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

We report a series of 7 glucagonoma patients seen between 1994 and 2001, 5 males and 2 females, aged 32-69 years, with: necrolytic migratory erythema (NME) (n = 2), liver metastases (n = 3), jaundice (n = 1) and 1 case of familial history of multiple endocrine neoplasia. The diagnosis combined histology and hyperglucagonemia; 6 patients developed metastasis (5 initially); during the follow-up 3 developed a necrolytic erythema migraticum (NEM) worsening the general status. Somatostatin receptor scanning was highly positive in all. Four patients were operated, 5 received chemotherapy (2 OR and 2 SD), 3 had chemoembolization (1 transient improvement). Somatostatin was efficient on general status or skin lesions in all patients. Two died and 5 are alive with a follow up ranging from 12 to 60 months. We want to emphasize on the higher frequency than expected of this disease, the frequency of NEM, the efficacy of SMS on NEM and general status and on the fairly good prognosis. The high uptake of SMS by tumors on scanning could rise hopes about radioconjugate therapy.
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PMID:[Glucagonoma: a recent series of 7 cases]. 1538 54

Gastric carcinoids are rare neuroendocrine tumors, usually classified as type I, if associated with atrophic body gastritis; type II, if associated with Zollinger-Ellison syndrome and multiple endocrine neoplasia type I, and type III, in the absence of any gastric pathology (sporadic tumors). The pathological features, as well as the prognosis of the tumor and the patient's survival strictly depend on this classification. The correct management of the patient with gastric carcinoid can only be proposed when the tumor has been classified by an accurate pathological and clinical evaluation of the patient. While the therapeutic approach in types I and II is based on a conservative strategy, including endoscopic resection, an adequate follow-up program, and the possible use of somatostatin analogues, an aggressive surgical approach is required in type III.
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PMID:Gastric neuroendocrine tumors. 1547 10

Early identification of pancreaticoduodenal endocrine tumors (PETs) in multiple endocrine neoplasia type 1 (MEN-1) is mandatory, because these tumors represent the most common cause of death within the syndrome. The diagnostic value of imaging procedures has therefore been evaluated in a prospective observational study. Between December 1997 and June 2003 twenty-two MEN-1 patients with genetically confirmed disease were followed for PETs using a standardized screening program with serum hormone measurements, endoscopic ultrasonography (EUS), computed tomography (CT), and somatostatin-receptor scintigraphy (SRS). Results could be validated by surgery and histopathology in 13 patients during 18 operations. In 12 asymptomatic patients with tumors measuring 10 mm or less, who have not yet undergone operation, PETs were detected by EUS in 12/12, by CT in 1/12, and by SRS in 2/11 cases. In 13 patients who have undergone surgical exploration EUS, CT, and SRS were true positive in 12 of 16, 7 of 13, and 12 of 17 cases, respectively, although the number of tumors detected by each imaging procedure alone was lower than the number detected intraoperatively and histopathologically in almost every case. A solitary liver metastasis in one patient and a nonfunctioning PET recurrence in another were identified only by SRS. Endoscopic ultrasonography is the most sensitive imaging procedure for the detection of small (< or = 10 mm) PETs in MEN-1, whereas SRS is the procedure of choice for the identification of metastases of MEN-1 PETs-i.e., for staging. Detection of PETs at an early stage by an aggressive screening program using EUS may lead to prompt surgical intervention and improved prognosis of MEN-1 PETs.
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PMID:Prospective evaluation of imaging procedures for the detection of pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. 1551 79

Multiple endocrine neoplasia syndrome type II-A (MEN-IIA) is a rare endocrinological disorder occurring in 0.04% of the general population. The combination of papillary thyroid carcinoma with MEN-IIA appears even less frequently. We describe the case of a 21-year-old woman with pheochromocytoma of the left adrenal gland, medullary thyroid carcinoma, hyperplasia of the parathyroid glands and papillary thyroid carcinoma. MEN II-A syndrome resulted from de novo mutation of the RET proto-oncogene, which was detected in the DNA of peripheral blood leucocytes. Three months postoperatively calcitonin levels were normal, whilst increased serum thyroglobulin values prompted the need for further investigation. Whole body scanning with (131)I and with (99m)Tc-sestamibi and also US test of cervical lymph nodes, were negative. The synthetic analogue of somatostatin (99m)Tc-depreotide was used for whole body scintigraphy, cervical and thoracic tomographic scanning and revealed anterior cervical, upper mediastinal and right hilar foci of pathological uptake[Fig.1 and Table 1: see text]. These findings were compatible with findings from CT and MRI that followed in order to complete the diagnostic evaluation. The patient underwent surgical resection of the metastatic foci with uneventful postoperative course. Histology showed lymph node metastases originating from the papillary thyroid carcinoma. Ratio values >2 were abnormal (Fig. 2). Computer processing of the corresponding ROIs on healthy tissues produced the following normal values: Th/Arm: 1.874,, Med/Arm: 1.699, Hi/Arm: 1.141 (Fig. 3).
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PMID:[A patient with MEN-IIA syndrome due to de novo mutation and papillary thyroid carcinoma; the role of 99m Tc-depreotide in diagnosing metastases and brief review of the literature]. 1584 Dec 92

