1/197. Endoscopically assisted removal of unilateral coronoid process hyperplasia.Coronoid process hyperplasia (CPH) is an uncommon disorder characterized by an enlarged coronoid process impinging against the posterior aspect of the zygomatic arch. Young male adults are usually affected, presenting with limited mouth opening, which is typically painless and progressive in nature. The diagnosis of true CPH is established by the findings of (1) uniform coronoid enlargement on radiographic examination and (2) normal bone structure on histopathological examination (i.e., the specimen should be free of any neoplastic growth, such as the previously reported cases of coronoid osteomas, osteochondromas, or exostoses). The treatment is mainly surgical, by means of a coronoidectomy. An intraoral approach is mostly preferred for this procedure to avoid an external scar. However, to avoid the drawbacks of this approach, such as limited exposure and the risk of hematoma and subsequent fibrosis, an extraoral approach may be indicated. This report describes a case of true unilateral CPH in a 17-year-old boy who presented with progressive limited mouth opening in the absence of any pain. Computed tomography (CT) demonstrated a uniformly enlarged right coronoid process. A coronoidectomy was performed with the aid of endoscopic systems, approaching via two short incisions in the temporal scalp. Histopathological examination of the specimen demonstrated essentially a normal bony structure with no evidence of a neoplasm. The authors present the endoscopically assisted technique of coronoid process excision as an alternative method of surgical treatment of CPH and any mass of the coronoid process in general. With this method, the incision is much shorter than a conventional coronal incision and thus morbidity is diminished considerably.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
2/197. 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 = 179.92262032414keywords = endocrine (Clic here for more details about this article) |
3/197. A case of Schnitzler's syndrome with nodular regenerative hyperplasia of the liver.Schnitzler's syndrome is a rare condition of urticaria, macroglobulinemia, and sclerotic bone lesions. We report a case in a 70-year-old man in whom inflammatory polyarthralgia was followed by a nonpruritic urticarial eruption with a moderate decline in general health. Laboratory tests showed inflammation and a modest isolated peak of monoclonal IgM kappa. There was no evidence of waldenstrom macroglobulinemia. Schnitzler's syndrome was considered. However, an ultrasound scan of the abdomen done because of mild gamma-glutamyl-transferase elevation disclosed multiple hepatic lesions. The liver histology showed incipient nodular regenerative hyperplasia. Only about 30 cases of Schnitzler's syndrome have been reported since the seminal description in 1972. Hepatic involvement was a common but nonspecific finding, and we found no cases with nodular regenerative hyperplasia. However, this abnormality is often found in patients with autoimmune or hematological disorders. The pathogenesis of Schnitzler's syndrome remains unknown, but the possibility of progression to a hematological malignancy requires prolonged follow-up.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
4/197. A rare case of pituitary hyperplasia with suprasellar extension due to primary myxoedema: case report.The development of pituitary tumours as a consequence of primary target organ failure is rare. We report here a rare case of pituitary hyperplasia with suprasellar extension due to primary myxoedema. This case presentation suggested the importance of detailed endocrine investigation and repeated magnetic resonance imaging for the differential diagnosis of pituitary enlargement to avoid unnecessary surgery.- - - - - - - - - - ranking = 89.961310162068keywords = endocrine (Clic here for more details about this article) |
5/197. liver cirrhosis with marked thrombocytopenia and highly elevated serum thrombopoietin levels.Three patients with liver cirrhosis (LC) and a bleeding tendency due to marked thrombocytopenia of less than 20 x 10(9)/l were admitted to our hospital for further examination. bone marrow examination revealed megakaryocytic hypoplasia in all three patients. All patients exhibited amegakaryocytic thrombocytopenic purpura, myelodysplastic syndrome, or bone marrow hypoplasia. 111In-labeled platelet kinetic studies revealed decreased platelet production in all patients. Although serum thrombopoietin (sTPO) levels are usually within the normal level in patients with LC, the sTPO levels of our patients were about 10 times higher than the levels of normal subjects (1.22 /- 0.37 fmol/ml): 13.34, 16.79, and 10.46 fmol/ml, respectively. These sTPO data supported our findings of decreased megakaryopoiesis. Our findings suggest that examination of sTPO levels is useful in determining the etiology of marked thrombocytopenia in LC patients.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
