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 deve...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 periampuUary 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.展开更多
Somatostatinomas are extremely rare neuroendocrine tumors of the gastrointestinal tract,f irst described in the pancreas in 1977 and in the duodenum in 1979.They may be functional and cause somatostatinoma or inhibi-t...Somatostatinomas are extremely rare neuroendocrine tumors of the gastrointestinal tract,f irst described in the pancreas in 1977 and in the duodenum in 1979.They may be functional and cause somatostatinoma or inhibi-tory 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-function-ing 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 synchro-nous somatostatinomas of the duodenum and the pancreas.The condition is discussed with reference to the literature.展开更多
Somatostatinoma is a very rare neuroendocrine tumor that originates from D cells and accounts for less than 1% of all gastrointestinal endocrine tumors. The duodenum is the most frequent site for this tumor, followed ...Somatostatinoma is a very rare neuroendocrine tumor that originates from D cells and accounts for less than 1% of all gastrointestinal endocrine tumors. The duodenum is the most frequent site for this tumor, followed by the pancreas. We here describe a 46-year-old Chinese woman who developed pancreatic somatostatinoma presenting with the characteristic "inhibitory" syndrome, but the symptoms were obscure and seemingly uncorrelated. This case is also unique for its large tumor size and mixed pathological pattern. Distal pancreatectomy was performed, and the patient has remained well since operation. As the syndromes of somatostatinoma may be obscure and atypical, clinicians should review all clinical findings to obtain an accurate diagnosis. Aggressive surgery is preferred to improve the survival.展开更多
Somatostatinomas are very rare endocrine tumors, accounting for less than 1% of all gastrointestinal endocrine tumors. It usually arises within the pancreas and some from extrapancreafic sites, including the duodenum,...Somatostatinomas are very rare endocrine tumors, accounting for less than 1% of all gastrointestinal endocrine tumors. It usually arises within the pancreas and some from extrapancreafic sites, including the duodenum, ampulla, jejunum, or cystic duct,with an annual incidence of 1 in 40 million. Since the first case of pancreatic somatostafinoma was reported in 1977 by Larsson et al,4 less than 200 cases of somatostatinoma have been reported in literature, and only a few cases have been reported in China. The most described features of patients with somatostatinoma induced by excessive somatostatin secretion are characterized by cholelithiasis, steatorrbea, achlorhydria, hypochlorhydria and mild hyperglycemia, As somatostafin inhibits the secretion of insulin from pancreatic islets,展开更多
基金Supported by Fondazione Cariverona 2005, Verona (Italy) Ministero della Salute, Regione Marche e Ministero dell’Università No. PRIN 2005069205
文摘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 periampuUary 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.
文摘Somatostatinomas are extremely rare neuroendocrine tumors of the gastrointestinal tract,f irst described in the pancreas in 1977 and in the duodenum in 1979.They may be functional and cause somatostatinoma or inhibi-tory 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-function-ing 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 synchro-nous somatostatinomas of the duodenum and the pancreas.The condition is discussed with reference to the literature.
基金Project (Nos.30672072 and 30872531) supported by the National Natural Science Foundation of China
文摘Somatostatinoma is a very rare neuroendocrine tumor that originates from D cells and accounts for less than 1% of all gastrointestinal endocrine tumors. The duodenum is the most frequent site for this tumor, followed by the pancreas. We here describe a 46-year-old Chinese woman who developed pancreatic somatostatinoma presenting with the characteristic "inhibitory" syndrome, but the symptoms were obscure and seemingly uncorrelated. This case is also unique for its large tumor size and mixed pathological pattern. Distal pancreatectomy was performed, and the patient has remained well since operation. As the syndromes of somatostatinoma may be obscure and atypical, clinicians should review all clinical findings to obtain an accurate diagnosis. Aggressive surgery is preferred to improve the survival.
文摘Somatostatinomas are very rare endocrine tumors, accounting for less than 1% of all gastrointestinal endocrine tumors. It usually arises within the pancreas and some from extrapancreafic sites, including the duodenum, ampulla, jejunum, or cystic duct,with an annual incidence of 1 in 40 million. Since the first case of pancreatic somatostafinoma was reported in 1977 by Larsson et al,4 less than 200 cases of somatostatinoma have been reported in literature, and only a few cases have been reported in China. The most described features of patients with somatostatinoma induced by excessive somatostatin secretion are characterized by cholelithiasis, steatorrbea, achlorhydria, hypochlorhydria and mild hyperglycemia, As somatostafin inhibits the secretion of insulin from pancreatic islets,
