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)

Few cases of malignant glucagonomas have been described in the literature. In this paper we present a case of a 77-year-old woman with necrolytic migratory erythema and high plasma glucagon and chromogranin A levels caused by a neuroendocrine tumour. An abdominal CT scan suggested a pancreatic lesion and two liver metastases. The patient underwent pancreatic debulking and liver metastasectomy. Histological and immunohistochemical investigations revealed a well differentiated neuroendocrine tumour with vascular invasion and scattered immunopositivity for somatostatin receptors. The patient was treated with octreotide (20 mg i.m. every 28 days) for three years without side effects. Three months after surgery symptoms of disease recurred accompanied by hyperglucagonaemia and newly diagnosed liver lesions. The patient was treated with octreotide (30 mg i.m. every 28 days) and interferon-alpha (6 MU s.cc 3 times per week) plus three cycles of hepatic chemoembolisation. Symptoms resolved after the first month of therapy, hormone levels decreased compared to untreated levels and metastatic growth slowed as observed by radiographic evidence. The patient is now asymptomatic with persistent hepatic disease and normal serum glucagon levels forty months after primary treatment. So far, only few immunohistochemical studies are reported on malignant glucagonoma and combined treatment schedules. We demonstrated, for the first time, a scattered immunopositivity for somatostatin receptors in a malignant glucagonoma. For this reason, the somatostatin analogs therapy was instituted. A combined antiproliferative medical treatment and the hepatic chemoembolization have been able to control tumor growth and disease symptoms for a long time after surgery.
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PMID:Malignant glucagonoma. New options of treatment. 1676 30

In most cases gastro-enteropancreatic neuroendocrine tumors grow slowly. Interferon-alpha and somatostatin analogs have shown symptomatic, biochemical, and, in a minority of cases, antiproliferative activity. Generally, they are proposed as single-agent therapy. However, based on in vitro and in vivo evidence, the combined use of these drugs was proposed in several non-randomized trials, indicating that there is an additive effect of the combination. Nevertheless, the three randomized trials published so far did not show a statistically significant survival benefit for the combination compared to the same agents alone, even though an advantage for the combination came out in all three studies. On the other hand, data from non-randomized trials would justify the sequential use of the two drugs or the combination after progression on single agent therapy. Therefore, at present the up-front combined use of interferon-alpha and somatostatin analog is not justified, whereas it could be indicated after progression to single-agent therapy. Further larger, international, prospective, randomized, multicentric clinical trials studying homogeneous populations would be necessary to give a final answer, but the rarity and heterogeneity of this malignancy does not assure that it will be possible.
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PMID:Interferon-alpha and somatostatin analog in patients with gastroenteropancreatic neuroendocrine carcinoma: single agent or combination? 1679 33

This review gives an introduction to the classification and staging of neuroendocrine tumors, as the prognostic implications of these classifications influence therapeutic decisions. The indications for biotherapy are given, together with a short update on the mechanism of somatostatin analogs and interferon-alpha therapy. This is followed by an in-depth description of the use of biotherapy, its results with respect to symptomatic and antiproliferative treatment, as well as its side-effects.
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PMID:Neuroendocrine tumors. Biotherapy. 1738 70

Traditional therapies have offered patients with advanced gastrointestinal neuroendocrine tumors limited benefit. Selected patients with hepatic metastases may benefit from surgical debulking, embolization, or other ablative therapies. While somatostatin analogs are highly effective in controlling symptoms of hormonal secretion, they are only rarely associated with tumor regression. The clinical benefit associated with the administration of systemic agents such as interferon-alpha or cytotoxic chemotherapy is less clear, and the widespread use of such regimens has been limited by their relatively modest anti-tumor activity, as well as concerns regarding their potential toxicity. The mixed clinical results seen with these agents in neuroendocrine tumors have led to great interest in the development of novel treatment approaches for patients with advanced disease. Recent clinical studies of novel agents, particularly those targeting the vascular endothelial growth factor pathway and mammalian target of rapamycin, have demonstrated promising activity in patients with advanced neuroendocrine tumors. Ongoing randomized studies should help better define the role these and other targeted agents will play in the future treatment of patients with this disease.
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PMID:Gastrointestinal neuroendocrine tumors: a role for targeted therapies? 1763 38

Endocrine pancreatic tumors (EPTs) are uncommon tumors, representing 1-2% of all pancreatic neoplasms. They are categorized on the basis of their clinical features into functioning and non-functioning tumors. EPTs may be part of the multiple endocrine neoplasia type 1 (MEN 1), an autosomal dominant syndrome due to inactivating germline mutation of the menin gene. Somatic mutations of menin are present in about 20% of sporadic neoplasms, particularly gastrinomas and insulinomas. 30-75% of patients with MEN1 have EPTs. The most prevalent are the gastrinomas (20-60%), then the insulinomas (5-10%), the glucagonamas and VIPomas (6-10%), whereas the nonfunctioning EPTs are present in 20-40% of patients. The most important biochemical screening marker for EPTs is chromogranin A, as it increases in 50-80% of patients. The most important negative prognostic factors are the presence of metastases, the gross invasion of adjacent organs, the angioinvasion, the perineural invasion and an immunopositivity for Ki-67 > 2%. Among the different imaging techniques, echoendoscopy, computed tomography (CT) and magnetic resonance imaging (MRI) are indicated for the detection of the primary tumor, but (III)In-octreotide scintigraphy has the highest sensitivity for detecting metastases. The choice of treatment is still debated and is different when the tumor occurs as a part of the MEN syndrome. The surgical treatment is the first choice for insulinomas and is more controversial for gastrinomas. The medical treatment includes somatostatin analogues (SA), chemotherapy and interferon-alpha (IFN-alpha). SA seem to improve the symptoms and have an antiproliferative effect, the most striking effect being seen in patients with VIPomas. Chemotherapy, which is generally proposed as a combination of streptozotocin (STZ) and 5-fluorouracil (5-FU) or doxorubicin, is indicated when the tumors tend to grow. Interferon-alpha (IFN-alpha) stimulates the immune system, blocks the tumor cells in the G1/S-phase of the cell cycle, inhibits protein and hormone synthesis and inhibits angionenesis. Treatment with IFN has been shown to produce symptomatic response in 40-60% of patients, a biochemical response in 30-60% and tumor shrinkage in 10-15%.
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PMID:[Endocrine tumors of the pancreas (EPTs) in multiple endocrine neoplasia (MEN1): up-date on prognostic factors, diagnostic procedures and treatment]. 1796 53