6/197. Expression of vesicular monoamine transporters in endocrine hyperplasia and endocrine tumors of the oxyntic stomach.BACKGROUND: Gastric enterochromaffin-like (ECL) cells selectively express the vesicular monoamine transporter (VMAT) VMAT2, and enterochromaffin (EC) cells the VMAT1 isoform. Aims: We investigated whether VMAT isoform selection indicates the origin of endocrine hyperplasia and neoplasia from oxyntic ECL or EC cells and may be of prognostic significance in different types of gastric carcinoids. methods: Tissue from patients with chronic atrophic gastritis (CAG), Zollinger-Ellison-syndrome (ZES), gastric carcinoids and neuroendocrine carcinoma (NEC) was investigated by immunohistology and in situ hybridization. RESULTS: endocrine cells forming diffuse, linear, and micronodular hyperplasia in CAG and ZES, as well as oxyntic microcarcinoids expressed both VMAT2 and chromogranin a (CgA) but neither VMAT1 nor serotonin. In five of six sporadic carcinoids VMAT2 and CgA but not VMAT1 were detected. One carcinoid was copositive for VMAT1 and serotonin but negative for VMAT2. Electron microscopy confirmed the VMAT2-positive tumors as ECLoma and the VMAT1-immunoreactive carcinoid as EComa. CONCLUSIONS: VMAT2 and VMAT1 are reliable markers for differentiation of gastric endocrine hyperplasia and neoplasia from ECL and EC cells, respectively. The significance of VMAT2 and VMAT1 as prognostic markers lies in the relatively poor prognosis for EComa compared to ECLoma, characterized by VMAT2 positivity. The absence of both VMAT2 and VMAT1 in NEC may indicate poor prognosis.- - - - - - - - - - ranking = 989.57441178275keywords = endocrine (Clic here for more details about this article) |
7/197. Colonic manifestations of multiple endocrine neoplasia type 2b: report of four cases.multiple endocrine neoplasia type 2b is best known for its endocrine manifestations and typical phenotype. The gastrointestinal manifestations, however, are also an important and commonly unrecognized component of the syndrome. We present four cases that demonstrate the varied presentation of patients with colonic manifestations of multiple endocrine neoplasia type 2b. We discuss the cause, diagnostic significance, and management of the colonic disease that is a component of multiple endocrine neoplasia type 2b.- - - - - - - - - - ranking = 719.69048129655keywords = endocrine (Clic here for more details about this article) |
8/197. Giant insulinoma in a patient with multiple endocrine neoplasia-type I: a case report.We report a case of giant cystic insulinoma constituting part of multiple endocrine neoplasia (MEN) type I. A 29-year-old Japanese man presented with a history of recurrent hypoglycemic attacks. Endocrine examination showed hyperinsulinemia discordant with hypoglycemia, and a giant cystic insulinoma (11 x 10 cm) located in the pancreatic tail was detected radiologically. hyperprolactinemia due to pituitary adenoma and hyperparathyroidism due to parathyroid hyperplasia were also present. The insulinoma, prolactinoma and hyperplastic parathyroid gland were surgically removed. Fluorescent microsatellite analysis detected loss of heterozygosity (LOH) in chromosome 11q13 in dna samples from all resected tissues but not from white blood cells. This is a rare case of MEN type I because of the giant cystic insulinoma and the evidence of common LOH detected in all MEN type I tissues.- - - - - - - - - - ranking = 449.80655081034keywords = endocrine (Clic here for more details about this article) |
9/197. Dizygotic twin sisters with myelokathexis: mechanism of its neutropenia.Dizygotic twin sisters were first found to have neutropenia at 1 year of age when evaluated for recurrent pulmonary infections. Since then they have remained neutropenic (0.05 approximately 0.5 x 10(9)/l). Despite of their neutropenia, myeloid hyperplasia was evident on a marrow smear examination, and a number of cells were hypersegmented with fine interlobular bridging with chromatin strands and cytoplasmic vacuolation. Electron microscopy showed apoptotic cells with condensed nuclei and apoptotic bodies in the cytoplasm. Although life span, hydrogen peroxide production, phagocytosis, spreading, and chemotaxis of peripheral neutrophils were normal, the survival of bone marrow neutrophils in both infants was markedly decreased when compared with that of normal bone marrow neutrophils. During the bone marrow culture apoptotic neutrophils were observed at an earlier stage in both patients than in normal controls, biochemically and morphologically. Morphology of bone marrow neutrophils in both patients resembled that of cultured control bone marrow neutrophils. Peripheral neutropenia and appearance of characteristic neutrophils in the bone marrow in myelokathexis are considered to be an expression of apoptosis of bone marrow neutrophils.- - - - - - - - - - ranking = 7keywords = bone (Clic here for more details about this article) |
10/197. Thymic carcinoid and parathyroid hyperplasia detection with 99mTc-MIBI men type 1.We report a case of a 35-year-old male, with a history of diarrhea, renal lithiasis with frequent expulsions of calculus and hypercalcemia during the last 2 years. The patient was studied and diagnosed with a multiple endocrine neoplasia type I (MEN I), familiar (mother with MEN I). A scintigraphic study with 99mTc-MIBI was performed in order to localize hyperfunctioning parathyroid glands because of biochemical diagnosis of primary hyperparathyroidism. Double phase 99mTc-MIBI scan detected one hyperfunctioning parathyroid gland and a large anterior mediastinal mass. Subsequent, plain radiograph and CT of the chest showed a soft-tissue mass in that localization. Punch biopsy of the lesion guided by CT revealed malignant cells of neuroendocrine tumor. The tumor was removed and histologically confirmed as a carcinoid within a thymus in a MEN type I syndrome. MEN I patients can benefit from the examination with this agent which can potentially localize not only parathyroid endocrine pathology but also unknown associated tumors.- - - - - - - - - - ranking = 269.8839304862keywords = endocrine (Clic here for more details about this article) |
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