1/510. The elevated serum alkaline phosphatase--the chase that led to two endocrinopathies and one possible unifying diagnosis.A 39-year-old Chinese man with hypertension being evaluated for elevated serum alkaline phosphatase (SAP) levels was found to have an incidental right adrenal mass. The radiological features were characteristic of a large adrenal myelolipoma. This mass was resected and the diagnosis confirmed pathologically. His blood pressure normalised after removal of the myelolipoma, suggesting that the frequently observed association between myelolipomas and hypertension may not be entirely coincidental. Persistent elevation of the SAP levels and the discovery of hypercalcaemia after surgery led to further investigations which confirmed primary hyperparathyroidism due to a parathyroid adenoma. The patient's serum biochemistry normalised after removal of the adenoma. The association of adrenal myelolipoma with primary hyperparathyroidism has been reported in the literature only once previously. Although unconfirmed by genetic studies this association may possibly represent an unusual variation of the multiple endocrine neoplasia type 1 syndrome.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/510. Multiple basal cell carcinomas associated with hairy cell leukaemia.We report the case of a caucasian woman who, between the ages of 49 and 51 years, developed multiple (> 20) basal cell carcinomas (BCC). There was no family history of BCC. No abnormalities in the human homologue of the drosophila segment polarity gene patched (PTCH), glutathione S-transferases T1 and M1, or cytochrome P450 1A1 were detected by polymerase chain reaction (PCR)-based molecular analysis. There was, however, actinic damage of the skin in sun-exposed areas. The patient was diagnosed as having hairy cell leukaemia (HCL) at the age of 51 years, based upon leucocyte morphology as assessed by light and electron microscopy, tartrate-resistant acid leucocyte phosphatase (TRAP) staining, fluorescence activated cell scanning of peripheral blood leucocytes and bone marrow histology. As the leukaemia slowly progressed over a 3-month period, the patient developed four further BCCs. Given that HCL is characterized by a profound defect in T-cell function, it is conceivable that T-cell immune dysregulation can contribute to the pathogenesis of BCC, possibly enhancing the aetiological effect of ultraviolet irradiation.- - - - - - - - - - ranking = 0.034318678472928keywords = bone (Clic here for more details about this article) |
3/510. A case of synchronous double primary lung cancer with neuroendocrine features.We report a case of unique double primary lung cancers with neuroendocrine features in a 63-year-old male smoker. The mass in the left lower lobe (LLL) was a small cell/large cell carcinoma with spindle cell sarcomatous areas and organoid structure. The mass in the left upper lobe (LUL) was a tubular adenocarcinoma with neuroendocrine features including organoid nests showing occasional rosette formation, nuclear palisading in the periphery of the nests and positive immunoreaction for CD56, chromogranin a and synaptophysin. The difference in histological structures between the two masses led us to diagnose double primary lung cancer. The combination of small cell lung carcinoma and spindle cell carcinoma is very uncommon. The relationship between LLL and LUL tumors remains unclear. Multiple lung cancers with neuroendocrine features have only rarely been reported in the literature. The patient in our case died of widespread cancer 2 years and 4 months after the surgery without adjuvant chemotherapy, a longer postoperative survival time than in cases of ordinary extensive small cell lung cancer. Multiple lung cancers with neuroendocrine features are extremely rare and similar cases have not been reported in the literature. Neuroendocrine differentiation has attracted widespread attention and, therefore, examining neuroendocrine features in lung cancers is important.- - - - - - - - - - ranking = 10keywords = endocrine (Clic here for more details about this article) |
4/510. brain metastases from adenoendocrine carcinoma of the common bile duct: a case report.A 68-year-old man with metastatic brain tumors from adenoendocrine carcinoma of the common bile duct is reported. A common bile duct tumor and a metastatic liver tumor had been resected 6 years and 3 years prior to admission, respectively. Microscopically they showed two components; moderately differentiated tubular adenocarcinoma and neuroendocrine carcinoma. He presented with headache and vomiting and MRI revealed two metastatic brain tumors. They were successfully resected and radiotherapy was carried out. Histological diagnosis of the metastatic brain tumors was neuroendocrine carcinoma, but carbohydrate antigen (CA)-19-9 and carcinoembryonic antigen (CEA)-immunoreactive cells were observed without glandular pattern. Immunohistochemically serotonin and pancreatic polypeptide were detected, but somatostatin was not. As the endocrine cells demonstrated in the normal extrahepatic bile ducts are only somatostatin-containing D cells, these cells are considered to originate as part of a metaplastic process. To our knowledge, this represents the second case of adenoendocrine carcinoma of the common bile duct.- - - - - - - - - - ranking = 9keywords = endocrine (Clic here for more details about this article) |
