1/13. Mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with zollinger-ellison syndrome: an autopsy case with a 24-year survival period.We report an autopsy case of mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with zollinger-ellison syndrome. A 38-year-old Japanese male was found to have zollinger-ellison syndrome and pancreatic gastrinoma, and gastrectomy and resection of the pancreatic tumor were performed. However, hypergastrinemia persisted, and the patient died of disseminated carcinomatosis at 62 years of age, 24 years after the onset of zollinger-ellison syndrome. At autopsy, the main tumor was present in the residual pancreas, and metastases were noted in many organs. In the pancreas and other organs, ductal and endocrine carcinoma areas were mixed and there was a gradual transition between the two. No acinar differentiation was noted. The ductal elements were positive for mucins and carcinoembryonic antigen but negative for neuroendocrine markers, while endocrine elements were positive for chromogranin a and synaptophysin and to a lesser extent for gastrin, but negative for mucins and carcinoembryonic antigen. The ductal elements comprised about 30% of the tumor cells, and endocrine elements 70%. According to the revised world health organization classification, our case was diagnosed as mixed ductal-endocrine carcinoma. Our case is rare because the tumor manifested as gastrinoma with zollinger-ellison syndrome and the patient survived for 24 years. To the best of our knowledge, no such case has been reported. Our case suggests that pancreatic endocrine tumors may evolve into mixed ductal-endocrine carcinomas.- - - - - - - - - - ranking = 1keywords = ductal (Clic here for more details about this article) |
2/13. Mixed ductal-endocrine carcinoma of the pancreas: a possible pathogenic mechanism for arrhythmogenic right ventricular cardiomyopathy.We describe herein a case of a mixed ductal-endocrine pancreatic carcinoma. Rare cases of mixed pancreatic tumors have been described, with endocrine and exocrine components each making up a significant proportion of the neoplasm; to our knowledge, only one case has been reported with a mixed liver metastasis. In our case, ductal and endocrine cells were intimately admixed in the primary tumor and in a peripancreatic lymph node metastasis, diagnosed by standard light microscopy and double immunostaining for cytokeratin 19 and synaptophysin. The endocrine component was immunoreactive for somatostatin. Tumors with admixed endocrine and exocrine components support the hypothesis of a common endodermal histogenesis for the ductal and endocrine cells in the human pancreas.- - - - - - - - - - ranking = 0.72761979984046keywords = ductal, neoplasm (Clic here for more details about this article) |
3/13. Hepatoid carcinoma of the pancreas.BACKGROUND: The majority of primary extrahepatic neoplasms exhibiting features of hepatocellular carcinoma in terms of morphology, immunohistochemistry, and behavior have been described in the stomach. To the authors' knowledge only a few cases have involved other organ sites. They frequently are associated with other histologic type tumors such as adenocarcinoma, and portend an aggressive behavior. methods: Two examples of hepatoid carcinoma arising from the pancreas are reported in the current study. RESULTS: One case was a malignant islet cell tumor with a full-blown clinical syndrome of glucagon overproduction, histologic evidence of hepatocytic differentiation, bile production, and alpha-fetoprotein (AFP) positivity. The second tumor was a ductal carcinoma showing periodic acid-Schiff positive and diastase-resistant hyaline globules, AFP production, and ultrastructural resemblance to hepatocytic cells. CONCLUSIONS: The rare observation of cellular phenotypic transformation that corresponds with the process of hepatocytic transdifferentiation of pancreatic cells demonstrated in animal models and the common embryologic foregut derivation of the pancreas and liver also may explain the phenomenon of pancreatic hepatoid carcinoma.- - - - - - - - - - ranking = 0.12761979984046keywords = ductal, neoplasm (Clic here for more details about this article) |
