1/25. Psychoneuroendocrine aspects of temporolimbic epilepsy. Part III: case reports.Many reproductive steroids have neuroactive effects that can modulate neuronal excitability and influence emotions. Emotional disorders may result when 1) abnormal endocrine states interact with normal brain, 2) normal endocrine states interact with abnormal brain, and 3) abnormal endocrine states interact with abnormal brain. An understanding of these pathogenetic relationships and the potential therapeutic role of reproductive hormones should lead to a more effective and comprehensive management of women and men with anxiety and mood disorders.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
2/25. Endocrine disorders in two sisters affected by melas syndrome.A variety of endocrine and metabolic defects, including hypothalamopituitary hypofunction and diabetes mellitus, has been reported in association with mitochondrial disorders. We describe two sisters affected by mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome in whom dna analysis showed an A-->G transition at the 3243rd nucleotide position on the transfer RNALeu(UUR) gene with 65% and 45% of mutant-type mitochondrial dna present in the blood cells of the younger and the older sister, respectively. The younger sister had severe involvement of the central nervous system with mental retardation, epilepsia partialis continua, and strokelike episodes. Endocrine investigations showed an extensive neuroendocrine dysfunction with growth hormone deficiency, hypothalamopituitary hypothyroidism, prepubertal gonadotropin levels, and absence of any secondary sexual characteristics at the age of 12 6/12 years. The neurologically normal older sister was affected by diabetes mellitus and had normal hypothalamopituitary function. Our report confirms that the endocrine system can be affected differently by the same mitochondrial dna mutation, depending on the heteroplasmia phenomenon. A complete endocrine evaluation must be performed in patients affected by mitochondrial disease and the existence of a mitochondrial disorder should be taken into account in patients with endocrine abnormalities, even if neuromuscular signs are lacking.- - - - - - - - - - ranking = 0.25keywords = neuroendocrine (Clic here for more details about this article) |
3/25. A neuroendocrine cause of oncogenic osteomalacia.All definite cases of oncogenic osteomalacia have, until now, been classified as mesenchymal tumours. We report here a case of oncogenic osteomalacia caused by a spinal tumour. Microscopically, it resembled the mixed connective tissue variant of previously described phosphaturic tumours. Immunohistochemical studies, however, showed the tumour cells to be positive for low molecular weight cytokeratin (CAM 5.2), S100 protein, PGP 9.5, and synaptophysin. Electron microscopy demonstrated neurosecretory granules. The histopathological findings strongly suggest that this is a neuroendocrine tumour.- - - - - - - - - - ranking = 1.25keywords = neuroendocrine (Clic here for more details about this article) |
4/25. Nine novel germline gene variants in the RET proto-oncogene identified in twelve unrelated cases.We report nine novel dna alterations in the RET proto-oncogene in 12 unrelated cases identified by dna sequencing of exons 10 and 11 of the gene. The novel variants K666E, IVS9-11G-->A, D631V in cis with H665Q, D631E (with C634Y), E623K (in trans with C618S), 616delGAG (in trans with C609Y), Y606C, C630R, and R635-T636insELCR;T636P were detected in patients with various clinical presentations ranging from thyroid goiter, medullary thyroid carcinoma, and pheochromocytoma to classic multiple endocrine neoplasia type 2a. When novel dna alterations are found, extended family studies can be helpful in determining the clinical significance of such findings. Segregation within families suggests that K666E and T636insELCR;T636P are likely to be disease-causing mutations. However, the mechanism by which they affect the normal activity of the RET receptor is unclear. Absence of segregation with disease was observed for E623K and 616delGAG. For the remainder of the dna alterations, family studies were not possible, and the clinical significance of these novel variants needs further assessment. Additional case reports, animal models, and/or functional studies are needed to determine the clinical significance of these newly identified variants.- - - - - - - - - - ranking = 0.0039777778449463keywords = carcinoma (Clic here for more details about this article) |
5/25. Neuroendocrine carcinoma of the ampulla of vater. A case of absence of somatostatin in a vasoactive intestinal polypeptide-, bombesin-, and cholecystokinin-producing tumor.A 31-year-old patient with a clinical picture of obstructive jaundice had surgical treatment, and a primary carcinoid of the ampulla of vater (VA) was found. The tumor was studied with light microscopy, immunohistochemistry, and electron microscopy. The neoplasm had histopathologic and cytopathologic features similar to those encountered in typical neuroendocrine neoplasms. It is interesting that immunohistochemical techniques disclosed the presence of vasointestinal polypeptide, cholecystokinin, and bombesin; however, unlike most neuroendocrine neoplasms arising in VA, no somatostatin-immunoreactive cells were found.- - - - - - - - - - ranking = 0.51591111137979keywords = neuroendocrine, carcinoma (Clic here for more details about this article) |
