1/45. Periampullary pancreatic somatostatinoma.BACKGROUND: Somatostatinomas involving the gastrointestinal tract are extremely rare neoplasms that typically present with indolent, nonspecific symptoms in the absence of systemic neuroendocrine manifestations that characterize the somatostatinoma syndrome. Because of a relatively large size at the time of presentation (average diameter of 5 cm) and common location within the head of the pancreas, the Whipple procedure (pancreaticoduodenectomy) serves as the predominant modality for curative and palliative surgical approaches. methods: Two cases of somatostatinoma involving the minor duodenal papilla with concomitant pancreatic divisum were reviewed, with a general overview of this unique islet cell tumor. RESULTS: Unlike typical somatostatinomas, these two tumors were subcentimeter in size but were associated with synchronous regional metastasis. CONCLUSIONS: Somatostatinomas are often associated with regional and/or portal metastases at the time of diagnosis, and only 60% to 70% of surgical cases result in complete tumor resection. Predictors of an unfavorable prognosis include size >3 cm, poor cytological differentiation, regional and/or portal metastasis, and incomplete surgical resection. Even in the presence of synchronous metastases, the 5-year overall survival for patients with somatostatinoma is approximately 40%. Currently, there are no clinical trials demonstrating significant improvement in survival with the use of adjuvant therapy.- - - - - - - - - - ranking = 1keywords = somatostatinoma (Clic here for more details about this article) |
2/45. A small, incidentally detected pancreatic somatostatinoma: report of a case.We report an asymptomatic 72-year-old woman with a small, incidentally detected, pancreatic somatostatinoma. The tumor, measuring 1 cm in diameter, showed a hypervascular pattern of contrast enhancement on computed tomography, and was found angiographically to receive a blood supply from the posterior superior pancreaticoduodenal artery. The results of preoperative hormonal assays all were normal. No assay for somatostatin was performed. No abnormality in either the pituitary or parathyroid was found. We thus considered the tumor to be a sporadic, nonfunctioning endocrine cell tumor, and enucleation was carried out. As some tumor cells in the resected specimen showed immunoreactivity for somatostatin, a diagnosis of somatostatinoma was made. Therefore, the possibility of somatostatinoma should be kept in mind when making a differential diagnosis of pancreatic endocrine tumors in cases where even a small hypervascular tumor is detected on enhanced computed tomography.- - - - - - - - - - ranking = 0.875keywords = somatostatinoma (Clic here for more details about this article) |
3/45. Atypical thymic carcinoid and malignant somatostatinoma in type I multiple endocrine neoplasia syndrome: case report.Thymic carcinoid and malignant somatostatinoma are both rare, and their concurrent presence in multiple endocrine neoplasia type 1 (MEN-1) has never been reported in the English literature to date. We present a patient with thymic carcinoid and malignant somatostatinoma in association with MEN-1. The patient eventually died of pulmonary aspergillosis and respiratory failure. autopsy showed a 16 x 10 x 8-cm thymic carcinoid tumor, parathyroid and adrenal gland hyperplasia, and malignant somatostatinoma of the pancreas with a metastatic tumor over the splenic hilum.- - - - - - - - - - ranking = 0.875keywords = somatostatinoma (Clic here for more details about this article) |
4/45. A case of multiple endocrine neoplasia: hyperparathyroidism, insulinoma, GRF-oma, hypercalcitoninaemia and intractable peptic ulceration.A 42-year-old woman with a family history of multiple endocrine neoplasia type 1 (MEN 1) presented with symptomatic hypoglycaemia and peptic ulceration. Investigation revealed an insulinoma, hyperparathyroidism, hypercalcitoninaemia with a positive pentagastrin stimulation test, acromegaly due to a GRF-oma, hyperprolactinaemia and normal serum gastrin levels. Five pancreatic tumours were removed at laparotomy and immunostaining was positive for insulin, calcitonin, somatostatin and glucagon. Post-operatively she developed elevated serum gastrin levels and gross peptic ulceration, despite H2-blockers, and died of gastro-intestinal haemorrhage suggesting that removal of the somatostatinoma may have allowed increased gastrin secretion from a gastrinoma. This case emphasizes the importance of measuring a wide variety of tumour marker peptides in MEN 1 and suggests that caution is required in interpretation of the pentagastrin stimulation test in such cases. patients with MEN 1 and known peptic ulceration may require perioperative omeprazole treatment even if serum gastrin levels are normal.- - - - - - - - - - ranking = 0.125keywords = somatostatinoma (Clic here for more details about this article) |
5/45. somatostatinoma of the pancreas with hypercalcaemia. A case report.A unique case of somatostatinoma of the pancreas complicated by severe hypercalcaemia is described. Surgical resection was not possible owing to tumour extent. A dramatic and prolonged clinical and biochemical response was achieved with streptozotocin.- - - - - - - - - - ranking = 0.125keywords = somatostatinoma (Clic here for more details about this article) |
