1/210. 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 = 1keywords = gland (Clic here for more details about this article) |
2/210. Resection of triple synchronous cancers: a case report.We herein present a case of synchronous triple cancer, which was successfully resected in a curative manner. These cancers consisted of primary duodenal, pancreatic and lung cancers, which were diagnosed in an asymptomatic 74 year-old male, who was referred to our department on December 14, 1996. On admission, his laboratory data showed no abnormality, including tumor markers (CEA 1.0, CA 19-9 1.0, AFP 8.1 U/ml), but he did show an impaired pulmonary function (FEV1.0: 57%). Upper gastrointestinal endoscopy showed a smooth surfaced duodenal tumor measuring 4 cm in size. The second tumor was found at the head of the pancreas by computed tomography (CT), showing a hypervascular mass measuring 3.0 cm, along with neighboring multiple cysts. In endoscopic retrograde cholangiopancreatography (ERCP), marked mucous secretion was observed through the papilla, while a filling defect was found in the dilated pancreatic duct. In a routine chest X-ray, a third tumor, which measured 1.5 cm in diameter, was recognized in the right upper lobe of the lung, and a moderately differentiated squamous cell carcinoma was also detected by a percutaneous CT guided biopsy. The pancreatic and duodenal tumors were surgically resected by a pancreatoduodenectomy (Stage I) in January 1997 and, 5 months later, a lung tumor underwent partial resection (Stage I). This patient tolerated these surgical procedures well and presently leads a normal, healthy life after discharge. In summary, a successful resection of synchronous triple cancers, which has never been previously reported in this specific combination, is described.- - - - - - - - - - ranking = 0.0066567774342207keywords = lobe (Clic here for more details about this article) |
3/210. 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 = 0.2keywords = gland (Clic here for more details about this article) |
4/210. Long-term survival after diagnosis of hepatic metastatic vipoma: report of two cases with disparate courses and review of therapeutic options.This report describes two patients with pancreatic cholera caused by vasoactive intestinal polypeptide (VIP)-producing tumors, which originated in the pancreas and showed metastases in both hepatic lobes at time of diagnosis. However, the two tumors displayed remarkably disparate clinical courses. Due to the protracted but progressive course over more than 10 years, a multifaceted therapeutic approach was performed to control symptoms and to improve quality of life. The long-acting somatostatin analog octreotide was the most effective treatment for relieving symptoms and correcting fluid and electrolytes disturbances. The effects of complementary treatments, including systemic chemotherapy and hyperselective chemoembolization, as well as concurrent application of octreotide and prednisolone or interferon with respect to clinical symptoms, VIP levels, and tumor growth are reviewed. Our experience, although small, emphasizes the need for an expert, well-planned, adaptive, and multidisciplinary approach in the care of these complex patients.- - - - - - - - - - ranking = 0.0066567774342207keywords = lobe (Clic here for more details about this article) |
5/210. 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 = 0.4keywords = gland (Clic here for more details about this article) |
6/210. Excessive nodular hyperplasia of brunner glands associated with gastric hypersecretion and lipomatous atrophy of the pancreas.The case of a 34-year-old woman complaining of diarrhoea and abdominal pain is presented. Contrast radiography and endoscopy showed multiple polypoid tumours in the second part of the duodenum. Moreover, a severe fatty infiltration of the pancreas was shown by magnetic resonance and computed tomography scans. Due to pain, pancreatoduodenectomy (Whipple operation) was performed, and subsequent histopathologic examinations showed excessive Brunner gland hyperplasia of the duodenum and severe lipomatous atrophy of the pancreas. The occurrence of these two rare conditions in one patient has not been described previously, and it is conceivable that the lipomatous atrophy and exocrine insufficiency of the pancreas may have caused a compensatory stimulation of the submucosal structures of the duodenum.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/210. Lymphoepithelial cyst of the pancreas. No evidence for Epstein-Barr virus-related pathogenesis.Compared to pseudocyst formation after prior pancreatitis, true cysts of the pancreas are rare. Pancreatic cysts with irregular wall components or a mucinous content raise the suspicion for the presence of a cystic neoplasm, and surgical resection is recommended. A case of a patient with a history of prostate cancer is described in whom a cyst of the pancreatic tail was discovered incidentally. Based on the radiographic features, which did not support the presence of a serous cystadenoma, a spleen-preserving distal pancreatectomy was performed. Histologic features were characteristic for a lymphoepithelial cyst (LEC) of the pancreas, lined with thinned squamous epithelium surrounded by benign lymphoid tissue. Since LECs of the parotid gland, which are associated with acquired human immunodeficiency, are frequently related to Epstein-Barr virus (EBV) infection, EBV in situ hybridization was performed and did not reveal evidence for EBV. Twenty-eight instances of pancreatic LECs have been reported, primarily affecting adult males, without evidence of increased numbers of EBV-positive cells. The pathogenesis, differential diagnosis, and clinical implications of lymphoepithelial pancreatic cysts are discussed.- - - - - - - - - - ranking = 0.2keywords = gland (Clic here for more details about this article) |
8/210. 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 = 8.9729035095159keywords = pituitary (Clic here for more details about this article) |
9/210. multiple endocrine neoplasia type 1: atypical presentation, clinical course, and genetic analysis of multiple tumors.multiple endocrine neoplasia type 1 (MEN1) is characterized by the development of endocrine tumors of the parathyroid and pituitary glands, pancreas, and duodenum. Less frequently occurring tumors associated with MEN1 include non-endocrine tumors such as lipomas and angiofibromas. An increased incidence of thyroid neoplasms, leiomyomas, adrenal cortical hyperplasia, hepatic focal nodular hyperplasia, and renal angiomyolipoma has been noted in the MEN1 population. The pathogenesis of non-neuroendocrine tumors in MEN1 is unknown. We report a complex clinical course and a detailed morphologic and genetic analysis of a series of tumors that developed in a patient with MEN1. All tumors were microdissected and analyzed for loss of heterozygosity of the MEN1 gene. A germline mutation of the MEN1 gene was detected, and deletions of the MEN1 gene were consistently detected in multiple neuroendocrine tumors involving the parathyroid glands and the pancreas and a hepatic neuroendocrine tumor metastasis, as predicted by Knudson's "two hit" hypothesis. Two hits of the MEN1 gene were also detected in esophageal leiomyoma tissue, suggesting that tumorigenesis was directly related to the patient's underlying MEN1. In contrast, follicular thyroid adenoma, papillary thyroid carcinoma, hepatic focal nodular hyperplasia, and adrenal cortical hyperplasia consistently showed retained heterozygosity of the MEN1 gene with flanking markers and an intragenic marker. Therefore, these tumors appear to develop along pathogenetic pathways that are different from classical MEN1-associated tumors.- - - - - - - - - - ranking = 8.0919148352831keywords = pituitary, pituitary gland, gland (Clic here for more details about this article) |
10/210. 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 = 4.6864517547579keywords = pituitary, gland (Clic here for more details about this article) |
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