4/13. Microcytic adenoma coexistent with low-grade malignant islet cell tumor of the pancreas.We report a case of microcystic (glycogen-rich) adenoma of the whole pancreas with coexistent pancreatic low-grade malignant islet cell tumor in a 29-year-old woman. She complained of nausea, vomiting, and growing abdominal mass. Abdominal computed tomography showed multiple cysts in the whole pancreas and a calcified solid mass in the pancreatic head. A Whipple's operation and total pancreatectomy with splenectomy was performed to treat pancreatic cystic neoplasm. The pancreas was entirely replaced by variable-sized, multilocular cysts, which were lined by a flattened-to-cuboidal glycogen-rich epithelium. Furthermore, in the head of the pancreas, a focal yellowish solid mass showed a positive reaction for chromogranin a and neuron-specific enolase. Careful examination of the pancreas is warranted in cases of microcystic adenoma to rule out a possible coexistent pancreatic malignancy.- - - - - - - - - - ranking = 0.027619799840462keywords = neoplasm (Clic here for more details about this article) |
5/13. Nonfunctioning islet cell carcinoma of the pancreas: case report.Islet cell carcinomas have an incidence of 5 per million per year; 50 per cent of these are nonfunctioning islet cell tumors. The presenting symptoms mimic pancreatic ductal adenocarcinoma. The CT finding of a pancreatic head mass that spares the main duct may distinguish between the two. The treatment of choice is resection. Most nonfunctioning islet cell tumors are not discovered until metastases are present. However, favorable survival rates have been reported in locally advanced tumors that have undergone resection. liver metastases carry an unfavorable prognosis. Five-year survival over 60 per cent has been reported. Node-negative patients have a median survival of more than 10 years, and node-positive patients who have undergone resection have a median survival of 75 months. Streptozotocin and 5-fluorouracil are used postoperatively in patients with advanced disease. Considering the favorable survival with resection aggressive surgical treatment is mandated in cases of nonfunctioning islet cell tumors.- - - - - - - - - - ranking = 0.1keywords = ductal (Clic here for more details about this article) |
6/13. Minute mixed ductal-endocrine carcinoma of the pancreas with predominant intraductal growth.We report a rare case of minute (5 mm x 4 mm) mixed ductal-endocrine carcinoma of the pancreas with predominant intraductal growth. A 34-year-old Japanese man was admitted because of elevated serum pancreatic enzymes. Endoscopic retrograde pancreatography revealed an unidentified material of 18 mm within the main pancreatic duct. Stone or parasite with acute pancreatitis was suspected clinically, and the biopsy revealed malignant cells positive for CA19-9, carcinoembryonic antigen (CEA) and synaptophysin. No apparent tumor was identified in the pancreas by various imaging techniques. Resection of pancreatic body and tail was performed. Grossly, the main pancreatic duct in the pancreatic body was occluded by as much as 20 mm. The pancreas had minute carcinoma of 5 mm x 4 mm just around the occluded main pancreatic duct. The tumor cells invaded the main pancreatic duct and spread within it as long as 20 mm. Histologically, the carcinoma had biphasic pattern; one was ductal carcinoma with tubular formations and another was carcinoma with neuroendocrine features. These two elements were admixed, and the ductal element comprised 30% while the endocrine element comprised 70%. The ductal element was immunoreactive for cytokeratins, CEA and CA19-9, while the endocrine element was immunoreactive for chromogranin a and synaptophysin. No immunoreactivity for pancreatic enzymes was noted. Ultrastructural observations showed dense core granules and no zymogen granules. Our case is unique clinically in that the tumor manifested as an intraductal material and no apparent tumor was found by imaging modalities, and pathologically in that the tumor was rare mixed ductal-endocrine carcinoma and the tumor was very small and mainly grew within the main pancreatic duct.- - - - - - - - - - ranking = 1.5keywords = ductal (Clic here for more details about this article) |
