. Among the
subgroups, DQB1*02 was significantly more frequent in EOMG (OR: 1.8),
in women with MG (OR: 2.4), and in women with EOMG (OR: 2.8), whereas
DQA1*0102 and DQB1*502 (OR: 2.3 for both) were increased and DQA1*0103
(OR: 0.04) was decreased in men with MG. Seropositivity was associated
with both DQA1*03 (OR: 12.1) and DQB1*0302 (OR: 14.2) in the patient
group. DQA1*02 (OR: 4.9) was associated with the presence of anti-titin
antibodies, whereas DQA1*0101 (OR: 3.7) and *0102 (OR: 2.9) were more
frequent in patients without this antibody. The presence of thymoma in
MG was positively associated with DQB1*0301 (OR: 2.8), and DQB1*02 (OR:
0.3) was significantly less frequent in this group. The HLA-DQ
associations in subgroups of MG suggest that the heterogeneity of the
disease may be influenced by different genes or even by different
alleles. DQ alleles have proved to be relatively informative
polymorphisms in studying MG.
PMID: 16720217 [PubMed - in process]
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Hypereosinophilia in a patient with invasive thymoma with clonal T-lymphocyte expansion expressing CD4, CD8, and CD25 antigens.
Sumi M, Nunoda K, Mizutani T, Ishii Y, Gotoh A, Kimura Y, Suga Y, Ohira T, Miyajima K, Serizawa H, Mukai K, Kato H, Ohyashiki K.
First Department of Internal Medicine, Tokyo Medical University, Tokyo, Japan. m-sumi@mta.biglobe.ne.jp
We
report the case of a patient with hypereosinophilia and invasive
thymoma harboring probable clonal proliferation of CD4+, CD8+, and
CD25+ T-lymphocytes. A 64-year-old woman had eosinophilia (14.1 x
10(9)/L) and an anterior mediastinal tumor with elevated levels of
serum immunoglobulin E (609.8 mg/dL) and interleukin 5 (239 pg/mL).
Bone marrow aspirate showed marked infiltration by morphologically
normal eosinophils with a normal karyotype but no FIP1L1-PDGFRA fusion
gene. Flow cytometric analysis revealed an increasing number of
CD3+/CD25+ lymphocytes in the peripheral blood, and the resected
thymoma had infiltrated lymphocytes with CD4/CD8/CD25 antigens.
Moreover, the thymoma had T-cell receptor rearrangements with a
cytogenetically clonal nature, ie, t(2;4)(p22;q26). Although the number
of patients with thymoma showing hypereosinophilia is small, this case
suggests that a subset of patients with thymoma may have clonal
expansion of T-lymphocytes with abnormal phenotypes that affect
clinical manifestations, including hypereosinophilia.
Publication Types:
PMID: 16720555 [PubMed - indexed for MEDLINE]
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Resected invasive thymoma with multiple endocrine neoplasia type 1.
Kojima Y, Ito H, Hasegawa S, Sasaki T, Inui K.
Department of General Surgery, Yokohama City University Medical Center, Yokohama, Japan.
We
report a rare case of multiple endocrine neoplasia (MEN) type 1 with
thymoma. A 57-year-old woman with a chronic duodenal ulcer and
hypoglycemia had been seen at a nearby clinic. Abdominal echogram
revealed two nodules in the pancreas and she was referred to our
hospital for evaluation. Her diagnosis was MEN type 1, gastrinoma and
hyperparathyroidism with anterior mediastinal tumor. There were high
calcium levels in the blood and urine. Gastrin was quite high. A chest
X-ray revealed a retrosternal tumor. Computed tomography revealed an
anterior mediastinal tumor without sign of invasion to the surrounding
organs, and two small masses in the pancreas. Cervical echogram
revealed a few masses in both sides behind the thyroid. From these
findings, her preoperative diagnosis was MEN type 1 with thymic
carcinoid or thymoma. We performed thymectomy and parathyroidectomy
concomitantly. The mediastinal tumor was diagnosed as invasive thymoma.
PMID: 16642925 [PubMed - in process]
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[A case of Good syndrome accompanied by myasthenia gravis: immunological evaluations]
[Article in Japanese]
Katsuki Y, Suzuki S, Takahashi Y, Satoh T, Nogawa S, Tanaka K, Suzuki N, Kuwana M.
Department of Neurology, Keio University School of Medicine.
Good
syndrome, characterized by both thymoma and hypogammaglobulinemia, is a
rare immunodeficient disorder. We experienced a case of Good syndrome
accompanied by myasthenia gravis (MG). A 58-year-old man was admitted
to our hospital because of muscle weakness and fatigability. Based on
the presence of anti-acetylcholine receptor (AChR) antibody and
thymoma, he was diagnosed as having MG. Peripheral blood lymphocyte
count was normal, but gammaglobulin levels were markedly decreased (IgG
283 mg/dl, IgA 17 mg/dl, IgM 1 mg/dl). Clinical remission of MG was
achieved by thymectomy followed by high-dose corticosteroids. Despite
monthly intravenous immunoglobulin supplementation, he suffered from
repeated respiratory tract infections and candidiasis. Body CT revealed
adrenal tumor and pancreatic cancer with liver metastasis, and he died
of bacterial pneumonia. Immunological evaluations showed complete lack
of CD19+ B cell in the peripheral blood and responses of peripheral
blood mononuclear cells to mitogens. Peripheral blood T cells responded
to a suboptimal concentration of a recombinant AChR fragment: this
pattern of AChR-induced T cell response was typical of MG patients. We
failed to detect IgG autoantibodies reactive with B cells in his serum.
Patients with Good syndrome represent imbalance of immune responses,
leading to both immunodeficiency and autoimmunity.
Publication Types:
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