Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thymoma is a relatively rare tumour. Twelve cases seen in Kuwait Cancer Control Centre over a period of 10 years were analyzed. Two patients had Myasthenia gravis at presentation, and one developed it after the treatment, during remission. Six patients had total resection and four had subtotal resection of the tumour; the remaining had only diagnostic biopsy. All the six patients who had total resection were alive, disease-free with three of them Stage III, whereas, of the four cases who had subtotal resection two are dead, one with local disease and the other with pulmonary metastases. Eight patients were given postoperative radiotherapy and none of them relapsed at the primary site. Of the three patients who had chemotherapy, two had partial remission, and the remaining one failed to show any response. Patients with predominantly epithelial type histology fared worse compared to predominantly lymphocytic type and mixed cell type. Histological subtypes, invasiveness of the tumour and completeness of resection and association of Myasthenia gravis as prognostic features are discussed. The role of radiotherapy and chemotherapy as adjunctive treatment to surgery is reviewed.
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PMID:Management of thymoma. A retrospective study. 843 36

Myasthenia gravis is a neurologic disorder characterized by intermittent muscle weakness which improves after anticholinesterase medication. The pathogenesis of myasthenia gravis is associated with production of autoantibodies to nicotinic acetylcholine receptor in the motor end plate. Most patients do not have an underlying neoplasm, but in 10-15% of the cases, a thymoma may be detected. Apart from thymoma, no other tumor type or organ has consistently been associated with myasthenia gravis. We describe an unusual case of myasthenia gravis in a patient with malignant mixed Mullerian tumor of the uterine corpus. Initial histology revealed malignant mixed Mullerian tumor of a predominant carcinomatous element. At that time, there were no symptoms of muscle weakness. Intraabdominal metastases were detected later, concommitantly with symptoms of muscle weakness and the diagnosis of myasthenia gravis. Histology of the metastases disclosed an exclusive mesenchymal element with striated muscle differentiation. To the best of our knowledge this case is the first report of myasthenia gravis in a patient with malignant mixed Mullerian tumor of the uterine corpus. We propose that the mechanism contributing to myasthenia gravis in this patient was closely associated with the evolving histology and with the nature of the tumor, so that antibodies produced to muscle-like epitopes exposed by malignant cells could have cross-reacted with acetylcholine nicotinic receptors and caused myasthenia gravis.
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PMID:Myasthenia gravis associated with malignant mixed Mullerian tumor of the uterus. 906 62

A group of biphasic low-grade thymic epithelial tumors is presented that we suggest calling low-grade metaplastic carcinoma of the thymus [37]. Four of the patients were men, their age ranging from 44 to 71 years. Three tumors invaded mediastinal fat or pleura. No metastases were present. Histologically, the tumors showed a biphasic pattern with solid carcinomatous areas merging with a spindle cell component. Only few lymphocytes were present. Cytological atypia and mitotic activity were variable in the solid areas, but low in the spindle cell component. The tumors showed expression of cytokeratin, vimentin and/or epithelial membrane antigen (EMA), both in the carcinomatous and in spindle cell components. In two cases, actin expression was also present in both components. In one case, chromogranin, S100 protein, glial fibrillary acidic protein, and neuron specific enolase were expressed in a minority of cells of both components. None of the patients had myasthenia gravis. All patients are alive without distant metastasis, but meanwhile one patient suffers from local recurrence. We conclude that metaplastic carcinoma of the thymus is a clinicopathological entity that is probably distinct from the recently described "thymoma with pseudosarcomatous stroma", and should be distinguished from the usually benign medullary thymomas and the highly aggressive carcinosarcomas and sarcomatoid carcinomas.
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PMID:Low-grade metaplastic carcinomas of the thymus: biphasic thymic epithelial tumors with mesenchymal metaplasia--an update. 1048 86

The ectopic expression of neuronal P/Q-type voltage-gated calcium channels in small cell lung carcinoma (SCLC) is thought to induce antisynaptic autoimmunity in the paraneoplastic Lambert-Eaton myasthenic syndrome. The gene CACNL1A4, encoding the principal (alpha1A) subunit of this calcium channel, is mutated in several inherited neurological disorders. One of these disorders (spinocerebellar ataxia, type 6, or SCA-6) involves the expansion of a trinucleotide (CAG) repeat unit. We hypothesized that a somatic CAG repeat instability of this gene in neoplastic cells might generate a non-self epitope capable of initiating autoimmunity to P/Q-type calcium channels. We therefore analyzed the CACNL1A4 gene in SCLC lines established from metastases derived from seven individual patients (four associated with Lambert-Eaton myasthenic syndrome, one associated with myasthenia gravis, and two not associated with neurological autoimmunity). We compared their CAG repeat numbers (determined by polymerase chain reaction (PCR) amplification followed by separation of products on a 6% polyacrylamide/8M urea gel) to published norms and to DNA from a patient with SCA-6. The number of CAG repeats in SCLC DNA fell within a normal range whether or not the neoplasm was complicated by neurological autoimmunity. Therefore, it is unlikely that somatically unstable CAG repeat units in the gene encoding the P/Q-type voltage-gated calcium channel account for this tumor protein's immunogenicity in the Lambert-Eaton myasthenic syndrome.
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PMID:Immunogenicity of P/Q-type calcium channel in small cell lung cancer: investigation of alpha1 subunit polyglutamine expansion. 1067 74

