1/619. pancreatic polypeptide hyperplasia causing watery diarrhea syndrome: a case report.Neuroendocrine tumours of the pancreas can secrete numerous peptides, leading to various recognizable clinical syndromes. The secretion of pancreatic polypeptide has been used as a marker for neuroendocrine tumours but is considered to be a biologically inert peptide. A 37-year-old woman had watery diarrhea syndrome from pancreatic polypeptide hyperplasia. Only 2 other reported cases in the literature have described pancreatic polypeptide hyperplasia; however, this is the first reported case in which the patient was successfully treated by surgical resection, with a 2-year follow-up. This report and review of the literature illustrate that pancreatic polypeptide hypersecretion may present as a clinical endocrinopathy.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/619. 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) |
3/619. Gastroenteropancreatic neuroendocrine tumor metastases to the thyroid gland: differential diagnosis with medullary thyroid carcinoma.neuroendocrine tumors (NET) of the thyroid gland are rare. Apart from medullary thyroid carcinoma (MTC), metastases of gastroenteropancreatic (GEP) NET may also occur. Features of six patients (five men, one female: age range, 39-67 years) with thyroid metastases from a GEP-NET are described. Thyroid metastases were bilateral in all patients and were associated with enlarged neck lymph nodes in five. In four cases, the thyroid tumor was either the first sign of the disease (n = 2) or was an isolated site of recurrence (n = 2). The tumors were well (n = 3) or poorly differentiated (n = 3). Five tumors for which the primary site could be determined corresponded to foregut-derived tumors (3 lungs, 1 thymus and 1 pancreatic NET). One tumor demonstrated calcitonin (CT) production as shown by immunohistochemistry and elevated plasma CT levels. However, the disease history and the clinical features strongly favored a metastasizing GEP-NET. No tumoral RET proto-oncogene mutation was found in this patient. The differential diagnosis between metastatic GEP-NET and MTC is crucial because prognosis, work-up, and treatment differ greatly.- - - - - - - - - - ranking = 2.5keywords = endocrine (Clic here for more details about this article) |
4/619. Benign pancreatic tumor treated with duodenum-preserving resection of the head of the pancreas. Case report.Cystic neoplasms of the pancreas constitute about 9% of all cystic lesions of the pancreas and less than 1% of all pancreatic neoplasms. Authors report the case of a 70 year-old woman with microcystic cystadenoma. Computed tomography (CT) scan of the abdomen diagnosed a 5 cm multilocular septated cyst, with calcifications in the context, localized in the head-uncinate process of the pancreas. The mass was well separated by a sharp cleavage plane with portal vein and superior mesenteric vessels. An endoscopic retrograde cholangiopancreatography (ERCP) showed cephalic symmetrical stenosis (diameter: 3 mm) of the main pancreatic duct (MPD), mildly dilated in the remaining tract (diameter: 6 mm). An intra-operative biopsy of the cystic wall had been performed. Therefore, it was decided to proceed with a duodenum-preserving resection of the head of the pancreas (DPPHR), including stenosis tract of the MPD in the surgical specimen. The reconstructive procedure consisted, by i.v. jejunal loop transposition, of a side-to-side pancreatico-jejunostomy, including in the anastomosis both corpocaudal stump and the resection cavity of the pancreatic head, and an end-to-side Roux-en-Y jejuno-jejunostomy. With respect to long-lasting pain relief and preservation of the endocrine and exocrine functions of the pancreas, duodenum-preserving resection of the head of the pancreas is a highly effective surgical procedure with low early and late morbidity and mortality due to limited surgical resections. This technique, introduced into surgical practice in 1972 by Beger, is indicated in patients with chronic pancreatitis with an inflammatory mass in the head of the pancreas. The authors conclude that this procedure can also be performed in cases of pancreatic benign tumors, such as microcystic cystadenoma. Advantages of this technique make DPPHR an attractive alternative to pylorus-preserving pancreatico-duodenectomy (PPPD).- - - - - - - - - - ranking = 0.5keywords = endocrine (Clic here for more details about this article) |
5/619. Pancreatoduodenectomy for neonatal myofibromatosis of the pancreas.myofibromatosis is a rare congenital disorder consisting of one or more fibrous nodules in the skin, soft tissues, bones, and internal organs. The authors report the unique case of a newborn who presented with obstructive jaundice caused by a single myofibroma in the head of the pancreas that was treated successfully by pancreatoduodenectomy on the eighth day of life.- - - - - - - - - - ranking = 9.2212444249965E-5keywords = bone (Clic here for more details about this article) |
