1/22. 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.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/22. 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 = 6keywords = endocrine (Clic here for more details about this article) |
3/22. 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 = 5keywords = endocrine (Clic here for more details about this article) |
4/22. Nonfunctioning islet cell carcinoma of the pancreas with high serum CEA & CA19-9, K-ras codon 12 mutation, and microsatellite instability.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.- - - - - - - - - - ranking = 0.5keywords = endocrine (Clic here for more details about this article) |
5/22. 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.- - - - - - - - - - ranking = 0.5keywords = endocrine (Clic here for more details about this article) |
6/22. 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.- - - - - - - - - - ranking = 0.5keywords = endocrine (Clic here for more details about this article) |
7/22. parathyroid hormone-related protein induced coupled increases in bone formation and resorption markers for 7 years in a patient with malignant islet cell tumors.parathyroid hormone-related protein (PTHrP) and PTH share the common PTH/PTHrP receptor. Although an elevated level of circulating PTHrP in patients with malignancies causes hypercalcemia as does PTH, chronic and systemic effects of PTHrP on bone metabolism in humans are not well understood because tumor-burden patients showing hypercalcemia usually have a poor prognosis. We investigated bone and calcium metabolism in a patient with malignant islet cell tumors showing hypercalcemia due to the elevated plasma PTHrP level for 7 years. hypercalcemia and hypercalciuria continued throughout the clinical course in spite of frequent infusions of bisphosphonates. bone resorption markers and a bone formation marker were consistently elevated as seen in primary hyperparathyroidism, a disease caused by an autonomous hypersecretion of PTH. Based on biochemical measurements including bone markers and serum 1,25-dihydroxyvitamin D, the clinical features of this case essentially are the same as those of primary hyperparathyroidism except for the elevated level of plasma PTHrP with suppressed intact PTH level. Therefore, it is suggested that chronic and systemic effects of PTHrP on bone as well as calcium metabolism are indistinguishable from those of PTH in human.- - - - - - - - - - ranking = 0.00060848647045338keywords = bone (Clic here for more details about this article) |
8/22. ACTH-secreting islet cell tumor of the pancreas presenting as bilateral ovarian tumors and Cushing's syndrome.A 41-year-old woman presented with hirsutism, a pelvic mass, and Cushing's syndrome. Imaging studies revealed bilateral ovarian masses and a solid and cystic mass within the pancreas. Partial pancreatectomy, bilateral oophorectomy, and excision of several peritoneal tumor nodules were performed. Pathological examination revealed a neuroendocrine islet cell tumor of the pancreas with bilateral ovarian metastases. The tumor was immunoreactive for ACTH, chromogranin, neuron-specific enolase, and keratin. The patient received postoperative chemotherapy and has been disease-free for 6 years. To our knowledge, this is the first reported case of an ACTH-secreting pancreatic neuroendocrine tumor presenting as bilateral ovarian metastases.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
9/22. Gastric carcinoma resembling pancreatic mixed acinar-endocrine carcinoma.A case of gastric carcinoma resembling pancreatic mixed acinar-endocrine carcinoma of 77-year-old female is presented. This type of gastric tumor has not been previously reported. The endoscopic mucosal resection specimen of the fundus contained a 1.2 x 0.9 x 0.3 cm, well-circumscribed, tan, soft nodular tumor with protruded configuration with a central recess. Histologically, the tumor was confined to the mucosa and submucosa and was characterized by three growth patterns; acinar, solid, and glandular. The growth patterns were intermingled. The tumor cells in the acinar component had round nuclei with prominent nucleoli and diastase-resistant, periodic acid-Schiff-positive, eosinophilic cytoplasm. Immunohistochemically, the tumor cells were positive for CAM5.2, cytokeratin (CK) 7, CK 20, trypsin, lipase, alpha-1-antitrypsin, and alpha-1-antichymotrypsin. The tumor cells in the solid component were positive for Grimelius stain and chromogranin a. The findings indicated that the tumor showed acinar and endocrine differentiation. There was no heterotopic pancreas tissue in the specimen. The patient was well without tumor at the 7-month follow-up. It is important to know the existence of this type of gastric cancer and to not confuse it with a metastatic lesion of the pancreatic origin.- - - - - - - - - - ranking = 3keywords = endocrine (Clic here for more details about this article) |
10/22. 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 = 4.5keywords = endocrine (Clic here for more details about this article) |
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