5/510. Heterotopic pancreas, periampullary somatostatinoma and type I neurofibromatosis: a pathogenetic proposal.This case documents the association of ectopic pancreatic tissue with a duodenal somatostatinoma in a patient with type I neurofibromatosis. pancreatic ducts have been noted within the centres of somatostatinomas, but little significance has been attached to this finding. Here we describe a patient in whom a separate proliferation of somatostatin cells occurred in association with the ectopic pancreatic ductular epithelium. This lesion bore a striking resemblance to the ductulo-insular or ductulo-endocrine complexes that are seen in nesidioblastosis in the pancreas. We therefore postulate that the ducts, which are sequestered within somatostatinomas, are of pathogenetic significance. The somatostatin-producing cells arise from these ducts, very much in the fashion of ductulo-endocrine complexes in nesidioblastosis.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
6/510. Large cell neuroendocrine carcinoma of the uterine cervix: a report of a case with coexisting cervical intraepithelial neoplasia and human papillomavirus 16.Large cell neuroendocrine carcinomas (LCNECs), one of the four newly categorised endocrine tumors of the uterine cervix, are unusual and aggressive tumors. The present report describes a case of LCNEC diagnosed at an early stage and associated with cervical intraepithelial neoplasia (CIN). The LCNEC showed organoid and trabecular growth patterns and was positive for chromogranin and synaptophysin. The CIN lesion was of a high grade and was negative for these neuroendocrine markers. polymerase chain reaction (PCR) using genomic dna extracted from archival tissue demonstrated human papillomavirus (HPV) type 16 dna in both the LCNEC and CIN lesions. These histological, immunohistochemical and PCR findings suggested that the LCNEC lesion was distinct from the CIN lesion and that both resulted from the carcinogenic field effect of HPV 16.- - - - - - - - - - ranking = 7keywords = endocrine (Clic here for more details about this article) |
7/510. Ampullary somatostatinoma in a patient with Merkel cell carcinoma.A 59-yr-old white man with Merkel cell carcinoma of his right leg status post extensive skin resection and chemotherapy had dilated hepatic and common bile ducts on a routine follow-up abdominal CT scan. A 1.9-cm ampullary mass was appreciated on endoscopy. histology showed psammoma bodies and positive immunoperoxidase staining consistent with a somatostatinoma. Merkel cell tumors and somatostatinomas are extremely rare neuroendocrine tumors derived from neural crest cells. Associations have been found between somatostatinomas and other islet cell tumors with multiple endocrine neoplasia syndromes, but no reported association has been published between islet cell tumors and Merkel cell tumors. This patient represents the first documented case of Merkel cell carcinoma and somatostatinoma in a single patient. Such an occurrence may represent a previously undescribed neuroendocrine tumor syndrome, and this possibility should be considered when either tumor is diagnosed.- - - - - - - - - - ranking = 3keywords = endocrine (Clic here for more details about this article) |
8/510. 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 = 3keywords = endocrine (Clic here for more details about this article) |
9/510. osteosarcoma arising in a solitary osteochondroma of the fibula.We present a case of osteosarcoma arising in an osteochondroma of the right fibula in a 30-year-old woman. The available radiographic studies of the lesion were not suggestive of malignant transformation. The lesion and underlying bone were excised. Histologic examination showed a conventional high-grade osteoblastic osteosarcoma that focally eroded the fibrocartilaginous cap. The patient received postoperative chemotherapy and shows no evidence of disease 27 months following operation. The occurrence of osteosarcoma in an osteochondroma is an extremely rare event, and only a few cases are on record in the literature.- - - - - - - - - - ranking = 0.034318678472928keywords = bone (Clic here for more details about this article) |
10/510. A case of Ollier's disease associated with two intracerebral low-grade gliomas.Ollier's disease, or multiple enchondromatosis, is a deforming dysplastic disease of cartilage, characterized by multiple, asymmetrically distributed intra-osseous cartilaginous masses in the metaphyses and diaphyses of bones. When associated with soft tissue hemangiomas it is referred to as Maffucci's syndrome, in which the enchondromatosis has no unilateral distribution. The emergence of malignant neoplasms, including gliomas, is a well-recognized complication in Maffucci's syndrome. We report a 28-year-old patient with a history of Ollier's disease, who developed two low-grade cerebral gliomas as well as an intracranial chondroma. This case history questions the distinction between the two forms of enchondromatosis and supports a continuum between these disease entities.- - - - - - - - - - ranking = 0.034318678472928keywords = bone (Clic here for more details about this article) |
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