Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01189 (beta-endorphin)
 21,003 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neoplasms of the larynx showing neuroendocrine differentiation are uncommon, with less than 100 previously reported cases. These tumors encompass subcategories that have been designated heretofore as "malignant carcinoid" and "oat cell carcinoma" and have been associated with ectopic production of neuropeptides and biogenic amines. We report a case of neuroendocrine carcinoma that arose in the epiglottis in an elderly man, metastasized widely, and was responsible for death. Immunohistochemical studies showed the presence of serotonin, calcitonin, ACTH, met-enkephalin, chromogranin, and neuron-specific enolase within tumor cells, although clinical endocrinopathy was absent. This case is used as a focus for discussion of the spectrum of neuroendocrine tumors of the head and neck, including recommended treatment approaches for such neoplasms.
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PMID:Neuroendocrine carcinoma of the larynx. 243 56

A neuroendocrine carcinoma of the thymus with an ectopic adrenocorticotropic hormone (ACTH) syndrome and melanocytic differentiation is described. ACTH, neuron-specific enolase (NSE) and S-100 protein were identified in the tumor by immunocytochemistry. Neurosecretory granules and melanosomes could be demonstrated in different cell populations by electronmicroscopy. The clinicopathologic findings are presented. The literature is briefly discussed.
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PMID:Melanocytic neuroendocrine carcinoma of the thymus. 302 5

Since the initial clinical presentation of visceral neuroendocrine carcinoma is occasionally a cutaneous metastasis, diagnostic confusion with primary neuroendocrine carcinoma of the skin (Merkel cell carcinoma) may ensue. In this study, seven cases of secondary cutaneous neuroendocrine carcinoma were immunohistochemically compared with twenty-one Merkel cell carcinomas for ten antigenic moieties that have been associated with endocrine tumors. Six of seven secondary tumors stained for bombesin, leucine enkephalin, methionine enkephalin, or beta-endorphin, none of which was detected in the primary cutaneous neuroendocrine carcinomas. These data suggest that immunohistochemical study may be useful in separating primary from secondary neuroendocrine tumors of the skin and may assist in directing clinical attention to the most probable site of visceral neoplasia.
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PMID:Secondary neuroendocrine carcinomas of the skin. An immunohistochemical comparison with primary neuroendocrine carcinoma of the skin ("Merkel cell" carcinoma). 316 11

A 58-year-old woman with a history of Cushing's syndrome for three years presented with a mediastinal mass and received the diagnosis of small cell neuroendocrine carcinoma of the thymus invading the pericardium. On immunohistochemical study, the neoplastic cells reacted with antibodies against cytokeratin, epithelial membrane antigen, neuron-specific enolase, chromogranin, synaptophysin, and ACTH. Clinicopathologic findings of this rare case of ectopic adrenocorticotropic hormone (ACTH) syndrome are discussed with a literature review.
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PMID:Small cell neuroendocrine carcinoma of the thymus complicated by Cushing's syndrome. Report of a 58-year-old woman with a 3-year history of hypertension. 1126 17

Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome is a rare disease. Recent evidence suggests that these tumors form part of a continuous spectrum ranging from well-differentiated carcinomas to small cell carcinomas. We report two new cases and review the 23 cases reported in the literature since 1972. The different diagnostic modalities are discussed, and an algorithm for the diagnosis of ectopic secretion of adrenocorticotropin (ACTH) is presented. In the future, the advent of radiologic and nuclear imaging as well as more accurate workup should help to diagnose these tumors at an earlier stage and improve the long-term outcome.
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PMID:Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome. 1260 97

Although ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a well-known paraneoplastic phenomenon, an association with large-cell neuroendocrine carcinoma of the lung (LCNEC) has not been reported. We describe a 63-year-old man with metastatic LCNEC to the left temporomandibular joint (TMJ) who presented with progressive muscle weakness and bilateral lower leg edema for 2 weeks. He did not have a typical Cushingoid appearance nor used diuretics. His newly noted hypertension, hypokalemia (plasma potassium (K) concentration 1.8 mEq/L) with renal K wasting, and metabolic alkalosis suggested a state of mineralocorticoid excess. His plasma renin activity and aldosterone concentrations were low, but cortisol and ACTH levels were extremely elevated, consistent with ACTH-dependent Cushing's syndrome. Nonsuppressible plasma cortisol level and normal sella turcica on magnetic resonance imaging pointed to EAS. A strongly positive stain for ACTH from the metastatic left TMJ mass supported LCNEC-related EAS. His hypokalemia and hypertension were controlled with spironolactone and K supplementation. This is the first reported case of EAS in LCNEC and should be kept in mind as a cause of hypokalemia in lung cancer patients.
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PMID:Ectopic ACTH syndrome associated with large-cell neuroendocrine carcinoma of the lung. 1809 71

