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)

This paper aims at describing the neuroendocrine cell growths of the gastric mucosa and their pathogenesis. In the corpus-fundus mucosa, gastric neuroendocrine nontumor growths are mostly represented by hyperplastic and, more rarely, dysplastic enterochromaffin-like (ECL) cell changes, while hyperplasia of gastrin-producing (G) cells and, rarely, of somatostatin-producing (D) cells are reported in the antral mucosa. The large majority of gastric neuroendocrine tumors is made by benign, gastrin-dependent, well-differentiated ECL cell growths arising in a background of chronic atrophic gastritis (type I) or, more rarely, associated with type I multiple endocrine neoplasia (MEN I) and Zollinger-Ellison (ZE) syndromes (type II). Rare, aggressive, frequently metastatic, well-differentiated gastric neuroendocrine tumors are gastrin-independent and arise as sporadic lesions in the absence of specific gastric pathology (type III). Poorly differentiated neuroendocrine carcinomas (PDEC) are rare, highly aggressive carcinomas. A central role for gastrin is postulated in the pathogenesis of well-differentiated type I and II ECL cell tumors with different possible genetic mechanisms. A more complex genetic background, independent of gastrin and possibly implicating altered function or mutation of p53 and other genes is highly suspected for the development of aggressive type III ECL cell carcinomas and PDECs.
 ...
PMID:Morphological, molecular, and prognostic aspects of gastric endocrine tumors. 1073 15

The preoperative evaluation and safe anesthetic treatment of patients with endocrine gland tumors mandate an understanding of the physiologic dysfunctions attributable to these tumors. Some patients will exhibit various signs and symptoms characteristic of the MEN syndromes. In the patient with acromegaly, a fiberoptic-guided intubation of the trachea to secure the airway before induction of general anesthesia must be anticipated. Anesthetic treatment of the patient with hyperadrenocorticism requires knowledge of the physiologic effect of excess cortisol. In the patient with severe hyperparathyroidism, we attempt to correct the markedly elevated plasma calcium levels and maintain adequate hydration and urine output perioperatively. Following thyroidectomy for MCT, 2 potential problems of concern are upper airway obstruction and aspiration resulting from injury (unilateral or bilateral) to the recurrent laryngeal nerve and the superior laryngeal nerve, respectively. The major focus during excision of an insulinoma is prevention of wide swings in blood glucose concentrations. In the gastrinoma patient, the anesthesiologist not only must correct any intravascular fluid volume deficit or electrolyte imbalance but must also consider the patient to have a full stomach at the time of anesthetic induction. Correction of hypokalemia and control of hypertension may be required in the preoperative preparation of the patient with an adrenal cortex tumor. Preoperative alpha-adrenergic blockade must be initiated in the patient with a pheochromocytoma to prevent dangerous elevations in blood pressure during anesthesia and surgery for the tumor's removal. Vasodilators with rapid onset and short duration are used to treat intraoperative hypertension. After ligation of the tumor's blood supply, falls in blood pressure may require treatment with fluids and vasopressors. Carcinoid syndrome patients should be treated with somatostatin to prevent stimuli such as anxiety, abdominal scrubbing, or tumor manipulation from precipitating severe hypotension, hypertension, bronchospasm, or tachycardia. In both pheochromocytoma and carcinoid patients, a smooth anesthetic induction and tracheal intubation plus avoidance of drugs that release histamine or activate the sympathetic nervous system may also prevent intraoperative crises.
 ...
PMID:Anesthetic implications for surgical patients with endocrine tumors. 1081 14

Neuroendocrine enteropancreatic tumors (NEEPTs) include tumors developing from the pancreatic islets and the duodenal loop, and those developing from gut serotonin-secreting cells (mid-gut carcinoids). When functioning, they are rare tumors with frequently inconsistant clinical and biological characteristics. Provocative dynamic tests are very useful. Their natural history, with the exception of insulinomas and mid-gut carcinoids, is still incompletely known due to their rarity, relatively recent description, slow evolution and the absence of definitive reliable markers for prognosis. The following factors affect the natural history of NEEPTs. (1) Inclusion or not within the spectrum of multiple endocrine neoplasia type-1 (MEN1), such an integration often dramatically modifies the management strategy. In patients with MEN1, NEEPTs are often multiple, diffusely spread in the duodenopancreatic area and of small size. Definite cure by surgery is then very scarce. Mid-gut carcinoid tumors are the most protean NEEPTs. In contrast to appendiceal carcinoids, usually with a benign course, mid-gut carcinoids have a more malignant course and are very common indeed: local transmural invasion, early metastasis to lymph nodes and/or liver, and symptoms from hormone oversecretion. Carcinoid syndrome is usually well controlled by long-acting somatostatin analogs. (2) Disease spreading, i.e. liver and bone metastases, are the major causes of death in patients with NEEPTs. Liver metastases (LMs) are synchronous in about 75% of the patients with LMs but may develop during follow-up (about 25% of the patients with LMs were without LMs at the initial evaluation). Development of bone metastases has a very poor prognosis accompanying LM progression.
 ...
PMID:Natural history of neuroendocrine enteropancreatic tumors. 1094 Jun 88