7/13. Malignant pancreatic endocrine tumor in a child with tuberous sclerosis.tuberous sclerosis complex (TSC) is an autosomal dominant condition whose signs and symptoms may vary from a few hypopigmented skin spots to epilepsy, severe mental retardation, and renal failure. The disease is caused by mutations in either TSC1 or TSC2 gene, at chromosome 9q34 and 16p13.3. Inactivation of both alleles at TSC1 or TSC2 loci is associated with the development of hamartomas in different organs, and only rarely with malignant neoplasms. In this study we present a 6-year-old boy with TSC and with a malignant islet cell tumor of the pancreas. mutation analysis of dna extracted from peripheral blood cells of the patient identified an R1459X de novo mutation in exon 33 of the TSC2 gene. Immunohistochemical analysis with anti-tuberin antibodies on paraffin-embedded tissue sections showed loss of tuberin immunostaining in tumor cells but normal expression in residual normal pancreas. dna analysis of tumor and normal cells showed chromosome 16p13 loss of heterozygosity in malignant pancreatic islet cell tumor but not in normal pancreas. These findings suggest a role for tuberin, the TSC2 gene product, in the pathogenesis of malignant pancreatic endocrine tumor.- - - - - - - - - - ranking = 0.027619799840462keywords = neoplasm (Clic here for more details about this article) |
8/13. Intraductal growth of a nonfunctioning endocrine tumor of the pancreas.Intraductal growth of nonfunctioning endocrine tumors of the pancreas may be very rare, and our survey of literature shows only two cases have been described. We report a case of a 43-year-old man with a nonfunctioning endocrine tumor of the pancreas that uniquely grew within the lumen of the main pancreatic duct (MPD) without ductal involvement and completely obstructed the MPD. Endoscopic ultrasonography (EUS) and endoscopic retrograde cholangiopancreatography (ERCP) were very helpful to delineate the intraductal growth of the tumor and to determine the resection line of the pancreas. A nonfunctioning pancreatic endocrine tumor is important to consider on differential diagnoses when complete obstruction of the MPD is demonstrated on ERCP. It is speculated that the tumor originated from precursor cells of the pancreatic duct or islet cells adjacent to the MPD and slowly proliferated within the lumen of the MPD.- - - - - - - - - - ranking = 0.7keywords = ductal (Clic here for more details about this article) |
9/13. Mixed exocrine-endocrine tumor of the pancreas.CONTEXT: neoplasms of the pancreas usually show ductal, acinar or endocrine differentiation. Tumors with mixed exocrine and endocrine components are unusual. We herein describe a case of a mixed ductal-endocrine tumor. CASE REPORT: A 65-year-old woman was referred to our department with a diagnosis of carcinoma of the tail of the pancreas. The patient had a short history of upper abdominal pain, nausea and melena. Upper gastrointestinal endoscopy revealed gastric fundus varices and CT scan demonstrated an inhomogeneous tumor located in the tail of the pancreas infiltrating the spleen and the splenic vein. The patient underwent distal pancreatectomy and splenectomy, and had an uneventful recovery. Pathological examination revealed a mixed ductal-endocrine tumor. The endocrine component was immunoreactive for glucagon, gastrin and somatostatin, and non-reactive for insulin. CONCLUSIONS: Because of the rarity and unpredictable biologic behavior of these tumors, the need for adjuvant therapy has not yet been well-defined. The patient has had a follow-up CT scan every six months, and one and a half years later remains disease free.- - - - - - - - - - ranking = 0.3keywords = ductal (Clic here for more details about this article) |
10/13. Multiple intracerebral metastases from an islet cell carcinoma of the pancreas: case report.Computed tomography and magnetic resonance imaging of an 85-year-old woman with bitemporal headaches revealed cystic lesions in the basal ganglia and cerebellum. The patient remained neurologically asymptomatic until just before her death at age 89. autopsy revealed tumors of the brain, lungs, liver, periaortic lymph nodes, adrenal gland, and the pancreas. light microscopy demonstrated a malignant small cell tumor with scant cytoplasm. Electron microscopy revealed intracytoplasmic dense core vesicles, characteristic of neuroendocrine tumors, which prompted the diagnosis of islet cell carcinoma. All lesions were found to be microscopically similar. Previous reports of islet cell carcinoma metastatic to the brain could not be found. This case emphasizes the importance of electron microscopy in cases of small cell neoplasms in the brain.- - - - - - - - - - ranking = 0.027619799840462keywords = neoplasm (Clic here for more details about this article) |
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