Functional pancreatic endocrine tumors other than gastrinoma and insulinoma are quite rare. Some of these tumors may be part of multiple endocrine neoplasia type one (MEN-1) syndrome or phakomatoses. Depending on their cell type, functional pancreatic endocrine tumors may cause distinct clinical endocrine syndromes, such as the 'glucagonoma syndrome', Verner-Morrison syndrome and the 'somatostatinoma syndrome'. The significant progress made in recent years, especially in the field of imaging procedures, has brought about great improvement in the identification and differentiation of these neoplasms. Currently, the only curative treatment for islet cell tumors is complete surgical resection. The medical treatment of endocrine pancreatic tumours consists of somatostatin analogues, chemotherapy, and interferon-alpha. The purpose of this manuscript is to provide an overview of the contemporary etiopathogenesis, diagnosis and treatment of rare pancreatic endocrine tumors.
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PMID:[Rare pancreatic endocrine tumors]. 1872 49

Traditional treatments for patients with advanced neuroendocrine tumors include surgical debulking, hepatic embolization, somatostatin analogues, and interferon-alpha. Patients with pancreatic neuroendocrine tumors also may benefit from treatment with the alkylating agents streptozocin or temozolomide. In recent years, several promising new approaches have been investigated in patients with advanced neuroendocrine tumors. One such approach has been the use of radiopeptide therapy targeting somatostatin receptors. Additionally, agents targeting the vascular endothelial growth factor pathway and mammalian target of rapamycin have shown preliminary evidence of activity and are currently being evaluated in large randomized studies.
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PMID:Progress in the treatment of neuroendocrine tumors. 1933 11

Rectal carcinoids are becoming more common: in the USA they have increased in frequency by 800 - 1000 % in the past 35 years. This dramatic increase is probably related to the introduction of colonoscopic screening which also results in the "incidentally" detected neuroendocrine rectal tumours/carcinomas being smaller than in the pre-screening era. Endosonography is the method of choice for determining the size and depth of penetration of the tumours and for detecting lymph node metastases. Tumours of < 10 mm in size that have not infiltrated the muscularis propria can usually be removed endoscopically. When invasion of lymph or blood vessels or lymph node metastases are found, surgical resection of the lymph nodes is indicated. Neuroendocrine rectal neoplasms (rectal carcinoids) of 10.1 - 20 mm in diameter have a metastasis risk of 17 - 42 (81) % for neuroendocrine rectal neoplasms > 20 mm in size this risk increases to 60 - 80 %. A carcinoid syndrome is rarely observed, even in cases of distant metastases of neuroendocrine rectal carcinomas. Stable somatostatin analogues and interferon-alpha constitute the drug therapies of choice for carcinoid syndrome. As a result of the increasing early detection of rectal carcinoids/carcinomas the prognosis for the patients has improved considerably in the last 30 years. In addition to the early detection of colorectal adenoma and adenocarcinoma, screening colonoscopy also makes possible the early detection and early therapy for neuroendocrine rectal tumours/carcinomas.
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PMID:[Rectal carcinoids on the rise - update]. 1935 64

The case of a 47-year-old patient is described with a carcinoid of the middle ear. Initial symptoms were hearing impairment, feeling of pressure and dizziness. Mastoidectomy was carried out for mastoid shadowing with space encroachment in the auditory canal. The histological examination initially revealed an atypical cholesteatoma and the subsequent immunohistochemical investigation revealed a carcinoid. A radical excavation with complete excision of the tumor and tympanoplasty was carried out. A carcinoid of the middle ear is definitely a rare finding and the primary treatment is complete surgical removal. If metastases are suspected octreotide scintigraphy has proved to be the best option in analogy to intestinal carcinoids. Radiation therapy has not proved successful but the use of the somatostatin analog octreotide, interferon-alpha or palliative chemotherapy (e.g. streptozotosine, 5-fluorouracil) for metastases are further therapy options.
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PMID:[Carcinoid of the middle ear. A case report]. 1963 19

Appendiceial carcinoid tumors (CAT) and goblet cell carcinoids (GCCAT) are rare. Most are asymptomatic and found after appendectomy. Metastases to regional nodes are seen in 10% of CATs larger than two cm. Ovarian or peritoneal metastases are seen in 20% of all GCCATs. Further surgical treatments are shown in Tables. Non-resectable CAT are treated with interferon-alpha, somatostatin analogs or radionuclides. Non-resectable GCCATs are treated like colo-rectal cancer. Patients with CAT larger than two cm, involvement of mesoappendix or metastases and all GCCAT should be followed life-long.
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PMID:[Appendiceal carcinoid tumors and goblet cell carcinoids]. 2052 67


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