Octreotide is one of the somatostatin analogue used for the treatment of endocrine tumors principally to suppress hormone secretion and to inhibit tumor growth. We experienced a case with multiple endocrine neoplasia type 1 who small amount of octreotide dramatically relieved the lumber pain caused by metastatic bone tumor. He had recurrent bronchial carcinoid tumors that metastasized to liver and bones. The spontaneous and radiated pain by bone tumors subsided within a few minutes after the initial injection of octreotide and the effect persisted for several hours. Combination therapy of octreotide and interferon alpha-2b significantly reduced the size of metastatic liver tumors and inhibited further growth of metastatic bone tumors for the last 27 months. The use of octreotide may be a good option for controlling pain by metastatic bone disease and combination therapy of octreotide and interferon alpha-2b is worth to try for patients with inoperable metastatic carcinoid tumor.
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PMID:Octreotide as a rapid and effective painkiller for metastatic carcinoid tumor. 1586 61

Zollinger-Ellison syndrome (ZES) is characterised by refractory peptic ulcer disease, severe diarrhoea and gastric acid hypersecretion associated with an islet-cell tumor of the pancreas (gastrinoma). ZES is sporadic in 62-80% of cases and in 20-38% of cases is associated with multiple endocrine neoplasia type 1 (MEN 1). The diagnosis of ZES is certain when the plasma gastrin is >1000 pg/mL and the basal acid output is >15 mEq/h in patients with an intact stomach, >5 mEq/h in gastrectomised patients, or when the hypergastrinemia is associated with a pH <2. Treatment is based on the control of gastric acid hypersecretion and of the malignant tumor and its possible metastases. Proton pump inhibitors are the most effective antisecretory drugs and can be administered at high dosages without drug-related adverse effects. All sporadic, localised gastrinomas should be excised if possible. When liver metastases are also present, their debulking may improve symptoms and survival, and facilitate medical treatment. There is some controversy as to the surgical approach for gastrinomas associated with MEN 1. Somatostatin analogues can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric enterochromaffin-like cells, and can thus contribute to treating the disease more effectively. Their antiproliferative effect can be used in treating liver metastases. Chemotherapy and/or interferon are indicated only in patients with malignant progressive disease. Embolisation and chemoembolisation are effective in controlling clinical symptoms; however, they do not seem to improve survival.
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PMID:Zollinger-Ellison syndrome. Diagnosis and therapy. 1617 61

Gastric endocrine tumours (gastric carcinoids) usually grow from enterochromaffin-like (ECL) cells. Three types of tumour may be distinguished on the basis of the background gastric pathology: type I, which develops in atrophic body gastritis (ABG); type II, which is associated with multiple endocrine neoplasia and Zollinger-Ellison syndrome; and the sporadic type III, which is not associated with any background pathology. This classification plays a major role in determining the optimal approach to these diseases. In fact, type I carcinoids can be considered to be benign lesions, with exceptional risk of metastases. Type II, in contrast, may be associated with distant metastases, which are also common in type III carcinoids. The therapeutic approach is based mainly on endoscopic excision and somatostatin analogues in types I and II, or on surgical resection in type III. Both types I and II grow under the stimulus of hypergastrinaemia through a well-described sequence. However, gastrin is sufficient to cause ECL cell hyperplasia and dysplasia, but not transformation, which is due to menin defects in MEN-I patients, or to other unknown alterations in ABG. Several other candidates--including Bcl2, p53 and MMP9--have been linked with carcinoid initiation and progression. The biology of type III tumours which are not associated with hypergastrinaemia is still poorly understood.
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PMID:Endocrine tumours of the stomach. 1625 92

