Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Somatostatinomas are the rarest pancreatic endocrine tumors and can arise in the pancreas or duodenum. Duodenal somatostatinomas are less common than, and are distinguished from, their pancreatic counterparts by a frequent association with type I neurofibromatosis, the presence of psammoma bodies, the less frequent presence of metastatic disease, and the absence of somatostatinoma syndrome (diabetes mellitus, steatorrhea, and cholelithiasis). We report a case of somatostatinoma with metastases and psammoma bodies presenting with all three features of the syndrome in a patient with neurofibromatosis. Although several reports have documented portions of the syndrome in patients with duodenal somatostatinomas, to our knowledge, this is the first report of the complete syndrome associated with a duodenal lesion.
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PMID:Duodenal somatostatinoma presenting with complete somatostatinoma syndrome. 1160 61

This is a retrospective review of 131I-MIBG therapy for metastatic neuroendocrine tumours in 25 adult patients. The tumours comprised 17 carcinoids, six paragangliomas, one somatostatinoma and one intestinal smooth muscle sarcoma. All patients (age range 28-84 years) had stage IV disease and a positive diagnostic 123I-MIBG scan. Patients received 11.1 GBq (300 mCi) of 131I-MIBG given in three cycles at 3-monthly intervals. The mean cumulative dose was 27.7 GBq (751 mCi). Symptomatic response was observed in 80%, hormonal response in 55% and tumour response in 48% (WHO criteria). Of the 25 patients, 40% are still under follow-up. Death was due to disease progression in all except one. The median survival time was 48 months from diagnosis of metastatic disease, and 17 months from the last 131I-MIBG therapy. The 5-year survival rate was 59% (95% confidence interval, 34%-78%). There was no statistical difference in survival between previously treated (chemo/radiotherapy) and treatment-naive patients. Side-effects were minimal and commonly include nausea (in the first 24 h) and a transient fall in platelet count. 131I-MIBG provides a good therapeutic response in patients with metastatic neuro-endocrine tumours.
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PMID:Treatment of neuroendocrine tumours in adults with 131I-MIBG therapy. 1284 98

All pancreatic neuroendocrine tumors derive from Langerhans islet cells and have a low incidence. Half of them are functioning tumors that produce diverse hormones and occasionally cause serious clinical endocrine syndromes. They may be malignant, but they have a better survival, if compared to pancreatic ductal adenocarcinoma. Insulinoma, gastrinoma, glucagonoma, VIPoma (VIP=vasoactive intestinal peptide), somatostatinoma and ACTHoma are functioning tumors and they may also be part of Multiple Endocrine Neoplasia type I (MEN 1) syndrome and of von Hippel-Lindau disease. Diagnosis of non-functioning tumors is usually late, when they reach a big size and have even developed nodal and hepatic metastases. Nowadays, there are effective medical treatments for the medical problems secondary to excessive hormone production. For example, the hypergastrinemia typical of the Zollinger-Ellison syndrome in gastrinoma, can be adequately managed. Surgical resection is the most advisable therapy for pancreatic endocrine tumors, especially when they are small, when long time survival is better. Pre and intra operative imagenology is a great aid to locate these tumors. There are several surgical alternatives, according to the tumor size and location within the pancreas. Furthermore, palliative therapy can be used in disseminated disease. Treatment success is the result of a multidisciplinary medical team work of endocrinologists, surgeons, gastroenterologists, pathologists and geneticists.
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PMID:[Pancreatic neuroendocrine tumors]. 1527 51

Somatostatinomas are rare, malignant, somatostatin-producing neuroendocrine tumors with a prevalence of one in 40 million. The coincidence of Von Recklinghausen's disease and duodenal somatostatinoma has been known since 1982. We report the case of a 57-year-old female patient with Von Recklinghausen's disease and a tumor of the pancreatic head that was diagnosed due to painless icterus. Histopathological examination after pylorus-preserving pancreatoduodenectomy revealed the existence of a duodenal somatostatinoma with lymph node metastases. Characteristics of the association of von Recklinghausen's disease and somatostatinoma, and therapy and prognosis will be discussed. In patients with Von Recklinghausen's disease and an ampullary tumor, a somatostatinoma should be considered. In contrast to its pancreatic counterparts, duodenal somatostatinoma is frequently associated with Von Recklinghausen's disease, often contains psammoma bodies, is rarely associated with a recognizable "somatostatin syndrome", and is hardly ever associated with demonstrable metastases at the time of diagnosis. Small tumors arising in the duodenum may be treated with local excision, whereas larger tumors should be treated by total excision, which may entail a partial pancreatoduodenectomy.
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PMID:Duodenal somatostatinoma associated with Von Recklinghausen's disease. 1561 18

