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)

The syndrome of multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant tumour disease of the neuroendocrine system with manifestations in the parathyroids, pancreas, duodenum and pituitary gland and rarely also in the stomach and thymus. Recently, the MEN 1 gene locus has been mapped to the long arm of chromosome 11. This gene most likely belongs to the tumour suppressor genes, the allelic loss of which causes tumour development. The pancreatic and duodenal tumours may metastasize, but usually have a low malignant potential. Clinically, most MEN 1 patients present between the age of 20 and 35 with hyperparathyroidism and/or Zollinger-Ellison syndrome.
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PMID:[Multiple endocrine neoplasia type 1 (MEN 1). Molecular genetics, morphology and prognosis]. 791 16

Interleukin-2 (IL-2) mediates the regression of metastatic cancer, but clinical application is restricted by associated toxicities. Previous studies implicate tumor necrosis factor (TNF) as an important mediator of certain IL-2-induced toxicities. We hypothesized that soluble TNF receptor (sTNFr), a TNF antagonist, would alter lymphocyte trafficking into normal tissues and ameliorate IL-2-induced toxicity. Four groups of C57BL/6 mice were treated for 4 days with intraperitoneal injections of 100,000 IU IL-2 alone, 100,000 IU IL-2 and 30 micrograms sTNFr combined, 30 micrograms sTNFr alone, or equal volumes of saline. Animal activity was graded and blood obtained for SGPT and SGOT. At necropsy, organs were harvested for wet:dry ratios as a measurement of organ edema. The lung, liver, and thymus were examined histologically for lymphocytic infiltration and graded on a scale of 1 to 5. IL-2-treated groups had a statistically significant increase in organ edema, lymphocytic infiltration into the lung and liver, liver enzyme elevation, and pancytopenia when compared with controls. Soluble TNFr significantly suppressed IL-2-induced pulmonary lymphocytic infiltration and associated serum lymphopenia without significant alteration of other IL-2-induced effects. These data implicate TNF as a mediator of the pulmonary lymphocytic infiltration and of lymphopenia that accompanies IL-2 therapy and further suggest that alternative mechanisms are involved in other IL-2-induced deleterious effects.
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PMID:Interleukin-2-induced lymphocyte infiltration of multiple organs is differentially suppressed by soluble tumor necrosis factor receptor. 812 Nov 66

Metastasis of malignant testicular teratoma to numerous sites is recognized and thymic hypertrophy following chemotherapy is frequently seen. The finding of metastatic malignant testicular teratoma in the thymus described here illustrates a further cause of thymic enlargement in this group of patients, which has not been previously reported.
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PMID:Case report: metastatic malignant testicular teratoma of the thymus. 813 Sep 85

Seventy-two anterior mediastinal tumors were operated upon between 1981 and 1991. This accounted for 2% of the 3579 thoracotomies performed during the same period (excluding cardiac surgery). With the exception of Hodgkin's disease, lymphomas and metastases diagnosed either by anterior mediastinoscopy or at thoracotomy, other tumors were treated by the widest possible excision followed by adjuvant treatment in case of incomplete excision. These tumors included 16 metastatic carcinomas and 55 affecting the thymus-including 27 malignant thymomas and 15 benign tumors of the thymus including 2 carcinoids, 6 cases of Hodgkin's disease involving the thymus, 5 non-seminomatous germinal tumors, one differentiated neuro-endocrine carcinoma, 1 thymus lymphoma and 1 seminoma. The approach was via sternotomy in 32 cases and thoracotomy in the other 40. Excision was deemed complete in 45 cases, incomplete in 25 cases and impossible in 2 cases. Extension of surgical excision involved various adjacent organs: the lung in 10 cases and the pericardium in 5. Involvement of the phrenic nerves was found in 25 cases, the aorta in 1, the pulmonary artery in 1 and the vena cava or its afferent vessels in 33 cases. Seven PTFE venous bypasses were performed. Operative mortality was nil. One patient died on the 8th day of undefined causes. Mean tumour survival in metastatic thymomas was nevertheless 19 months. Survival in malignant thymomas was related to the stage of progression of the tumour. When complete excision was possible, survival was 89% with a mean follow-up of 57 months.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Primary surgical resection of tumors of the anterior mediastinum]. 831 77

