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)

Two women, 62 and 66 years old, with combined small cell carcinoma and transitional cell carcinoma of the renal pelvis are reported. The clinical picture was similar to that seen in conventional transitional cell carcinoma. A brief review of the literature indicates that in the urinary tract, small cell carcinoma occurs most commonly in the bladder and is exceedingly rare in the renal pelvis; only 2 cases have been reported previously. The morphological spectrum of the small cell carcinomatous component is similar to that seen in lung tumors. Neuroendocrine differentiation of the small cell carcinoma component was supported by a positive immunoreaction to neuron-specific enolase in both cases and to synaptophysin in 1. One patient died with metastases 8 months after diagnosis, and 1 was alive with clinical evidence of lymph node metastases and contralateral papillary transitional cell carcinoma of the renal pelvis 16 months after diagnosis.
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PMID:Small cell carcinoma of the renal pelvis: a clinicopathological, morphological and immunohistochemical study of 2 cases. 216 82

Twenty-seven cases of classical bronchial carcinoid were followed up for at least 10 years after curative resection. Surgery was conservative in 15 cases. True lymph-node metastases were identified at operation in two cases. Distant metastases were confirmed in two patients after 5 and 10 years, respectively. One died of unrelated cause 10 years, and the other is alive 19 years, postoperatively. Radiotherapy relieved bone pain in both cases, and chemotherapy halted disease progression in one of them. In immunostaining the original histologic material, all carcinoids expressed neuron-specific enolase, and most expressed chromogranin A, synaptophysin and serotonin. Demonstration of ACTH and related opiopeptides did not portend a more aggressive course or identify cases with nodal metastases. Despite their low malignant potential, long-term follow-up of bronchial carcinoids is warranted. Even in the rare cases of distant metastases, long-term survival seems to be the rule. The occasional presence of nodal metastases and/or demonstration of ACTH and other opiopeptides, while more frequent in neuroendocrine carcinomas, are not inconsistent with bronchial carcinoids and seemingly do not alter their overall favorable prognosis.
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PMID:Long-term follow-up of classical bronchial carcinoid tumors. Clinicopathologic observations. 216 32

To further characterize the clinicopathologic spectrum of colorectal neuroendocrine neoplasia, 24 carcinomas with neuroendocrine differentiation were subtyped as either small cell neuroendocrine, oat cell variant (six cases), small cell neuroendocrine, intermediate variant (16 cases), or moderately differentiated neuroendocrine carcinoma (two cases). Five oat cell variants, 14 intermediate variants, and two moderately differentiated tumors were studied with antibodies to cytokeratin, vimentin, epithelial membrane antigen (EMA), neuron-specific enolase (NSE), chromogranin (CRG), synaptophysin (SYN), neurofilament, S-100 protein, carcinoembryonic antigen (CEA), and Leu-7. All tumors were immunoreactive for cytokeratin and the majority were also positive for EMA and NSE. Positivity for specific neuroendocrine markers was uncommon, with SYN reactivity noted in one oat cell variant and four intermediate variants, and CRG positivity observed in four intermediate variants and one moderately differentiated tumor. Ultrastructural analysis of four oat cell, eight intermediate, and one moderately differentiated tumor revealed neurosecretory-type, dense-core granules in all lesions, except two oat cell variants studied from paraffin-retrieved material. Hepatic and regional lymph node metastases were noted in five of six oat cell, eight of 16 intermediate, and two of two moderately differentiated tumors. Of 17 patients with follow-up (four oat cell, 11 intermediate, and two moderately differentiated tumors), only two individuals were alive after 1 year. There were no detectable differences in survival or response to treatment between morphologic subtypes. The prognosis of colorectal neuroendocrine carcinoma appears worse than for adenocarcinoma of comparable stage. Their distinction is thus warranted, especially in regard to the intermediate variant and moderately differentiated tumors, which may be potentially misinterpreted as forms of adenocarcinoma.
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PMID:Neuroendocrine carcinoma of the colon and rectum. A clinicopathologic, ultrastructural, and immunohistochemical study of 24 cases. 217 27

