Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An autopsy case of ACTH-producing thymic carcinoid with Cushing's syndrome is reported. The patient was a 63-yr-old man with multiple bone metastases from an undetermined primary site. Hyperpigmentation was observed at the terminal stage. The plasma levels of ACTH, cortisol, chromogranin A, and urinary 17-hydroxy-corticosteroids were extremely high, and ectopic ACTH-producing neuroendocrine tumor was diagnosed. In addition, plasma CRH was high. Autopsy revealed that the patient had primary thymic carcinoid with extensive metastases. Remarkable hyperplasia of the adrenal cortexes and Crooke's hyaline degeneration of the pituitary gland were consistent with Cushing's syndrome by ectopic ACTH production. There were multiple CRH-producing cells without degenerative changes in the hypothalamus. The tumor cells were immunoreactive to ACTH, CRH, and the cleavage enzyme PC2. POMC messenger ribonucleic acid and PC2 messenger ribonucleic acid were detected in the tumor cells by an in situ hybridization method. Expression of PC2 was considered to induce hyperpigmentation by producing alpha MSH. Despite hypercortisolism and ectopic production of CRH by the tumor cells, hypothalamic CRH cells were not atrophic. This case is a good example to demonstrate the correlation between CRH and the hypothalamo-pituitary-adrenal axis as well as hyperpigmentation in Cushing's syndrome.
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PMID:Expression of prohormone convertase, PC2, in adrenocorticotropin-producing thymic carcinoid with elevated plasma corticotropin-releasing hormone. 855 Jul 83

A rare insulin-immunoreactive neuroendocrine tumor of the duodenum in a 54 year old male is reported. The incidentally identified tumor was located on the anterior free wall of the duodenal bulb and measured approximately 6 mm in diameter. Uncomplicated endoscopic resection of the tumor was carried out. The lesion exhibited classic histologic features of insulinoma of the beta-islet cell type with stromal amyloid deposition. In addition to positive reactivities of chromogranin A, neuron-specific enolase, synaptophysin, Leu 7 (CD57), cystatin C, CA15-3 and cytokeratin, the non-argyrophilic tumor cells were strongly immunoreactive for insulin and C-peptide. The stromal amyloid was clearly labeled for amylin. A few cells were stained for somatostatin, whereas other hormones were negative. Interestingly, a few isolated insulin-positive cells were identified in the non-neoplastic duodenal mucosa in the proximity of the tumor. Immunoelectron microscopy using paraffin sections disclosed insulin-immunoreactive secretory granules in the cytoplasm. The patient exhibited no signs or symptoms of hypoglycemia. Serum insulin levels were not measured prior to resection. No tumors were demonstrated in the pancreas. Magnetic resonance imaging revealed a 1 cm asymptomatic pituitary mass, in association with moderately elevated serum prolactin levels. The patient is currently being followed up in the outpatient clinic.
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PMID:Aberrant insulinoma of the duodenal bulb. 858 Nov 56

A subgroup of patients with metastatic carcinomas of unknown origin may benefit from combination chemotherapy. The relevance of immunohistochemistry in detecting such patients was investigated. Immunohistochemical studies with a panel of antibodies were performed on the tissue specimens of 41 patients having a light-microscopic diagnosis of poorly differentiated adenocarcinoma or undifferentiated carcinoma of unknown origin, who had been treated with cisplatin- containing chemotherapy. The study aimed to answer the following questions: (a) Can the tissue type of the tumor be verified? (b) Can a primary organ site be identified? (c) Can a prognostic immunohistochemical profile be recognized? The original diagnosis had to be changed in 2 of the 41 patients, who turned out to have a malignant lymphoma and neuroblastoma, respectively. The primary site was diagnosed in a patient with prostate cancer, whereas in one case the diagnosis could be narrowed down to a neuroendocrine tumor. No certain immunohistochemical profile with prognostic significance could be identified. It was concluded that immunohistochemistry should be routinely used in cases of undifferentiated carcinoma of unknown primary origin to verify the histological diagnosis and to select the appropriate therapy.
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PMID:The value of immunohistochemistry in patients with poorly differentiated adenocarcinomas and undifferentiated carcinomas of unknown primary. 860 68