Thymomas constitute majority of the thymic neoplasms. In contrast, neuroendocrine tumors (carcinoid and neuroendocrine carcinoma) of thymus are extremely rare. Thymic carcinoids may present rarely with Cushing's syndrome due to the ectopic production of adrenocorticotropic hormone (ACTH). Recognition of this association is imperative for appropriate management of patients. We describe three cases of rare atypical carcinoid tumor (neuroendocrine carcinoma) of the thymus. Case 1, of a 26-year-old man presenting with Cushing's syndrome, case 2--a 23-year-old female with Cushingoid features, and Case 3--a 39-year-old man complaining of progressively worsening dyspnea. Computed tomography (CT) scans of chest in all three patients revealed anterior mediastinal mass. Excision of tumors and histological examination of the three tumors showed a carcinoid tumor with nuclear pleomorphism, increased mitotic activity and focal necrosis. The features suggested a diagnosis of atypical carcinoid tumor in all the three cases. The tumor cells in Cases 1 and 2 showed focal immunohistochemical staining for ACTH. Atypical carcinoid (neuroendocrine carcinoma, well-differentiated and moderately-differentiated) of the thymus is a rare thymic tumor which carries a worse prognosis compared to thymoma and requires aggressive therapy. Hence, an accurate diagnosis is essential.
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PMID:Primary neuroendocrine carcinoma of thymus: a rare cause of Cushing's syndrome. 2009 Feb 49

Malignancies of the gallbladder, including neuroendocrine tumors, are uncommon, mostly found incidentally after cholecystectomy and are frequently asymptomatic in the early stages, but highly fatal. Limited data is available on adrenocorticotropic hormone (ACTH)-producing neuroendocrine tumors specifically originating from the gallbladder. We report the clinical and radiographic findings, which included positron emission tomography and computed tomography, of a patient with a gallbladder mass who presented with Cushing's syndrome. Subsequently, a diagnosis of ACTH-producing large cell neuroendocrine carcinoma of the gallbladder was made. Despite being rare and having a poor prognosis, hormone-producing neuroendocrine tumors should be part of the differential diagnosis in the approach of patients with Cushing's syndrome.
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PMID:Cushing's syndrome caused by an ACTH-producing large cell neuroendocrine carcinoma of the gallbladder. 2116 Aug 18

We report the case of a 36-year-old woman with Cushing's syndrome caused by a malignant unresectable neuroendocrine carcinoma of the pancreas that developed bilateral ovarian metastases 7 years after diagnosis. In November 2001, because of abdominal pain and jaundice, the patient underwent radiological investigations and exploratory laparotomy that demonstrated the presence of a 3-cm mass of the head of the pancreas, infiltrating the superior mesenteric vein, associated with enlargement of multiple abdominal lymph nodes and with a liver nodule. Histological examination of one lymph node and of the liver nodule demonstrated the presence of metastases from a well-differentiated neuroendocrine carcinoma showing corticotropin immunoreactivity. A few months later, the patient started to show the clinical symptoms of Cushing's syndrome and underwent steroid-blocking ketoconazole therapy. The clinical endocrine picture was controlled until the end of 2008, when the endocrine symptoms of the Cushing's syndrome worsened and bilateral ovarian tumors appeared. Hysteroannexectomy was performed and ovarian tumors were found to be metastases from a well-differentiated neuroendocrine carcinoma with morphological and immunohistochemical features overlapping those observed in 2002. The clinical situation worsened and the patient died in November 2009. The clinical aspects and the problems in the differential diagnosis are discussed.
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PMID:Cushing's syndrome due to a pancreatic neuroendocrine tumor metastatic to the ovaries: a clinicopathological description of a case. 2139 May 54

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) have been rarely reported in patients with Crohn's disease, being usually small and incidentally detected in areas uninvolved by the inflammatory process. We describe the case of a young female patient with Crohn's disease and a fulminant Cushing's syndrome induced by the ectopic secretion of adrenocorticotropic hormone (ACTH) by an aggressive gastrointestinal neuroendocrine carcinoma (NEC). Despite a multi-therapeutic approach, including the administration of multiple courses of chemotherapy, hypo-cortisolemic agents, somatostatin analogues, as well as the performance of bilateral adrenal vein embolization followed by bilateral adrenalectomy, patient's condition progressively deteriorated and she died nine months after the diagnosis of NEC due to liver failure. The available literature addressing the possible connection between Crohn's disease and NEC is discussed in detail.
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PMID:Fulminant ectopic Cushing syndrome in a patient with metastatic neuroendocrine carcinoma and Crohn's disease. 2228 92


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