Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P61278 (somatostatin)
 22,083 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In-111 pentetreotide scintigraphy of 10 patients with residual or metastatic medullary thyroid carcinoma is described. Six patients had sporadic tumor and 4 had MEN IIB. Foci of increased tracer uptake were observed in 9 patients: in the thyroid bed (4 patients), the mediastinum (3 patients.), the shoulder area and left lower abdomen (1 patient), and the left upper abdomen (1 patient). The 10th patient had no abnormal uptake. CT confirmed 2 mediastinal lesions and 2 out of 3 thyroid masses, but did not detect the thyroid remnants or the lesions in the shoulder area and abdomen. Lung lesions < or = 1 cm in diameter and ill-defined liver foci (2 patients) were seen on CT, but not on scintigraphy. Small liver metastases not demonstrated on CT or on scintigraphy were identified at surgery in a MEN IIB patient. Elevated urinary epinephrine was found in 2 out of 4 MEN IIB patients. In one, tracer uptake in the left adrenal corresponded to a mass on CT, to pathological uptake of MIBG and DMSA, and to a tumor removed at surgery. The second patient had peritoneal spread of malignant pheochromocytoma (at surgery), but negative CT and only a single focus in the left lower abdomen on scintigraphy. Somatostatin-receptor imaging is useful for the detection of residual and recurrent medullary thyroid carcinoma, and may identify pheochromocytoma in MEN IIB patients.
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PMID:Somatostatin-receptor imaging of medullary thyroid carcinoma. 791 71

This is a consensus statement from an international group, mostly of clinical endocrinologists. MEN1 and MEN2 are hereditary cancer syndromes. The commonest tumors secrete PTH or gastrin in MEN1, and calcitonin or catecholamines in MEN2. Management strategies improved after the discoveries of their genes. MEN1 has no clear syndromic variants. Tumor monitoring in MEN1 carriers includes biochemical tests yearly and imaging tests less often. Neck surgery includes subtotal or total parathyroidectomy, parathyroid cryopreservation, and thymectomy. Proton pump inhibitors or somatostatin analogs are the main management for oversecretion of entero-pancreatic hormones, except insulin. The roles for surgery of most entero-pancreatic tumors present several controversies: exclusion of most operations on gastrinomas and indications for surgery on other tumors. Each MEN1 family probably has an inactivating MEN1 germline mutation. Testing for a germline MEN1 mutation gives useful information, but rarely mandates an intervention. The most distinctive MEN2 variants are MEN2A, MEN2B, and familial medullary thyroid cancer (MTC). They vary in aggressiveness of MTC and spectrum of disturbed organs. Mortality in MEN2 is greater from MTC than from pheochromocytoma. Thyroidectomy, during childhood if possible, is the goal in all MEN2 carriers to prevent or cure MTC. Each MEN2 index case probably has an activating germline RET mutation. RET testing has replaced calcitonin testing to diagnose the MEN2 carrier state. The specific RET codon mutation correlates with the MEN2 syndromic variant, the age of onset of MTC, and the aggressiveness of MTC; consequently, that mutation should guide major management decisions, such as whether and when to perform thyroidectomy.
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PMID:Guidelines for diagnosis and therapy of MEN type 1 and type 2. 1205 Feb 90

Medullary thyroid carcinoma (MTC) is a relatively rare neuroendocrine tumour originating from the parafollicular C cells and releases calcitonin (hCt), carcinoembryonic antigen (CEA) and occasionally other substances. In 20-30% of cases MTC presents a germline mutation of the RET proto-oncogene and occurs in 3 different hereditary forms: familial MTC, multiple endocrine neoplasia (MEN) 2A and MEN 2B syndrome. Prognosis of MTC is largely related to tumour extension at disease onset. Surgery remains the most effective therapy for potential cure. Overall survival is strictly linked to the occurrence of relapse. After surgery, serum hCt remains the most sensitive test for occult disease. Diagnostic imaging work-up includes ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), bone scintigraphy, as the more frequent sites of recurrence or metastases are cervical and mediastinal lymph nodes, lungs, liver and bone. Nuclear medicine procedures include (111)In-labelled somatostatin analogs, radioiodinated metaiodobenzylguanidine (MIBG), and several PET radiopharmaceuticals. Experience with radionuclide therapy in MTC is restricted to few patients treated with (131)I-MIBG or (90)Y-DOTATOC. Since 1987, 1 027 diagnostic MIBG scans were performed in the Department Department of Diagnostic Imaging and Therapy of the Istituto Nazionale Tumori IRCCS Foundation (Milan, Italy), 85 of which for MTC, with a sensitivity of 38.7% in patients with evidence of disease and 30.7 % if all patients were considered. Since 1994, 13 MTC patients were treated with MIBG with 4 partial responses and 4 stable diseases. Patients with liver or bone involvement responded to therapy and showed long-term partial remission of disease, others showed stability of disease, which was apparently unrelated to therapy. Improvement of efficacy can be achieved through dosimetric calculation of administered activity.
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PMID:MIBG for diagnosis and therapy of medullary thyroid carcinoma: is there still a role? 1908 96