Somatostatin-producing endocrine tumors are rare neoplasms usually arising in the pancreas and duodenum and they account for less than 1% of all gastrointestinal endocrine tumors. Besides somatostatinoma syndrome, which is characterized by diabetes mellitus, steatorrhea and cholelithiasis, patients with somatostatin-producing endocrine tumors commonly complain of nonspecific symptoms such as vague abdominal pain, weight loss or changes in bowel habits. Tumor behavior cannot be predicted by histological features alone, and malignancy is determined by the presence of metastases. We report here a case of malignant pancreatic endocrine tumor producing somatostatin presented as relapsing cholangitis who was treated with Whipple pancreatoduodenectomy.
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PMID:Somatostatin-producing pancreatic endocrine carcinoma presented as relapsing cholangitis -- a case report. 1584 91

The authors report a large duodenal somatostatinoma, a very rare tumor entity. A 8.5 cm globular mass in the area of the unicate process of the pancreas was detected in a 45 year old caucasian female by computerized tomography. The patient had only mild complaints. Initial treatment consisted of right pancreatectomy with preservation of the pylorus. Histological evaluation rendered a diagnosis of low-grade malignant neuroendocrine carcinoma with expression of somatostatin, respectively of somatostatinoma arising in the duodenum and infiltrating into the pancreas. 26 months after the initial surgery liver and lymph node metastases were detected and surgically removed. This case confirms that duodenal somatostatinomas are very difficult to diagnose preoperatively because of unspecific symptoms. Most duodenal somatostatinomas are found incidentally. Treatment of choice is radical surgical resection with a possible cure in early stages of the disease. Even a large tumor as ours is resectable with negative surgical margins. Management of recurrent or metastatic disease is also surgical. Additional chemotherapy and supportive care may be beneficial for the patient.
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PMID:Duodenal somatostatinoma: clinical and immunohistochemical patterns--difficult differential diagnosis in regard to gangliocytic paraganglioma: report of a case. 1585 80

Von Recklinghausen's disease is an autosomal dominant hereditary disease associated with a wide number of neoplasms. We report a case of a 47-year-old Caucasian male affected by Von Recklinghausen's disease who developed a malignant somatostatinoma of the papilla major and minor associated with jejunal gastrointestinal stromal tumour with uncertain behaviour. At laparotomy, multiple hepatic metastases were evident. Whipple pancreaticoduodenectomy, jejunal resection, extensive lymphadenectomy and multiple hepatic wedge resections were performed. The patient was alive without recurrence after 24 mo. This is the fourth case reported in the world literature of a patient with Von Recklinghausen's disease associated with periampullary somatostatinomas and jejunal stromal tumor. In patients with Von Recklinghausen's disease who complain of gastrointestinal symptoms, a high suspicion index for periampullary endocrine tumours and/or gastrointestinal stromal tumour is required. An aggressive surgical approach seems to give long term survival also in metastatic patients.
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PMID:Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen's disease. 1756 51

