1/30. Study of a primary gastrinoma in the common hepatic duct - a case report.Primary endocrine neoplasms of the biliary tract are exceedingly rare. We report on a 60-year-old man with diarrhea, vomiting and gastroesophageal reflux disease, with a primary gastrinoma of the common hepatic duct. The tumor was positive for a variety of immunohistochemical markers. Postoperatively the patient's symptoms disappeared and in the follow-up the patient was symptom free. To our knowledge, this is the first case in the literature of a primary gastrinoma in the common hepatic duct. The fact that the common hepatic duct is not located within the gastrinoma triangle made the diagnosis difficult and the distinct localization made the surgical treatment demanding.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/30. A case of zollinger-ellison syndrome produced by gastrinoma in the duodenum accompanied with multiple endocrine neoplasia type 1.A case of zollinger-ellison syndrome produced by gastrinoma in the duodenum accompanied by multiple endocrine neoplasia type-1 (men-1) is reported. A 46 year-old female underwent distal gastrectomy due to gastric ulcer 5 years ago. As ulceration of the residual stomach recurred, further examination was performed. hyperprolactinemia, hypergastrinemia, primary hyperparathyroidism, pancreatic tumor, and duodenal carcinoid were evident, and the diagnoses of zollinger-ellison syndrome and men-1 were established. The origin of the gastrin secretion was suspected to be from the pancreatic tumor, so sampling of the portal blood was performed. As lesion on the gastrinoma in the pancreas could not be identified, total parathyroidectomy was performed for primary hyperparathyroidism. The level of the gastrin secretion, however, remained high. Partial resection of the duodenum for the duodenal carcinoid and a distal pancreatectomy were carried out concurrently. Immunohistochemical study of the anti-gastrin antibody revealed duodenal tumor cells. Initially, the gastrinoma was thought to be in the pancreas, however, the lesion accompanied with men-1 and the zollinger-ellison syndrome had occurred in the duodenum.- - - - - - - - - - ranking = 1022638.9028899keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, neoplasia (Clic here for more details about this article) |
3/30. A novel germline mutation of multiple endocrine neoplasia type 1 (MEN1) gene in a Japanese MEN1 patient and her daughter.Familial multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited disorder characterized by tumors of the parathyroid, anterior pituitary and gastro-entero-pancreatic endocrine tissues. The MEN1 gene has recently been cloned and its germline mutations have been considered to play an important role in the tumorigenesis of MEN1. We analyzed a Japanese MEN1 patient and her daughter for germline mutations of the MEN1 gene. The proband (60 y.o.) had primary hyperparathyroidism (PHP) and gastrinoma, and her daughter (30 y.o.) had prolactinoma. Clinical examinations revealed no evidence of PHP in the daughter. We identified a novel heterozygous germline mutation (712 A del) at codon 201 in exon 3 of the MEN1 gene in the proband. Restriction digestion analysis revealed the same mutation pattern in her daughter. These findings suggest that this family has familial MEN1 including a rare case of MEN1 with a single lesion of the pituitary. Genetic examinations are useful as diagnostic tools for any rare or variant case of familial MEN1.- - - - - - - - - - ranking = 1022638.9028899keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, neoplasia (Clic here for more details about this article) |
4/30. Overview of chronic diarrhea caused by functional neuroendocrine neoplasms.Eight different neoplastic disorders can cause chronic diarrhea attributable to humoral-mediated diarrhea. These include pancreatic endocrine tumor (PET) syndromes (gastrinomas, VIPomas, glucagonomas, somatostatinomas, PET's releasing calcitonin), carcinoid syndrome, medullary thyroid cancer, and systemic mastocytosis. Because these disorders are an uncommon cause of all chronic diarrheas (<1%), they are not often considered in the differential diagnosis, leading to a delay in diagnosis. This is problematic not only because all are treatable, but also because the neoplasm is frequently malignant. In this article, the characteristics and pathogenesis of the diarrhea, important clinical and diagnostic laboratory features, and treatment of each disorder are briefly reviewed, with an emphasis on recent insights.- - - - - - - - - - ranking = 5keywords = neoplasm (Clic here for more details about this article) |
5/30. Large goiter and multiple rib tumors.We report an interesting case of a 47-yr-old who had a large goiter and multiple rib tumors. The patient was initially suspected of having thyroid cancer, which had metastasized on the ribs, based on imaging studies. However, laboratory tests revealed a high level of ionized calcium and parathyroid hormone (PTH). The large goiter was diagnosed as having parathyroid tumors owing to the high level of PTH in the tissue fluid. The biopsy specimen from a rib tumor was diagnosed as containing brown tumors associated with primary hyperparathyroidism (PHP). The patient also had prolactinoma and pancreatic gastrinoma. Her daughter had both prolactinoma and PHP, and her brother and her father had PHP. Thus, the patient was diagnosed as having multiple endocrine neoplasia type 1.- - - - - - - - - - ranking = 204527.78057799keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, neoplasia (Clic here for more details about this article) |