Gastroenteropancreatic endocrine tumors are difficult to localize. At the same time the tumor is localized, though, there is an opportunity for cure or to remove tumor tissue. In this study we have prospectively examined the ability of 111In-octreotide scintigraphy, magnetic resonance imaging (MRI), and computed tomography (CT) to localize tumor lesions in 24 patients with a biochemical or histologic diagnosis of neuroendocrine tumor. In eight patients a surgical assessment of the imaging results was prospectively performed. Planar and abdominal single-photon emission tomography (SPET) images acquired 4 and 24 hours after 180 to 220 MBq of 111In-octreotide injection were evaluated and compared with conventional imaging techniques. SPET scintigraphy visualized more presumed tumor lesions (n = 39) than conventional imaging studies (MRI,n = 25; CT,n = 13); 23 of 24 patients had positive octreotide scintigraphy, 17 of 24 had positive MRI-scans, and 12 of 24 patients had positive CT scans. It was concluded that 111In-octreotide scintigraphy combined with conventional imaging improves the preoperative localization of presumably tumorous lesions in patients with gastroenterohepatic endocrine tumors.
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PMID:Somatostatin receptor localization of pancreatic endocrine tumors. 866 25

IA-2 is a transmembrane protein tyrosine phosphatase isolated recently from a human insulinoma subtraction library. Its expression in normal human tissues is restricted primarily to the pancreatic islets and brain. In this report, we describe the expression of IA-2 mRNA in a panel consisting of 20 lung tumor cell lines with neuroendocrine and non-neuroendocrine phenotype and 17 non-lung tumor cell lines. IA-2 mRNA was detected in 8 of 11 neuroendocrine small cell lung carcinomas, 4 of 4 non-small cell lung carcinomas with neuroendocrine phenotype, and 11 of 12 non-lung neuroendocrine tumor cell lines. In contrast, IA-2 mRNA was not detected in five non-neuroendocrine lung carcinomas, nor in a panel of other non-neuroendocrine tumor cell lines. The expression pattern of IA-2 mRNA suggests that IA-2 may represent a new marker for neuroendocrine differentiation In human lung cancer cells and perhaps other neuroendocrine tumors.
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PMID:IA-2, a transmembrane protein tyrosine phosphatase, is expressed in human lung cancer cell lines with neuroendocrine phenotype. 866 6

A 40-year old male patient presented with a history of subtotal strumectomy, excision of multiple cutaneous lesions at the upper trunk and gastrointestinal polyposis of unknown origin. The patient was admitted for weight loss and intermittent diarrhea. Physical examination revealed craniomegaly, papillomatosis of the oral mucosa and epigastric tenderness. Endoscopically, multiple polyps were seen in the stomach, the duodenum, the terminal ileum, the distal colon and the rectum. Histologically, these lesions were classified as hamartomatous and hyperplastic polyps. In the punctate of the relapsed nodular goitre, neoplastic follicular cells were found. These findings led to the diagnosis of Cowden's disease. A complete thyroidectomy was performed. The histology verified a follicular thyroid carcinoma and showed a combined expression of thyroglobulin and of the neuroendocrine marker synaptophysin (appr. 50% of all tumor cells). Chromogranin A (a neuroendocrine tumor marker) was also elevated in the serum of the patient. Postoperatively, a radioiodine therapy was performed and the clinical condition of the patient has improved ever since. The presented case of Cowden's disease is the first male patient with thyroid carcinoma. Early consideration of Cowden's disease is substantial as multiple malignant neoplasms may occur in this disorder in increased incidence.
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PMID:[Gastric hamartoma and thyroid gland carcinoma with follicular and neuroendocrine differentiation in Cowden syndrome]. 877 73