The authors describe the case of a 51-year-old male with Zollinger-Ellison syndrome manifested by epigastralgia, nausea, vomiting, hypergastrinemia and multiple endocrine neoplasia type 1. History included a Billroth II procedure for a perforated duodenal ulcer. Multiple metastatic liver lesions were found that were gastrin-negative and chromogranin-positive. Endoscopy revealed a large ulcerated gastro-jejuno-colonic fistula which was surgically repaired. Pre- and postoperative imaging studies, including the highly sensitive somatostatin-receptor scintigraphic scan using In-pentetreotide, have consistently failed to disclose other tumors. Recent reports indicate that most Zollinger-Ellison syndrome-associated gastrinomas are small, easily overlooked lesions located in the proximal duodenum rather than in the pancreas as formerly believed. In the present patient therapy with omeprazole and alpha-interferon has produced complete remission of the Zollinger-Ellison syndrome and a stabilization of tumor growth has occurred during the last 7 years, allowing the patient to live a normal life. This peculiar response to therapy is discussed.
 ...
PMID:Liver metastases of endocrine tumour associated with multiple endocrine neoplasia type 1: a sustained response to interferon therapy or a peculiar benign course? 1110 Mar 30

We report a very rare case of ovarian gastrinoma in the context of multiple endocrine neoplasia type I, including primary hyperparathyroidism and Zollinger-Ellison syndrome. Somatostatin receptor scintigraphy revealed the ovarian involvement at an early stage. Oophorectomy led to the final diagnosis and complete healing.
 ...
PMID:Ovarian gastrinoma in multiple endocrine neoplasia type I: a case report. 1117 12

Carcinoid tumors were identified in the antro-pyloric mucosa of four patients with multiple endocrine neoplasia type 1 (MEN-1)/Zollinger-Ellison syndrome, accounting for 8.7% of 46 patients with this condition examined by endoscopy and histology. In contrast, no tumors were found in the antral biopsies from 124 cases of sporadic Zollinger-Ellison syndrome (P < 0.001), indicating a prominent role for the MEN-1 gene defects in tumor development. Immunohistochemically the tumors did not express the hormones produced by antral endocrine cells (gastrin, somatostatin, serotonin). In contrast, two of them were diffusely immunoreactive for the isoform 2 of the vesicular monoamine transporter (VMAT-2), a marker specific for the gastric nonantral enterochromaffin-like (ECL) cells. In one of these patients a second antral VMAT-2-positive carcinoid was seen 21 months after the first diagnosis. The other two antral carcinoids were unreactive for VMAT-2. Multiple ECL cell tumors were found in the gastric body-fundus mucosa of the two patients with VMAT-2-positive, but not in those with VMAT-2-negative, antral carcinoids. In one case, the former tumors were diagnosed 22 months after the detection of the antral tumor. We conclude that the antral mucosa is an additional tissue that may harbor endocrine tumors in MEN-1 syndrome. These tumors did not express the phenotype of normal antral endocrine cells and, in at least two cases, were identified as ectopic ECL cell carcinoids.
 ...
PMID:The antral mucosa as a new site for endocrine tumors in multiple endocrine neoplasia type 1 and Zollinger-Ellison syndromes. 1134 33

Pancreatic endocrine tumours (PET) are rare but nonetheless important to recognize and treat in a timely fashion. Significant morbidity occurs due to excess secretion of hormones, with all of the PET having some degree of malignant potential. Surgeons must plan directed operative strategies to deal with these tumours and be prepared to undertake aggressive palliative debulking resections if indicated. Somatostatin receptor scintigraphy and endoscopic ultrasound have been particularly helpful in both localizing and staging patients with PET. Other important advances in management include the use of long-acting somatostatin analogues to inhibit hormonal secretion and tumour growth. The possibility of multiple endocrine neoplasia type 1 (MEN-1) should be considered in any patient with a PET. The present article will review the various classes of PET, describe MEN-1 in relation to PET and examine advances in imaging and localization. The role of surgery for PET is also discussed in the present review.
 ...
PMID:Endocrine tumours of the pancreas: review and recent advances. 1150 92

