1/45. Malignant pancreatic tumour within the spectrum of tuberous sclerosis complex in childhood. A 12-year-old boy with tuberous sclerosis complex (TSC) presented with a large retroperitoneal tumour. Exploratory surgery revealed an infiltrative tumour originating from the pancreas, with local metastases to the lymph nodes. The histologal diagnosis was a malignant islet cell tumour. Retrospectively measured pancreatic hormone levels, however, were normal. A connection between the malignancy and TSC was demonstrated by loss of heterozygosity of the TSC2 gene in the tumour. The primary mutation Q478X in this patient was identified in exon 13 of the TSC2 gene on chromosome 16. CONCLUSION: Pancreatic islet cell tumours have been mainly associated with multiple endocrine neoplasia syndrome type 1. In our case we demonstrate a direct relationship of this tumour to tuberous sclerosis complex, in the absence of further signs of multiple endocrine neoplasia syndrome type 1. ( info) |
| 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. ( info) |
3/45. 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. ( info) |
4/45. octreotide treatment for a malignant islet cell tumor with variable hormone secretion: case report. It is well known that an islet cell tumor can secrete multiple hormones depending on its cell type. We report the case of a 70-year-old woman who initially presented with peptic ulcer symptoms, an elevated serum gastrin level, and multiple liver tumors. liver biopsy and distal pancreatectomy were performed, and the pathological diagnosis was malignant islet cell tumor. Additionally, the immunohistochemical staining revealed scattered positivity for gastrin, and then also positivity for insulin 14 months later. A subsequent hypoglycemic episode and elevated serum gastrin and insulin levels suggested that the disease had developed into a condition of multiple hormone secretion. The plasma gastrin and insulin levels decreased from 584 pg/ml and 90.8 microIU/ml to 49.1 pg/ml and 20.9 microIU/ml, respectively, 5 days after treatment with subcutaneous octreotide 100 micrograms every 6 to 8 hours. In addition, follow-up computed tomography showed shrinkage of the metastatic liver tumors. In conclusion, we found a case of malignant islet cell tumor with variable hormone secretion which could be effectively controlled with octreotide. ( info) |
5/45. Pancreatic serous cystadenoma associated with islet cell tumour. We report the case of a 29-year-old female patient with a diffuse type of serous cystadenoma involving the entire pancreas except for part of the head, which was replaced by islet cell tumour. Ultrasound and CT showed multiple cysts in the entire pancreas and a solid mass with calcification in the head. MRI characterized the fluid content of the cysts and the extent of disease. ( info) |
6/45. 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. ( info) |
| A 55-year-old man with nonfunctioning islet cell carcinoma showing elevation of serum carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) levels is described with genetic analyses. Pathological examination of the resected specimen revealed two independent islet cell carcinomas, one in the body and the other in the tail of the pancreas. It was proved immunohistochemically that the tumor cells, particularly those in the tail, were immunoreactive to CEA and CA 19-9 and had the property of duct cells, as well as endocrine cells. Gastrin was demonstrated immunohistochemically in these tumor cells, although its level in serum was not elevated. Genetic analyses of the fresh specimens from the tumor in the body revealed K-ras codon 12 mutation and microsatellite instability. These findings are consistent with its progressive clinical course and strongly suggest that these tumors originate, not from the islet cells of Langerhans, but from protodifferentiated cells, capable of giving rise to all the pancreatic cell types. ( info) |
8/45. Nonfunctioning pancreatic tumor manifestated as abdominal mass in a child. This report concerns a 9-year-old girl with a nonfunctioning pancreatic tumor presented as an abdominal mass. An encapsulated tumor, measuring approximately 8 x 7 cm, was found during operation, and debulking surgery was done. Histopathologic studies proved that it was a pancreatic tumor, compatible with non-functioning islet cell tumors. She displayed neither clinical picture of jaundice, nor excessive hormonal manifestations such as diarrhea or flushing. No evidence would suggest its association with multiple endocrine neoplasia type I. To our knowledge, it has never been reported in children in taiwan. ( info) |
9/45. Acinar-islet cell tumor of the pancreas: report of a malignant pancreatic composite tumor. An unusual case of malignant pancreatic composite tumor with both components of acinar cell tumor (ACT) and islet cell tumor (ICT) was investigated histologically, immunohistochemically, and ultrastructurally. The pancreatic tumor with central cyst formation was found on computerized tomographic examination of a 72-year-old man reporting appetite and weight loss. The ACT component was present in the original pancreatic region and the ICT region was adjacent to the ACT. ACT was immunohistochemically positive for pancreatic amylase, whereas ICT had argyrophil tumor cells immunohistochemically positive for chromogranin a. There were several tumor cell nests positive for both pancreatic amylase (acinar differentiation) and chromogranin a (islet differentiation). We speculated that ICT may have arisen from the de-differentiated tumor cells in the ACT after the occurrence of ACT. ( info) |
10/45. Resection of a non-functioning islet cell carcinoma occupying the entire pancreas. A 46-year-old woman with jaundice was found to have enlargement of the entire pancreas on computed tomography. endoscopy revealed a tumor at the papilla of Vater, although examination of biopsy specimens did not demonstrate a malignancy. The jaundice resolved spontaneously, but reappeared at 7 months. Also noted was increased swelling of the papilla. The serum pancreatic endocrine and exocrine hormone concentrations were within the reference ranges. At surgery, a hard mass was found occupying the entire pancreas. Examination of incisional biopsy specimens from the pancreatic mass and papilla of Vater revealed an islet cell tumor. A total pancreatectomy was performed. The tumor extended from the pancreatic tail to the papilla of Vater. Histopathologic and immunopathologic examination revealed a non-functional islet cell carcinoma. There was no evidence of recurrence at 3 years. A good outcome can be achieved with resection of non-functioning islet cell carcinomas, even if they are advanced. ( info) |