Merkel cells are clear oval cells in the epidermis and outer root sheaths of hair follicles, which are probably of epithelial origin, share ultrastructural features with neuroendocrine cells, and are found in association with touch receptors. In the eyelid, they occur singly in the epidermis and external root sheaths of hairs and eyelashes, and in specialized touch spots alternating with eyelashes. Their typical electron microscopical and antigenic features include dense-core granules, intranuclear rodlets, spinous processes, and a positive reaction for specific cytokeratins, epithelial membrane antigen, neuron-specific enolase, chromogranin and synaptophysin. Merkel cell carcinoma probably develops from precursor cells which give rise to keratinocytes and Merkel cells, and nearly one out of ten Merkel cell carcinomas occur in the eyelid and periocular region. They tend to be bulging lesions near the lid margin of elderly patients, reddish in color, and erythematous with telangiectatic vessels. The diagnosis is based on the frequent presence of neurofilaments and paranuclear aggregates of intermediate filaments in addition to features typical of normal Merkel cells. The tumor often mimics lymphoma or undifferentiated carcinoma and frequently invades lymphatic vessels. One third of Merkel cell carcinomas recur, almost two thirds give rise to regional node metastases, and up to one half metastasize widely and result in death. Initial treatment should be prompt and aggressive, with wide resection and routine postoperative irradiation. Although metastatic lesions often respond to radiation therapy and cytostatic drugs, these treatments are mainly of palliative value.
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PMID:The Merkel cell and associated neoplasms in the eyelids and periocular region. 227 47

A large mediastinal tumour was discovered radiologically in a 50-year-old man, with at times blood-tinged sputum and dyspnoea. Parasternal needle biopsy revealed a thymic carcinoid. After surgical excision of the tumour, which had argyrophilic foci (total weight of tumour 1,605 g), histochemical analysis demonstrated neuron-specific enolase, synaptophysin and chromogranin, as well as calcitonin (as the only hormone). For the first time in a thymic carcinoid, radioimmunoassay demonstrated growth-hormone-releasing hormone (2 micrograms/g tumour tissue). Post-operative radiotherapy (total dosage 50.5 Gy) was instituted because histological examination had shown invasion of the capsule and blood vessels. There has been no local recurrence or metastases after three years.
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PMID:[Morphology and clinical picture of thymus carcinoid]. 231 18

The nuclear DNA content of 17 pancreatic neuroendocrine tumors was measured from paraffin-embedded tissue with flow cytometry. The tumors were classified by immunostaining with antisera for synaptophysin, insulin, gastrin, glucagon, pancreatic polypeptide, somatostatin, and vasoactive intestinal polypeptide. Eight (47%) of the 17 tumors were aneuploid, and two (12%) were multiploid (had two aneuploid stemlines of cells). Seven of the eight insulinomas, one of the four gastrinomas, and two of the four nonspecified neuroendocrine tumors had an abnormal nuclear DNA content. The DNA indices of the aneuploid and multiploid cases ranged from 1.13 to 1.93, and three cases had a DNA index greater than 1.50. During the follow-up for up to 16 years (mean, 7 years), one patient with diploid nonspecified tumor died of the disease, another patient with a multiploid gastrinoma had metastatic disease develop, and a third patient with a multiploid nonspecified tumor was alive with the disease. The authors conclude that many neuroendocrine tumors of the pancreas have an abnormal nuclear DNA content as measured by DNA flow cytometry. DNA multiploid pancreatic neuroendocrine tumors may be associated with a less favorable clinical course, but this needs to be confirmed in additional studies.
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PMID:DNA ploidy in pancreatic neuroendocrine tumors. 234 35

Acetone-fixed frozen sections of 15 malignant melanomas of the skin with metastases were studied immunohistochemically for the presence of different types of intermediate filament proteins, synaptophysin, muscle cell actins, and desmoplakins. One of the melanomas was a primary toe tumor, and the others mainly regional lymph node metastases. The original diagnosis of melanoma was reconfirmed in each case, and the melanoma diagnosis of the metastases was verified by S100 protein immunostaining in all cases and by a monoclonal antibody to melanoma cells (NK1C3) in 7 cases. All melanomas were prominently vimentin-positive. In 10 of 15 cases, immunoreactive keratin could be demonstrated with antibody CAM 5.2. The presence of keratins was confirmed in selected cases with three other monoclonal antibodies including AE1, PKK1, and a monoclonal antibody specific for keratin number 18. Desmoplakin, another marker of epithelial differentiation, was not found in melanoma cells. Two melanomas contained neurofilament-positive tumor cells, which were however negative for synaptophysin. Desmin, muscle actins, and glial fibrillary acidic protein were not found in the neoplastic cells. On the basis of the present results one could conclude that the protein composition of the cytoskeleton of melanomas is more complex than has been previously thought and most importantly that melanomas may contain keratins.
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PMID:Immunohistochemical spectrum of malignant melanoma. The common presence of keratins. 248 Nov 51