Endocrine pancreatic tumours (EPTs) are uncommon tumours occurring in approximately 1 in 100,000 of the population, representing 1-2% of all pancreatic neoplasms. Some of the tumours may be part of multiple endocrine neoplasia type one (MEN-1) syndrome or von Hippel-Lindau (vHL) disease. EPTs are classified as functioning or non-functioning tumours on the basis of their clinical manifestation. The biochemical diagnosis of EPT is based on hormones and amines released. Besides specific markers such as insulin, there are also general tumour markers such as chromogranin A, which is the most valuable marker and has been reported to be increased in plasma in 50-80% of patients with EPTs and correlates with tumour burden. The location of endocrine tumours of the pancreas includes different techniques, from endoscopic investigations to scintigraphy (e.g. somatostatin receptor scintigraphy) and positron emission tomography. The medical treatment of endocrine pancreatic tumours consists of chemotherapy, somatostatin analogues and alpha-interferon. None of these can cure a patient with malignant disease. In future, therapy will be custom-made and based on current knowledge of tumour biology and molecular genetics.
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PMID:Endocrine tumours of the pancreas. 1625 99

Zollinger-Ellison syndrome (ZES) is characterised by peptic ulcers of the upper gastrointestinal tract failing to heal despite maximal medical therapy, diarrhoea and marked gastric acid hypersecretion associated with a gastrin-secreting tumour (gastrinoma). ZES might be associated with multiple endocrine neoplasia type 1. The main diagnostic features are hypergastrinemia and acid hypersecretion. When these parameters give borderline results, provocation test (with secretin or calcium) may be required. To identify the localisation of gastrinoma several imaging techniques have been proposed. Somatostatin receptor scintigraphy is capable to localise the tumour in 80% of the cases and to identify it even in anatomic sites other than pancreas and duodenum. Endoscopic ultrasonography has a sensitivity as high as 79-93% and a specificity of 93%. The 2 main principal therapeutic strategies are to control both the gastric acid hypersecretion and the growth of the neoplasia. Proton pump inhibitors (PPIs) are the drugs of choice for patients with ZES. Furthermore, safety of PPIs in the maintenance therapy has been proven both in short- and in long-term studies. The best surgical treatment is excision of gastrinoma before metastatic spread has occurred. Somatostatin-analogues can reduce both gastric acid hypersecretion and serum gastrin levels. Moreover, they have an antiproliferative effect. Chemotherapy, interferon and embolisation are indicated in rapidly evolving tumours or in cases in which the tumoral symptoms cannot be treated by other approaches.
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PMID:Zollinger-Ellison syndrome in 2006: concepts from a clinical point of view. 1663 30

Gastric carcinoid tumors are uncommon, but their percentage among all gastric malignancies has increased to 1.8%. Although they are most often discovered incidentally during endoscopy, gastric carcinoids can present with abdominal pain, bleeding, or symptoms related to the secretion of bioactive substances, most commonly histamine. Gastric carcinoids originate from the foregut and are derived from histamine-containing enterochromaffin-like (ECL) cells. Type I gastric carcinoid, the most common, exhibits slow growth and benign behavior. It occurs within the setting of chronic atrophic gastritis with achlorhydria-induced hypergastrinemia. Gastrin acts directly on ECL cells to induce hyperplasia, dysplasia, and, eventually, neoplasia. Type II gastric carcinoid, the least common type, occurs in patients with gastrinoma-associated multiple endocrine neoplasia syndrome-type 1 (MEN-1). The overall survival is related more to the underlying MEN-1 syndrome than to the gastric carcinoid. Rodents readily develop gastric carcinoid tumors in response to hypergastrinemia. However, in humans, other factors in addition to hypergastrinemia, such as pernicious anemia or MEN-1, must be present, implying that a genetic predisposition is necessary for the development of these tumors. Type III or sporadic gastric carcinoids exhibit a more malignant behavior, with overall 5-year survival rates of less than 50% and normal serum gastrin concentrations. Treatment of all types of gastric carcinoids is predicated upon accurate classification and staging. Radiolabeled somatostatin analogues are superior to conventional radiologic imaging techniques in detecting both primary and metastatic lesions. Treatment of choice for localized disease is excision, either endoscopically or surgically. Antrectomy, by eliminating the trophic effect of gastrin, can be useful for select type I carcinoids. Long-acting somatostatin analogues are excellent palliative agents.
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PMID:Treatment of gastric carcinoids. 1739 27


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