6/30. Bilateral pituitary adenomas occurring with multiple endocrine neoplasia type one.We report a case of synchronous bilateral pituitary adenomas in a patient with multiple endocrine neoplasia type one (MEN1). The patient was previously known to have a pancreatic gastrinoma and had first-degree relatives with MEN1. Both adenomas were concurrently revealed by high-resolution MR imaging of the pituitary gland as part of the investigation of the patient's severe, persistent headaches and elevated serum prolactin level.- - - - - - - - - - ranking = 1022638.9028899keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, neoplasia (Clic here for more details about this article) |
7/30. Development of an inveterate gastroduodenal ulcer caused by antral G-cell hyperplasia of the stomach (pseudo-zollinger-ellison syndrome): report of a case.We describe herein the case of a 54-year-old Japanese woman in whom an inveterate peptic ulcer developed in association with pseudo-zollinger-ellison syndrome (pseudo-ZES). The patient presented with weight loss and abdominal distension caused by antral and duodenal stenosis due to an inveterate peptic ulcer. Her serum gastrin level was very high; however, no evidence of a gastrinoma or carcinoid tumor was detected by preoperative examinations or surgery. A total gastrectomy and double-tract reconstruction was performed, and pathological examination revealed a gastric ulcer (UL-IV) with no histopathological evidence of a neoplasm. Immunohistochemical staining showed an obvious increase in the number of endocrine cells that were positive for chromogranin a, and marked G-cell hyperplasia was observed in the antral mucosa. Furthermore, the number of enterochromaffin-like cells was remarkably high. From the results of the immunohistochemical examination, the patient was diagnosed as having hypergastrinemia due to antral G-cell hyperplasia. Postoperatively, the patient's serum gastrin level fell rapidly to within the normal range, her nutritional status improved, and her weight increased by about 10 kg within 1 year.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/30. Ovarian gastrinoma in multiple endocrine neoplasia type I: a case report.We report a very rare case of ovarian gastrinoma in the context of multiple endocrine neoplasia type I, including primary hyperparathyroidism and zollinger-ellison syndrome. somatostatin receptor scintigraphy revealed the ovarian involvement at an early stage. Oophorectomy led to the final diagnosis and complete healing.- - - - - - - - - - ranking = 1022638.9028899keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, neoplasia (Clic here for more details about this article) |
9/30. A malignant gastrointestinal stromal tumour in a patient with multiple endocrine neoplasia type 1.loss of heterozygosity for polymorphic markers flanking the multiple endocrine neoplasia type 1 (men-1) gene in parathyroid and pancreatic islet tumours from subjects with men-1 has been well documented and has led to the hypothesis that the men-1 gene functions as a recessive tumour suppressor gene. We report a case of men-1 with duodeno-pancreatic gastrinoma, parathyroid hyperplasia, pituitary adenoma, adrenal adenoma, and lipomas, whose rare association with a malignant gastrointestinal stromal tumour (GIST) represents an undescribed combination. men-1 mutation in this family was shown as a frameshift (1607delA) in exon 10. To assess the role of the men-1 gene in the pathogenesis of tumours less commonly associated with men-1, we studied GIST dna for loss of the unaffected men-1 gene allele. Stromal tumour and peripheral leucocyte DNAs from our patient were examined for loss of heterozygosity using the PYGM microsatellite polymorphism and an intragenic polymorphism (D418D in exon 9) in the men-1 gene. We showed no evidence for loss of the wild-type men-1 allele in GIST. The men-1 germline inactivating mutation 1607delA-ter558 in exon 10 was detected in the stromal tumour dna, but no somatic mutation in the wild-type men-1 allele in GIST dna was detected. Occurrence of GIST could be consistent with the possibility that this men-1-related uncommon neoplasm arose independently by a mechanism unrelated to the men-1 gene.- - - - - - - - - - ranking = 585813.90085619keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, neoplasia, neoplasm (Clic here for more details about this article) |
10/30. Concurrent parathyroid adenomas and carcinoma in the setting of multiple endocrine neoplasia type 1: presentation as hypercalcemic crisis.We describe a patient with multiple endocrine neoplasia type 1 characterized by the simultaneous occurrence of parathyroid cancer, parathyroid adenomas, and pancreatic gastrinoma, who presented with an episode of acute hypercalcemia. The rapid parathyroid hormone assay provided a basis for the diagnosis of parathyroid hyperfunction. Mediastinal metastasis of the parathyroid carcinoma was found at autopsy. However, the staining of pancreatic and gastric tissue for parathyroid hormone-related protein does not make it possible to exclude completely the contribution of this peptide in mediating the hypercalcemia. To our knowledge, this is the first reported case of parathyroid carcinoma as part of the multiple endocrine neoplasia type 1 syndrome.- - - - - - - - - - ranking = 1095727.0472814keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, neoplasia (Clic here for more details about this article) |
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