The left eye of an otherwise healthy child was enucleated at the age of 2 months because of an enlarging mass involving the temporal iris, ciliary body, and anterior retina. The initial histopathologic diagnosis was malignant medulloepithelioma with orbital extension. Closer study revealed a superotemporal chorioretinal and ciliary body coloboma; dysplasia of the adjacent retina; a glioneuromatous mass replacing the temporal ciliary body, chamber angle structures, and iris and extending through the sclera to involve the insertion of the lateral rectus muscle; neuroepithelial elements resembling medulloepithelioma; and abnormally developed iris pigment epithelium, and dilator and sphincter muscles. Immunohistochemistry demonstrated that the main mass consisted of neurons positive for neuron-specific enolase (NSE), synaptophysin and neurofilaments, and glial cells expressing vimentin, glial fibrillary acidic protein, and S-100 protein. The neuroepithelial elements reacted positively for cytokeratins and S-100 protein, in addition to NSE and vimentin, suggesting ciliary epithelial rather than embryonic retinal origin. The tumor was rediagnosed as glioneuroma, which in this case was part of a widespread colobomatous dysplasia of the anterior uvea and retina. The patient is alive without metastases or local recurrence 2 years following enucleation and subtotal removal of the lesion.
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PMID:Glioneuroma associated with colobomatous dysplasia of the anterior uvea and retina. A case simulating medulloepithelioma. 262 23

Twenty-one cases of cutaneous neuroendocrine (Merkel cell) carcinoma (CNEC) were examined by the ABC-immunoperoxidase method with a panel of antibodies to 5 intermediate filaments, 6 neuroendocrine-associated antigens, 6 peptide hormones, as well as melanoma-associated cytoplasmic antigen (HMB-45) and leukocyte common antigen. All tumors showed strong cytokeratin staining in characteristic dense, inclusion-like, cytoplasmic globules and in a reticular peripheral cytoplasmic pattern. Cytoplasmic coexpression of inclusions of neurofilament antigen was observed in 9/21 cases. Staining for one or more neuroendocrine markers in formalin-fixed tissue (bombesin, 7/20; chromogranin, 11/21; synaptophysin, 6/21) was weak and focal but present in 17/21 cases. In 3 cases, sections of unfixed, snap-frozen tumor were compared with formalin-fixed tissue, and these showed strong, diffuse staining for multiple neuroendocrine antigens. Immunostaining for peptide hormones was not observed, with the exception of weak, focal staining for insulin (1 case), calcitonin (1 case) and somatostatin (2 cases). In 13 cases DNA indices and S-phase fractions (SPF) were determined by flow cytometry on nuclear suspensions from paraffin blocks. DNA histograms in 12 of 13 cases had normal range DNA content (diploid) and elevated S-phase fractions (mean 15%, range 8 to 22%). Mean SPF was not significantly different in the group of patients who developed recurrent and/or metastatic disease (15.6%, N = 10) compared with patients without recurrence (15.8%, N = 10).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Cutaneous neuroendocrine (Merkel cell) carcinoma: an immunophenotypic, clinicopathologic, and flow cytometric study. 266 40

Four plexosarcomas (gastrointestinal autonomic nerve tumors) characterized by light microscopic, immunocytochemical, and ultrastructural examination, including immunoelectron microscopy in one case, are described. The four neoplasms occurred in the small intestine (duodenum, two; jejunum, one; and ileum, one) and they had an aggressive course with either local or distant metastases. The light-microscopic patterns varied from epithelioid and organoid to spindle cells, mimicking endocrine and sarcomatous neoplasms. Ultrastructurally, these tumors exhibited interdigitating cytoplasmic processes that contained scattered aggregates of membrane-bound granules varying in size from 100 to 300 nm intermixed with empty vesicles and numerous diffusely distributed intermediate filaments. Basal lamina covering cell surfaces, attachment plaques, and myofilaments, as expected in smooth-muscle tumors, were not identified, and diffusely distributed membrane-bound granules, as seen in paragangliomas and carcinoid tumors, were also absent. By immunocytochemistry, the tumors were intensely positive for vimentin and neuron-specific enolase and focally positive for neurofilaments and synaptophysin. In addition, three tumors were S100 protein positive and one stained for vasoactive intestinal peptide. Similar positive immunocytochemical reactions were identified in normal enteric plexus. It is essential to recognize plexosarcomas, which are invariably accompanied by aggressive clinical behavior, in spite of a seemingly benign, mitotically inactive light-microscopic appearance in most instances. Ultrastructural examination can readily separate plexosarcomas from paragangliomas and other sarcomatous and endocrine neoplasms.
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PMID:Gastrointestinal autonomic nerve tumors. 'Plexosarcomas'. 275 84


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