6/45. Duodenal somatostatinoma associated with Von Recklinghausen's disease.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.- - - - - - - - - - ranking = 1.125keywords = somatostatinoma (Clic here for more details about this article) |
7/45. plasma pancreatic hormone levels in a case of somatostatinoma: diagnostic and therapeutic implications.plasma somatostatin immunoreactivity (SIR) was elevated 40-fold in an insulin-treated diabetic with disseminated pancreatic carcinoma. The diagnosis of somatostatinoma was supported by histological and ultrastructural similarities between metastatic cells and pancreatic D cells. Under acid conditions, 75% of the plasma SIR eluted as a 6000- to 7000-dalton protein and 25% as synthetic somatostatin (mol wt 1600), whereas the 20-fold elevated urine SIR consisted almost exclusively of the higher molecular weight fraction. The hypersomatostatinemia was associated with reduced basal and stimulated pancreatic hormone levels, which might reflect its involvement in the steatorrhea and diabetes, and its protection against ketoacidosis. plasma SIR rose 50% upon insulin withdrawal and 10-fold after tolbutamide injection and fell 30% after diazoxide. It is concluded that an increase in plasma and urine SIR, the presence of a 6000- to 7000-dalton SIR fraction in plasma and urine, a reduction in basal and stimulated pancreatic hormone levels, and tolbutamide-induced somatostatin release can be diagnostic for a somatostatinoma. Streptozotocin reduced tumor volume, hypersomatostatinemia, and tolbutamide-induced somatostatin release, suggesting that this drug may be useful in the treatment of disseminated somatostatinoma.- - - - - - - - - - ranking = 0.875keywords = somatostatinoma (Clic here for more details about this article) |
8/45. Somatostatin-producing pancreatic endocrine carcinoma presented as relapsing cholangitis -- a case report.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.- - - - - - - - - - ranking = 0.125keywords = somatostatinoma (Clic here for more details about this article) |
9/45. Metastatic somatostatinoma of the chest wall.A 72-year-old male was diagnosed as having a thoracic tumor in the left chest wall five years after resection of primary pancreatic somatostatinoma. The tumor was suggestive of metastasis to the chest wall or to the left lung from the previously resected somatostatinoma based on the abnormally elevated serum somatostatin level. Percutaneous biopsy confirmed that the lesion was an islet cell tumor and thoracotomy demonstrated metastasis to the left third rib without involvement of the left lung. Our case represents a rare documentation of somatostatinoma metastatic to the chest wall for which complete resection was performed.- - - - - - - - - - ranking = 0.875keywords = somatostatinoma (Clic here for more details about this article) |
10/45. Pancreatic endocrine tumors are a rare manifestation of the neurofibromatosis type 1 phenotype: molecular analysis of a malignant insulinoma in a NF-1 patient.The tumorigenesis of sporadic endocrine tumors is still not fully understood. It is well known that patients with von Recklinghausen syndrome (NF-1) (OMIM 162200) carrying NF1 germline mutations are predisposed to endocrine tumors including pheochromocytomas and duodenal somatostatinomas. It is unclear, however, whether the rarely reported occurrence of pancreatic insulinomas in NF-1 patients represents a coincidental finding or whether insulinomas are a rare manifestation of the NF-1 syndrome. To determine the potential association between the NF-1 syndrome and pancreatic endocrine tumors, we analyzed a NF-1 patient with a well-differentiated pancreatic endocrine carcinoma for NF1 mutation, allelic loss of the NF1 gene and its expression in peripheral blood and tumor cells. The germline mutation c. 499 del TGTT known in the family was confirmed by polymerase chain reaction (PCR) and direct sequencing of exon 4 in dna extracted from peripheral blood. loss of heterozygosity (LOH) analysis of the NF1 gene was carried out using 3 intragenic microsatellite markers on 17q11.2. rna expression was examined by reverse transcription and a consecutive PCR spanning intron 3 of the NF1 gene including the mutated site in exon 4. immunohistochemistry was used to analyze NF-1 protein expression. mutation analysis of peripheral blood leukocytes confirmed the 4 base pair deletion in exon 4 starting at codon 167 (499 del TGTT). LOH analysis of tumor tissue revealed retention of both NF1 alleles. While reverse transcriptase-PCR of peripheral blood showed bi-allelic expression of both the wild-type NF1 and the mutated form, reverse transcriptase-PCR of tumor extracts demonstrated expression of the mutated but not the wild-type NF1 allele. Additionally, neurofibromin, the NF1 gene product, was absent in the tumor tissue of the NF-1 patient. These results show that the wild-type NF1 transcrips and protein are reduced, in the reported insulinoma, supposedly by epigenetic mechanisms. This provides strong evidence that there is a relationship between von Recklinghausen disease and the patient's insulinoma. In this line, insulinomas may be viewed as a rare manifestation of the NF-1 syndrome. Furthermore, the NF1 gene must be considered as a candidate tumor suppressor gene for sporadic insulinomas and probably other pancreatic endocrine tumors.- - - - - - - - - - ranking = 0.125keywords = somatostatinoma (Clic here for more details about this article) |
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