The aim of this article is to present a case of primary neuroendocrine tumor (typical carcinoid) of the mandible that occurred in a 46-year-old black woman who was seropositive for the human immunodeficiency virus. Radiologically the lesion presented as a poorly circumscribed honeycomb radiolucency that extended from tooth 21 to the ascending ramus. Histologically the tumor cells were variously arranged in small islands, trabeculae, follicles, and slitlike spaces lined by a single layer of palisaded low-columnar cells. The follicles contained an eosinophilic colloid-like substance. Immunocytochemical staining showed diffuse, intense positivity for MAK 6, pancytokeratin, S-100, and neuron-specific enolase and focal, intense, positive staining for chromogranin A. Electron microscopy showed the presence of interdigitating cell membranes, rudimentary cell attachments, and varying numbers of membrane-bound dense core granules. Special investigations failed to reveal a primary tumor, and no metastases were found. Urine and hematologic assessment did not show any evidence of functional activity. The tumor was resected, and no recurrence or spread has been seen for 2 years. Origin from foregut-derived, immature, and functionally uncommitted endocrine cells is presumed.
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PMID:Neuroendocrine (carcinoid) tumor of the mandible: a case report and review of the literature. 885 Apr 88

A 21 cm retroperitoneal cystic mass was excised from a 71 year old woman. The cyst was filled with a hemorrhagic fluid and contained a 5 cm parietal hemorrhagic nodule. On histology, the nodule was composed of a uniform population of round cells arranged in trabeculae and nests. The neoplastic cells were immunoreactive to cytokeratin, EMA, NSE, chromogranin A, pancreatic polypeptide (PP) and Gastrin (G). Ultrastructural observation of neurosecretory granules confirmed the neuroendocrine nature of the tumor. No other lesions were detected and a diagnosis of primary epithelial neuroendocrine tumor was rendered. The histogenesis of the tumor including the possibility of a paraganglionic origin is discussed.
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PMID:Retroperitoneal cystic neuroendocrine tumor. A case report. 886 85

Malignant transformation in bile duct hamartomas has been previously reported in very rare instances. Here, we describe a unique case of a neuroendocrine tumor of the liver arising within an area of unusually large hamartoma with predominant bile duct component, hitherto unreported and distinct from the conventional von Meyenburg complex. The tumor was apparently secreting gastrin and chromogranin, with associated gastrinoma syndrome over several years. The histologic picture was reminiscent of a moderately differentiated adenocarcinoid, with positive mucin staining in a signet ring pattern. Tumor cells showed positive staining for neuron-specific enolase, chromogranin A, gastrin, and serotonin. Staining for pancreatic hormone peptides was negative. Resection of the tumor was apparently curative, with complete resolution of the patient's symptoms.
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PMID:Adenocarcinoid of the liver arising within an area of hamartoma with predominant bile duct component. 887 46

A 62-year-old woman was examined with In-111 pentetreotide and Ga-67 citrate. She had undergone an operation to resect a neuroendocrine tumor of the pancreas and still had masses in the liver. One of her hepatic lesions had been biopsied and acinar cell carcinoma was suspected. Fluid in the cyst of the tumor, however, contained a high concentration of gastrin and the tumor was strongly suspected of being a metastasis from the neuroendocrine tumor of the pancreas. The hepatic tumors quickly accumulated In-111 pentetreotide immediately after the injection, but there was no Ga-67 citrate uptake in the tumor. Five months after pentetreotide scintigraphy, her hepatic tumors were resected and histologically proven to be metastasis of islet cell carcinoma. In-111 pentetreotide provides information of the somatostatin-receptor status on the tumor and supports the diagnosis made by hormonal survey.
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PMID:Early massive accumulation of In-111 pentetreotide in a metastatic liver tumor of islet cell carcinoma. 888 11


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