Carcinoid of the thymus rarely occurs during childhood. The authors identified eight cases in patients younger than 17 years of age. All were associated with Cushing syndrome. Adrenocorticotropic hormone (ACTH) produced by the tumor may be released intermittently, delaying the findings of Cushing syndrome. The authors describe a case of ectopic ACTH production in a teenaged boy who had longstanding hyperpigmentation, increased ACTH levels, and normal cortisol levels. Magnetic resonance imaging of the pituitary had normal findings. Subsequently, severe Cushing syndrome developed. Computed tomography (CT) scans of the chest showed a mediastinal mass that proved to be a thymic carcinoid. The lesion was inoperable. Radiation and chemotherapy were of limited benefit. Metyrapone was used to control hypercortisolism. The patient died with extensive metastases 6 years after initial presentation. CT scans of the chest should be performed in an attempt to localize ectopic ACTH-producing tumors. Surgical excision of the lesion is the treatment of choice. Control of hypercortisolism is essential.
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PMID:Adrenocorticotropic hormone--producing thymic carcinoid in a teenager. 838 Jan 12

Patients with malignant thymoma rarely may also have a peripheral T-cell lymphocytosis. "Lymphocyte spillover" from the thymus into the peripheral blood as well as a second, associated neoplasm (ie, T-cell chronic lymphocytic leukemia) are two hypotheses that have been proposed to explain this clinical phenomenon. We describe another patient with a lymphocyte-rich malignant thymoma associated with peripheral T-cell lymphocytosis. At the time of initial diagnosis, the patient's complete blood cell count was unremarkable. However, subsequent to the development of pulmonary metastases, the patient developed persistent lymphocytosis. The total leukocyte count ranged from 20 to 30 x 10(9)/L, 80% of these cells being lymphocytes. Immunophenotypic analysis of peripheral blood specimens from this patient proved that the circulating cells were mature, polyclonal T cells. The cells expressed the alpha/beta T-cell receptor and the pan-T-cell antigens CD2, CD3, CD5, and CD7, and were negative for both terminal deoxynucleotidyl transferase (TdT) and the CD1 antigen. A mixture of T-helper (CD4+) and T-suppressor (CD8+) cells were present in a ratio of 1:1.6. Gene rearrangement studies revealed that the T-cell receptor beta chain and the immunoglobulin heavy-chain genes were in the germline configuration. Serum samples from this patient were also analyzed for thymic hormones; the level of thymosin alpha 1 was markedly elevated, while that of thymosin beta 4 was decreased. These results effectively exclude the hypothesis that the lymphocytosis represents a second, associated neoplasm. The lymphocyte spillover hypothesis also seems unlikely (although not excluded), since the lymphocytes in lymphocyte-rich thymomas usually have an immature thymic cortical immunophenotype. Furthermore, one might expect nonspecific elevation of all thymic hormone levels with lymphocyte spillover. Thus, we suggest that the lymphocytosis results from a poorly defined immunoregulatory disorder, related to the presence of thymoma, and perhaps secondary thymic hormone imbalance.
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PMID:Malignant thymoma associated with T-cell lymphocytosis. A case report with immunophenotypic and gene rearrangement analysis. 797 8

