Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present the case of a patient with Cushing's syndrome secondary to ectopic ACTH secretion. A MR of the head showed a left-sided nasal mass extending down from the cribriform plate. The patient underwent endoscopic resection with nearly complete removal of the mass. Histological examination showed an ACTH-secreting olfactory neuroblastoma (ONB). The patient's cortisol levels returned to normal range after surgery and have remained normal for over a year. ONB is a rare cause for ectopic ACTH secretion. This case highlights the diagnostic and management difficulties in patients with ectopic ACTH secretion, and provides a brief review of ONB.
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PMID:Ectopic Cushing's syndrome secondary to olfactory neuroblastoma. 2934 Jul 76

Recent development of imaging tools such as computed tomography, ultrasonography, and magnetic resonance imaging have incidentally discovered hormonally functioning or nonfunctioning adrenal tumors. Most adrenal medullary tumors are pheochromocytomas and neuroblastoma group tumors. They are representative of neuroendocrine tumors and can be diagnosed using neuroendocrine markers such as chromogranin A, synaptophysin, and neurofilament proteins. Catecholamine-synthesizing enzymes are also useful markers for these catecholamine-producing tumors. Both pheochromocytoma and neuroblastoma group tumors have cells that are immunohistochemicaJly positive for many peptide hormones including m-enkephalin, neuropeptide Y, somatostatin, vasoactive intestinal peptide, corticotropinreleasing hormone, adrenocorticotropic hormone, calcitonin, and calcitonin gene-related peptide, among others. The evidence for production of these hormones is confirmed by mRNA analysis using in situ hybridization or Northern blot hybridization and by measuring protein levels with radioimmunoassay. Only a limited number of patients, however, complain of clinical symptoms associated with excessive peptide hormone production such as watery diarrhea, hypokalemia, and achlorhydria syndrome or Cushing's syndrome. The monoclonal human neuroblastoma cell line (NB-1) is a good model by which to understand the mechanism of excessive hormone production. NB-1 cells are usually nonfunctioning, but when they are stimulated by cyclic adenosine monophosphate and phorbol ester, they become capable of production and release of many peptide hormones and undergo morphological changes in their endocrine features. Thus, microenvironmental change seems to be one of the factors regulating gene expression and hormone production. Some molecular studies of oncogenes and growth factors are reviewed to gain an understanding of cell differentiation and proliferation. Finally, several chromosomal abnormalities reported in multiple endocrine neoplasia are introduced as potential tumorigenic factors.
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PMID:Functioning and nonfunctioning adrenal medullary tumors. 3235 8

A 40-year-old woman who had a history of recurrent olfactory neuroblastoma presented with full moon face, central obesity, buffalo hump, impaired glucose tolerance and bilateral cervical lymph node swelling. Laboratory tests showed morbidly elevated levels of ACTH and cortisol, which were not suppressed by high-dose (8 mg) dexamethasone. Biopsies of the enlarged cervical lymph nodes revealed adrenocorticotropic hormone (ACTH)-positive metastatic olfactory neuroblastoma, and ectopic ACTH syndrome was diagnosed. Metyrapone was used to suppress cortisol production and resulted in decreased levels of ACTH and cortisol. Bilateral cervical tumor resection further reduced the ACTH and cortisol levels, accompanied by a reduction in the metyrapone dosage. Cushing's syndrome was alleviated through ACTH-producing tumor removal.
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PMID:A Rare Case of Ectopic Adrenocorticotropic Hormone Syndrome with Recurrent Olfactory Neuroblastoma. 3292 82


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