1/3. Malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease.We describe a case of malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease. A 66-year-old woman with familial von Recklinghausen's disease was admitted to our hospital with weight loss and lower abdominal pain. endoscopy revealed a submucosal tumor in the second part of the duodenum. Contrast-enhanced computed tomography showed a heterogeneous low-density tumor, measuring 20 x 15 mm, in the head of the pancreas, and metastatic lymphadenopathy. angiography showed faint tumor staining without encasement or apparent increase in vascularity. Pancreatoduodenectomy was performed. Macroscopically, there was a solid tumor, measuring 20 x 18 mm, in the head of the pancreas. Histologically, a malignant endocrine tumor was shown, with direct invasion to the duodenum, and lymph node metastases. This is the thirteenth case of pancreatic malignant tumor and the fourth case of pancreatic endocrine tumor associated with von Recklinghausen's disease reported in the world literature.- - - - - - - - - - ranking = 1keywords = von (Clic here for more details about this article) |
2/3. Neuroendocrine carcinoma of the stomach with extensive somatostatin immunoreactivity.upper gastrointestinal tract neuroendocrine tumors producing predominantly somatostatin have thus far been described only in the duodenum; their characteristic features include the frequent presence of psammoma bodies (psammomatous somatostinomas), and the association with von Recklinghausen's neurofibromatosis. Gastric neuroendocrine tumors, on the other hand, tend to display immunoreactivity to serotonin but may include small subpopulations producing gastrin, motilin, pancreatic polypeptide, and somatostatin. In this report we describe a neuroendocrine carcinoma of the stomach with rapidly fatal outcome, displaying neurosecretory granules by electron microscopy and immunoreactivity to pan-neuroendocrine markers, ie, chromogranin and neuron-specific enolase. The only neuroendocrine regulatory peptide detected in the tumor was somatostatin, identified by immunohistochemistry in the majority of neoplastic cells. In contrast with duodenal somatostinomas, there were no psammoma bodies and no demonstrable association with von Recklinghausen's neurofibromatosis. To our knowledge this appears to be the first report of a malignant neuroendocrine tumor with diffuse somatostatin immunoreactivity.- - - - - - - - - - ranking = 0.28571428571429keywords = von (Clic here for more details about this article) |
3/3. 111In-octreotide scintigraphy: a tool to select patients with endocrine pancreatic tumors for octreotide treatment?The results of octreotide scintigraphy, performed in two patients with malignant endocrine pancreatic tumors, were compared with the effect of somatostatin-14 and its analogue octreotide on hormonal levels and clinical outcome. Radiolabeled octreotide failed to demonstrate any tumor localisation in a patient with a malignant insulinoma. Nevertheless, IV injection of somatostatin and octreotide resulted in a significant decrease in peripheral insulin levels. Moreover in this patient, chronic treatment with a high dose of octreotide subcutaneously was able to transiently lower the incidence of hypoglycemic events. In a second patient with metastatic PP-oma, 111In-octreotide disclosed a pancreatic tumor in the tail of the pancreas and metastatic supraclavicular lymph nodes. In this patient IV administration of somatostatin and octreotide inhibited the hormonal secretion of the tumor but subcutaneous injection of octreotide induced hardly any decrease in plasma PP levels and failed to affect tumor growth. These observations find a possible reason for this in the heterogeneous affinity of the somatostatin receptors in endocrine pancreatic tumors. They indicate that octreotide scintigraphy alone should not be used to select patients with neuroendocrine tumors who can benefit from chronic treatment with the somatostatin analogue.- - - - - - - - - - ranking = 0.0018970767920291keywords = peripheral (Clic here for more details about this article) |