Somatostatin-producing neuroendocrine tumors (SOM-NETs) of the duodenum and pancreas appear to be heterogeneous. To determine their clinicopathological profiles, respective data were analyzed on a series of 82 duodenal and 541 pancreatic NETs. In addition, the clinical records of 821 patients with duodenal or pancreatic NETs were reviewed for evidence of a somatostatinoma syndrome. Predominant or exclusive expression of somatostatin was found in 21 (26%) duodenal and 21 (4%) pancreatic NETs. They were classified as sporadic (n=31) or neurofibromatosis type 1 (NF1)-associated duodenal NETs (n=3), gangliocytic paragangliomas (GCPGs; n=6), or poorly differentiated neuroendocrine carcinomas (pdNECs; n=2). In addition, five duodenal and four pancreatic SOM-NETs were found in five patients with multiple endocrine neoplasia type 1 (MEN1). Metastases occurred in 13 (43%) patients with sporadic or NF1-associated SOM-NETs, but in none of the duodenal or pancreatic MEN1-associated SOM-NETs or GCPGs. Sporadic advanced (stage IV) SOM-NETs were more commonly detected in the pancreas than in the duodenum. None of the patients (including the 821 patients for whom only the clinical records were reviewed) fulfilled the criteria of a somatostatinoma syndrome. Our data show that somatostatin expression is not only seen in sporadic NETs but may also occur in GCPGs, pdNECs, and hereditary NETs. Surgical treatment is effective in most duodenal and many pancreatic SOM-NETs. MEN1-associated SOM-NETs and GCPGs follow a benign course, while somatostatin-producing pdNECs are aggressive neoplasms. The occurrence of the so-called somatostatinoma syndrome appears to be extremely uncommon.
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PMID:Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. 1831 Feb 90

This article describes a newly recognized highly malignant neoplastic entity in young bearded dragons (Pogona vitticeps), gastric neuroendocrine carcinomas, which readily metastasize. Ten bearded dragons with histories of anorexia (8), vomiting (3), hyperglycemia (2), and anemia (3) were included in this study. All animals had neoplastic masses in their stomach, with metastasis to the liver. Microscopically, 6 of these neuroendocrine carcinomas were well-differentiated and 4 were poorly differentiated. For further characterization, immunohistochemistry for protein gene product 9.5, neuron-specific enolase, endorphin, chromogranins A and B, synaptophysin, somatostatin, insulin, glucagon, gastrin, pancreatic polypeptide, and vasoactive intestinal peptide was performed on 5 animals. Because only immunolabeling for somatostatin was consistently observed in all neoplasms, a diagnosis of somatostatinoma was made for these 5 bearded dragons. Some neoplasms also exhibited multihormonal expression. Electron microscopy performed on 1 tumor confirmed the presence of neuroendocrine granules within neoplastic cells. Gastric neuroendocrine carcinomas, and specifically somatostatinomas, have not been previously reported in bearded dragons, or other reptiles, and may be underdiagnosed due to inconsistent, ambiguous clinical signs. In humans, pancreatic somatostatinomas are associated with a syndrome of hypersomatostatinemia, which includes hyperglycemia, weight loss, and anemia, as observed in some of these bearded dragons. Somatostatinomas in humans are commonly associated with neurofibromatosis type 1 (Von Recklinghausen's disease), caused by a mutation in the tumor suppressor gene NF1, which results in decreased expression of neurofibromin. In all 5 animals examined, neoplasms exhibited decreased neurofibromin expression compared with control tissues, suggesting that decreased functional neurofibromin may play a role in the pathogenesis of somatostatinomas in bearded dragons.
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PMID:Gastric neuroendocrine carcinomas in bearded dragons (Pogona vitticeps). 1960 3

Somatostatinomas are extremely rare neuroendocrine tumors of the gastrointestinal tract, first described in the pancreas in 1977 and in the duodenum in 1979. They may be functional and cause somatostatinoma or inhibitory syndrome, but more frequently are non-functioning pancreatic endocrine tumors that produce somatostatin alone. They are usually single, malignant, large lesions, frequently associated with metastases, and generally with poor prognosis. We present the unique case of a 57-year-old woman with two synchronous non-functioning somatostatinomas, one solid duodenal lesion and one cystic lesion within the head of the pancreas, that were successfully resected with a pylorus-preserving Whipple's procedure. No secondaries were found in the liver, or in any of the removed regional lymph nodes. The patient had an uneventful recovery, and remains well and symptom-free at 18 mo postoperatively. This is an extremely rare case of a patient with two synchronous somatostatinomas of the duodenum and the pancreas. The condition is discussed with reference to the literature.
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PMID:Two synchronous somatostatinomas of the duodenum and pancreatic head in one patient. 1999 10


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