Murine liver contains alpha beta T cells with intermediate TCR (TCRint) as well as alpha beta T cells with bright TCR. Liver TCRint cells express NK1.1 Ag (NK1+ TCRint) and IL-2 receptor beta chain, both of which are NK cell markers and are not expressed on conventional T cells. Liver NK1+ TCRint cells consist of CD4-8- double negative T cells and CD4+ T cells and have V beta 8+ T cell preponderance. They are dependent on class Ib or CD1 molecules of APC for their development. They can also develop thymus independent manner, because athymic nude mice have this population. These NK1+ TCRint cells in the livers of both euthymic and athymic mice were found to be activated by systemic administration of IL-12 and increased NK1 expression (NK1high TCRint) and cytotoxicity against various NK-sensitive and resistant tumors. Cytotoxicity assays after treatment of IL-12 stimulated hepatic MNC with respective Abs and C revealed that CD4+ NK1high TCRint cells are responsible for IL-12 induced cytotoxicity. Although NK1+ TCRint cells were normally few in the lungs, a significant proportion of NK1high TCRint cells with strong cytotoxicity was also induced in the lung by IL-12. Interestingly, adoptive transfer of IL-12 stimulated hepatic MNC into other mice, which were pre-injected with tumors, inhibits hepatic metastases of EL4 cells and pulmonary metastases of 3LL cells as similarly as IL-12 administration. Transfer experiments after treatment of IL-12 stimulated hepatic MNC with respective Ab and C revealed that depletion of either NK1+ cells, CD3+ cells or CD4+ cells but not CD8+ cells greatly impaired antimetastatic effect in both organs. Thus, CD4+ NK1high TCRint cells are a major antimetastatic population, especially, against hematogenous metastases.
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PMID:[The function and role of extrathymic T cells]. 853 54

A 14-yr-old boy underwent a total thyroidectomy with bilateral neck dissection for a papillary carcinoma with lymph node metastases. Total-body scanning with 3.7 GBq 131I revealed radioiodine accumulation in the anterior mediastinum. CT and MRI demonstrated a mediastinal mass which corresponded to the area of increased radioactivity. Five months later, another therapeutic dose of 131I was followed by a sternotomy and removal of the thymus because a hand-held radiodetecting surgical probe demonstrated that the thymus was the mediastinal structure which concentrated iodine. Thymus histology was negative for thyroid cancer metastases (as further confirmed by the negative immunostaining) and showed cystic Hassall's bodies. Secondary ion mass spectrometry microscopy demonstrated that iodine was located only in the Hassall's bodies, bound to proteins. This finding suggests that an acquired "thyroid follicle-like" structure, as that observed in cystic Hassall's bodies, could be responsible for the epithelial cell iodine uptake. In conclusion, we have provided evidence for the iodine-trapping property of the cystic Hassall's bodies of the thymus, which may be a possible cause of misleading mediastinal radioiodine uptake.
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PMID:Iodine concentration by the thymus in thyroid carcinoma. 891 86

We report a case of an intrathoracic, extrapleural, infantile rhabdomyofibrosarcoma in a 4-year-old boy. Histologically, the primary lesion showed extensive hyalinization and stroma sclerosis and was composed of relatively uniform spindle-shaped, at least focally rather polygonal tumour cells with scattered intracytoplasmatic globoid inclusions. Although chemo- and radiotherapy was given postoperatively, local recurrences and metastases in the lung and thymus have developed; the patient died of tumour disease 3 years later. Recurrences and metastases showed features of tumour progression with higher cellularity and increased mitotic activity. Immunohistochemically, the tumour cells stained strongly positive for vimentin, desmin, and muscle-specific actin, and at least focally for MyoD1; the tumour did not stain for alpha-smooth muscle actin, neural and epithelial markers, or CD34 and CD31. The differential diagnosis of these aggressive tumours in the spectrum of spindle-cell lesions in early childhood is discussed.
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PMID:[Infantile rhabdomyofibrosarcoma. An aggressive tumor in the spectrum of spindle cell tumors in childhood]. 892 96

Neuroendocrine tumors of the thymus, also known as thymic carcinoids, are rare tumors of the anterior mediastinum. They occur sporadically or in association with MEN I syndrome. We present five patients (four male, one female; age of first manifestation 19-53 years) who were operated on at our hospital between 1984 and 1995 for neuroendocrine thymic tumors. A hormone-producing tumor presented with Cushing's syndrome in two patients. Two patients had MEN I syndrome. Only in the female patient the primary tumor was confined to the thymus. We found lymph node metastases in three patients and a distant metastasis in one. The neuroendocrine tumors have a high rate of local recurrences and thus we performed 11 operations using a transthoracic or transsternal approach. Since there was no operative mortality and adjuvant therapies are of limited value, we recommend surgery even in case of recurrence.
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PMID:[Surgical therapy of neuroendocrine tumors of the thymus]. 915 79


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