Malignant thymomas are rare indolent tumours of the anterior superior mediastinum. Despite a benign histologic appearance, some thymomas invade nearby structures or metastasize. Patients are commonly asymptomatic, but some may present with unusual paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, or hypogammaglobulinemia. Since tumour biopsy may potentially disrupt the thymic capsule, it is often not performed. Patients are therefore diagnosed and staged at the time of definitive surgery. Thymomas can generally be categorized into two stages: non-invasive and invasive. Prognosis closely parallels the disease stage. Surgery is the principal treatment and is curative in early stage disease. Radiation therapy, either alone or in combination with chemotherapy, is an option for both incompletely or completely resected disease. Chemotherapy is offered to patients with locally advanced, recurrent, or metastatic thymoma, with excellent responses and prolonged survival. Multicentre co-operative group clinical trials are required to assess novel thymoma therapies to maximize patient resources in this uncommon tumour.
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PMID:Malignant thymoma: current status and future directions. 1077 69

Cancers that cause disturbances of organs or tissues remote from the site of the tumor or its metastases are called paraneoplastic syndromes. The nervous system can be affected at virtually any site, including the neuromuscular junction (e.g., Lambert-Eaton myasthenic syndrome, myasthenia gravis). Paraneoplastic syndromes affecting the central nervous system are characterized by (1) high titers of antibodies that react with both the cancer and the affected portion of the nervous system, (2) specifically reacting T cells in the blood and cerebrospinal fluid, and (3) autopsy evidence of neuronal destruction, inflammatory infiltrates, and antibody penetration. Clinically, paraneoplastic syndromes affecting the central nervous system are usually subacute in onset, rapid in evolution, and cause severe damage, but generally stabilize after several months with or without treatment. Immune suppression does not appear to be particularly effective in treating these disorders. Treatment of the underlying cancer sometimes ameliorates symptoms.
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PMID:Immunology of paraneoplastic syndromes: overview. 1459 73

We describe the clinicopathologic findings in a so far unrecognized thymic tumor. The tumor occurred in a 70-year-old woman with respiratory distress but neither myasthenia gravis nor other symptoms. Metastases or another primary tumor were absent. The well-circumscribed neoplasm was located in the thymic region, measured 18 x 12 x 8 cm, and showed a homogeneous, tan-colored, soft cut surface. By histology, the tumor lacked a true capsule and a lobular growth pattern, was almost devoid of stroma, and infiltrated among remnant thymus lobules. The polygonal tumor cells formed solid sheets, trabeculae, or occurred as single cells that resembled hepatocytes. Proliferative activity was low. Portal structures, sinuses, and bile were absent as were areas of conventional thymoma, adenocarcinoma, or germ cell tumor. The tumor expressed cytokeratins 7 and 19, alpha1-antitrypsin, alpha1-antichymotrypsin, and hep-Par-1. Alpha-fetoprotein (AFP), human beta-chorionic gonadotropin (beta-HCG), placental alkaline phosphatase, CD5, CD30, CD31, CD34, CD45, CD68, CD99, S-100, HMB45, desmin, actin, or neuroendocrine markers were not expressed, and intratumorous CD1a+ or TdT+ immature T cells were absent. AFP was repeatedly undetectable in the blood. Mediastinal tumor recurrence was detected 6 months after surgery. Following radiochemotherapy, the patient has remained free of disease for 26 months. We conclude that this tumor is a thymic carcinoma (WHO type C thymoma). A diagnosis of hepatoid yolk sack tumor appears unlikely considering absence of a bona fide germ cell component, lack of AFP expression, and the patient's female gender. Because of its morphologic and immunohistochemical features, we propose the term "hepatoid thymic carcinoma" for this new type of thymic carcinoma.
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PMID:Hepatoid thymic carcinoma: report of a case. 1504 16

We report on a 50-year-old female who developed pulmonary metastasis 12 years following the resection of a thymoma with microscopic capsular invasion. The patient was found to have a mediastinal mass at the age of 18 years; however, she refused to undergo surgery. At the age of 38 years, the patient underwent surgery for resection of the tumor; it was diagnosed as a macroscopically encapsulated thymoma with microscopic capsular invasion. Multiple pulmonary metastases occurred 12 years following the resection of the tumor; all the metastatic masses were resected. Although the patient suffered from myasthenia gravis 4 months following the resection of pulmonary metastases, she remains free of myasthenia gravis with no recurrence of tumor at 2 years post-surgery. Long-term follow-up is essential for the detection of recurrence after resection of a thymoma with microscopic capsular invasion, and surgery could be the best treatment for distant metastasis in case of resectable lesions.
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PMID:Pulmonary metastasis 12 years after resection of thymoma with microscopic capsule invasion. 1559 63

Thymoma is a rare neoplasm usually with an indolent growth pattern; however, local invasion and/or metastases may occur. The association with several paraneoplastic syndromes, especially myasthenia gravis, makes thymoma an interesting biologic tumor model. Surgery has been the standard of care for early stage disease with high cure rates anticipated. Multimodality therapy can result in long-term disease-free survival for patients presenting with locally advanced disease.
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PMID:Surgical treatment of thymic tumors. 1610 57

Thymoma is a rare epithelial tumor of the thymus, but the most common malignancy in the anterior mediastinum. A unique feature is its association with paraneoplastic syndromes, in particular myasthenia gravis. According to the WHO classification 6 histologic types of thymic epithelial tumors can be discriminated. Tumor stage according to MASAOKA is the most important prognostic factor. Non-invasive tumors (stage I) are usually completely resected and no further therapy is warranted. For incompletely resected tumors and locally advanced invasive thymomas (stage Ill-IV) postoperative radiotherapy with 50-60 Gy is advisable. Chemotherapy, preferably with Cisplatinum, is indicated with inoperable thymomas or metastatic disease. In general thymomas have a fair prognosis even in advanced stage. Long term follow-up is mandatory up to 10 years.
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PMID:[Thymoma]. 1641 43


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