Therapeutic strategy of neuroendocrine tumours is complex, due to their heterogeneity and to the fact that although generally slow growing, a significant proportion demonstrates aggressive tumour growth. Symptomatic carcinoid syndrome and various pancreatic endocrine tumours with symptomatic syndromes are well controlled with somatostatin analogues. Surgery remains the mainstay of treatment if the tumour can be resected. Metastatic pancreatic neuroendocrine tumour are treated when resection is not feasible with combination chemotherapy using adriamycin and streptozotocin, which remains a standard of care. In well differentiated tumour of the gut or the lung there is no clear standard of chemotherapy and treatment vary according to the tumour course. In indolent cases, somatostatin analogues are the best treatment, in case of aggressive tumours chemoembolisation should be preferred when the disease is located or predominant in the liver. Poorly differentiated tumours are treated by combination chemotherapy with etoposide and cisplatin, and surgery has no indication. Gastrinoma and other pancreatic tumours arising in the context of multiple endocrine neoplasia type I disease need a specific therapeutic strategy.
 ...
PMID:[Treatment strategy of neuroendocrine tumors]. 1192 20

Gastrinoma treatment has evolved considerably in the last 20 years. In particular, the advent of effective acid-reducing pharmacologic agents has changed the primary morbidity of this disease entity from one of acid hypersecretion to one of tumor growth and spread. Thus, while symptoms can be temporized using histamine receptor antagonists, proton pump inhibitors, or somatostatin analogs, cure can be effected only by surgical means. Recent advances in operative techniques and pre- and intra-operative imaging studies, including routine duodenotomy, somatostatin-receptor scintigraphy, and intraoperative ultrasound, have allowed for identification and subsequent resection of more than 95% of gastrinoma tumors. Most experts agree that all sporadic cases of localized gastrinoma should be excised. In addition, debulking of metastatic tumor may improve symptoms and survival when cure cannot be ascertained. There is, however, some controversy as to the surgical approach for gastrinoma found in the setting of multiple endocrine neoplasia, type 1. Because of the usual multiplicity and particular indolence of these tumors, two primary strategies have emerged: aggressive approaches have been advocated in an effort to eradicate all present and potential tumor; and less aggressive, or nonoperative, approaches have been suggested because it is unclear whether intervention offers survival or disease-free benefit in this population. We advocate surgical intervention for patients with gastrinoma and multiple endocrine neoplasia, type 1 when tumors exceed 2.5 cm in size. This tumor size has been associated with a higher likelihood of hepatic metastases, which ultimately affects survival. The role of adjuvant therapies for gastrinoma remains limited.
 ...
PMID:Gastrinoma. 1205 14

Carcinoid tumours are relatively rare neoplasms that often present as diagnostic dilemmas due to obscure or non-specific symptomatology. The ability of carcinoid tumours to cause clinical symptoms by secretion of hormones or biogenic amines is best recognised in the form of the carcinoid syndrome. Although generally slow growing, a significant proportion demonstrates aggressive tumour growth and may be difficult to manage. Ten percent of carcinoids are part of the spectrum of multiple endocrine neoplasia type 1, which should be considered in the investigation and management of these patients. A further 10% of carcinoid tumours are associated with other noncarcinoid tumours of various histological types. This review discusses recent improvements in biochemical diagnosis with the introduction of plasma chromogranin A, and puts into context the use of the imaging modalities, including Indium-111 Octreotide scintigraphy, and newer developing techniques, such as positron emission tomography. The therapeutic options are reviewed, including the use of somatostatin analogues as the treatment of choice in the control of hormone-mediated symptoms, the role of surgery, the use of chemotherapy, biotherapy using interferon, and the exciting new field of receptor-targeted therapy. In addition, the challenging interventional management of liver metastases is discussed, including the role of hepatic-artery embolisation and chemo-embolisation, radiofrequency ablation and the place of orthotopic liver transplantation in selected patients. We conclude that with the increasing number of investigative procedures and therapeutic options available to diagnose and treat carcinoid tumours, a multidisciplinary approach is needed. Furthermore, additional scientific research and controlled clinical trials are needed to determine the efficacy of the many treatment options, which for these rare tumours can only be achieved by collaboration.
 ...
PMID:Carcinoid tumour. 1241 Jan 71


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>