6/25. A malignant mixed mesodermal tumor of the uterine corpus with hypercatecholaminemia.We report an unusual case of malignant mixed mesodermal tumor of the uterine corpus associated with various symptoms related to overproduction of catecholamine by the tumor cells. Histologically, the tumor was dominated by carcinomatous epithelium with foci of malignant mesenchyma. The type of epithelium was endometrioid with papillary adenocarcinomas containing foci of malignant squamous epithelium. The malignant mesenchyma consisted mainly of a fibrous stroma with many large and bizarre cells and spindle cells mimicking leiomyosarcoma, many of which were pleomorphic and contained large bizarre hyperchromatic nuclei. Foci of atypical adult-type cartilage and neoplastic osteoid formation were noted. In the tumor tissue, membrane-bound neurosecretory-type cytoplasmic granules were demonstrated by electron microscopy and polypeptide hormone synthesis was demonstrated by immunohistochemistry. Furthermore, the patient suffered frequent attacks of sudden hypertension with hypercatecholaminemia.- - - - - - - - - - ranking = 0.0079555556898926keywords = carcinoma (Clic here for more details about this article) |
7/25. Ectopic apudocarcinomas and associated endocrine hyperplasias of the foregut.Foregut endocrine polypeptide-secreting apud cells (Amine-Precursor-Uptake and decarboxylation), in their embryologic migration from neural crest to foregut may become "arrested" in the mesoderm or in other ectopic locations. They may become hyperplastic, adenomatous or malignant. Eight illustrative patients are reported. One patient had "pancreatic hyperparathyroidism" with hypercalcemic crises, pancreatic apudocarcinoma, normal parathyroids, biologically active parathormone, but inert immunochemically to the usual parathyroid antisera. Two had gastrin-secreting malignancies in the mesoderm. Remission after excision, but eventual recurrence of the syndrome due to islet cell hyperplasia required total gastrectomy. One patient had a gastric corpus apudocarcinoma found prospectively with hypergastrinemia which required excision of the tumor. One patient had acromegaly with hypergastrinemia and antral gastrinosis treated by pituitary irradiation, One patient had the antral or intermediary type of the zollinger-ellison syndrome with moderate hypergastrinemia, duodenal ulcer and antral gastrinosis, treated by vagotomy and antrectomy. One patient had hyperparathyroidism with antral gastrinosis, treated by parathyroidectomy. One patient had malignant zollinger-ellison syndrome and developed associated thyroid parafollicular cell hyperplasia and parathyroid chief cell hyperplasia, treated by total gastrectomy and multiple endocrine excisions. These investigative observations demonstrate ectopic loci and associated hyperplasias which support the concept of migration and bizarre potentiality of polypeptide-secreting cells of the foregut.- - - - - - - - - - ranking = 0.023866667069678keywords = carcinoma (Clic here for more details about this article) |
8/25. The value of tumor markers in a mixed tumor, mucinous and neuroendocrine carcinoma of the ovary.serum CA125 is frequently used to monitor the treatment response and to detect early recurrence during the follow-up of patients who have epithelial ovarian tumors. In this report, the level of serum CA125 and urinary 5-hydroxyindole acetic acid of a patient with a carcinoid tumor which occurred in association with a mucinous adenocarcinoma of the ovary is described. The level of serum CA125 decreased despite the presence of a clinically progressive tumor. There was a rise of the 5-hydroxyindole acetic acid level 16 weeks before pelvic recurrence was detected clinically. This indicates that it was the neuroendocrine component, and not the mucinous component of the tumor, that was not responsive to the chemotherapy. The problem of using tumor markers to monitor patients with mixed tumors is discussed.- - - - - - - - - - ranking = 1.2698888892247keywords = neuroendocrine, carcinoma (Clic here for more details about this article) |
9/25. Specific antibody activity against neuroendocrine tissue in a case of poems syndrome with IgG gammopathy.Specific antibody binding activity directed against pituitary tissue was demonstrated in a typical case of POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome with paraproteinemia of IgG lambda type. IgG binding against various other tissues could not be detected by immunoelectroblotting and immunocytochemistry. We propose that poems syndrome is an antibody-mediated immune disorder, in which the hypophysis is the primary target of the pathologic myeloma antibodies. The immunopathogenetic lesion of the hypophysis could induce secondary disturbances of other organs, thus explaining the multisystemic disorder.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
10/25. Benign oncocytic endocrine tumor of the pancreas in a patient with polyarteritis nodosa.An 8-cm mass in the tail of the pancreas was resected from a 40-year-old man with polyarteritis nodosa. The tumor cells contained abundant, finely granular, eosinophilic cytoplasm arranged in a gyriform pattern that suggested the tumor was an oncocytoma of the endocrine pancreas. Electron microscopy confirmed that the tumor was an oncocytoma by demonstrating tumor cell cytoplasm packed with mitochondria. Ultrastructural and immunocytochemical studies confirmed the neuroendocrine nature of the tumor by demonstrating dense-core, membrane-bound structures consistent with neurosecretory granules and neuron-specific enolase immunoreactivity. No immunoreactivity for insulin, glucagon, gastrin, somatostatin, or pancreatic polypeptide was found. No human chorionic gonadotropin alpha-chain immunoreactivity was detected. The patient is well without evidence of tumor five years after operation. The apparently benign behavior of the pancreatic endocrine oncocytoma reported here is in contrast to the malignant nature of another case reported recently.- - - - - - - - - - ranking = 0.25keywords = neuroendocrine (Clic here for more details about this article) |
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