6/619. US and CT findings of multicentric leiomyosarcomatosis.This article presents a case of leiomyosarcomatosis with widespread lesions involving the soft tissues and the most unlikely organs such as thyroid and salivary glands, pancreas, ligamentum teres, bladder wall, and bones without lymph node or distant metastasis. The CT and US findings of this rare phenomenon are discussed with regard to the literature.- - - - - - - - - - ranking = 9.2212444249965E-5keywords = bone (Clic here for more details about this article) |
7/619. Solid-pseudopapillary tumor of the pancreas--a rare and frequently misdiagnosed neoplasm.INTRODUCTION: We describe a young woman with an unusual pancreatic tumor. FINDINGS AND DISCUSSION: Intraoperatively, a smoothly demarcated and encapsulated tumor was exposed. It was large (5 cmx4 cm) and of solid consistency, with a small stalk attached to the uncinate process. The tumor was partially surrounded by the pancreatic head. The macroscopic appearance suggested a benign tumor. frozen sections revealed a benign pancreatic tumor, most likely of endocrine nature. Based on these findings, tumor enucleation was performed. The patient recovered rapidly from the intervention and was discharged from hospital after 2 weeks. One year after surgical treatment, the patient is without recurrence. The final diagnosis of the tumor was a solid pseudo-papillary tumor.- - - - - - - - - - ranking = 0.5keywords = endocrine (Clic here for more details about this article) |
8/619. Cytogenetic and CGH studies of four neuroendocrine tumors and tumor-derived cell lines of a patient with multiple endocrine neoplasia type 1.A malignant insulinoma (LOHG-I), a carcinoid of the lung (LOHG-L), a parathyroid adenoma (LOHG-NSA), and a fibroma (LOHG-F) were obtained from a patient with multiple endocrine neoplasia type 1 (MEN1). Long-term cultures were established. Essential neurobiological properties of the cell lines were proven immunocytochemically and by electron microscopy. Molecular analysis of the germline dna showed a 4 bp deletion in exon 3 of the MEN1 gene. Cytogenetic and CGH analyses of the tumors/tumor cell lines revealed diploidy and balanced and unbalanced structural aberrations different for each tumor. chromosomes 6q21, 11q and 17q were most frequently involved in clonal structural aberrations.- - - - - - - - - - ranking = 4.5keywords = endocrine (Clic here for more details about this article) |
9/619. Tumor-to-tumor metastasis to follicular variant of papillary carcinoma of thyroid.OBJECTIVE: To describe and document tumor-to-tumor metastases in the thyroid gland. methods AND RESULTS: In this series we describe 3 cases of tumor-to-tumor metastasis in which the recipient tumor was a follicular variant of papillary thyroid carcinoma. The donor tumors and sites were small cell carcinoma of the lung, neuroendocrine carcinoma probably of pancreatic origin with initial presentation as liver metastasis, and clear cell carcinoma of the kidney with metastasis to liver and pancreas. The donor tumor cells infiltrated the substance of the follicular variant of papillary thyroid carcinoma, the nontumorous thyroid parenchyma, and the lymphovascular spaces. Small cell carcinoma and neuroendocrine carcinoma showed positive reactivity for neuroendocrine markers and were negative for thyroglobulin and calcitonin. The follicular variant of papillary thyroid carcinoma showed positivity with thyroglobulin and cytokeratin 19. CONCLUSIONS: Although tumor-to-tumor metastases in thyroid gland are exceedingly rare, one should be aware of this phenomenon as the metastatic lesion may simulate a thyroid primary. history of a previous tumor and immunohistochemical stains can be helpful in distinguishing between primary and metastatic thyroid neoplasms.- - - - - - - - - - ranking = 1.5keywords = endocrine (Clic here for more details about this article) |
10/619. Treatment of metastatic glucagonoma to the liver: case report and literature review.glucagonoma, a rare neuroendocrine pancreatic tumour, is frequently malignant and often accompanied by hepatic metastases. Our aim was to consider the different treatments of metastatic glucagonoma to the liver and their results. A case of glucagonoma with metachronous, small, multiple and bilobar liver metastases is reported. Combined treatment with octreotide and hepatic arterial chemoembolization was applied with good results in terms of symptom relief, plasma glucagon levels and regression of hepatic metastases. survival rates were also improved. Based on our experience, glucagonoma with metachronous, multiple, diffuse and bilobar hepatic metastases should be treated with octreotide plus hepatic arterial chemoembolization with improved outcome and prognosis.- - - - - - - - - - ranking = 0.5keywords = endocrine (Clic here for more details about this article) |
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