1/30. Cushing's syndrome associated with a chemodectoma and a carcinoid tumour.We present a case of Cushing's syndrome where 111In-octreotide scanning provided evidence for the presence of two neuroendocrine tumours. Uptake in the right neck corresponded to a chemodectoma, but there was no change in the clinical condition or fall in ACTH levels following surgical resection. Uptake in the left chest was assumed to relate to a bronchial carcinoid, but a tumour could not initially be localized on magnetic resonance imaging (MRI), spiral CT scanning or on selective venous sampling. A 1 cm bronchial carcinoid tumour was identified post-mortem which immunostained for ACTH. This case demonstrates that 111ln-octreotide scanning is a useful technique for identifying the source of ectopic ACTH production in difficult cases of Cushing's syndrome. Reliance should not be placed solely on standard imaging techniques to localize the tumour prior to surgery. Although rare, the possibility of a non-ACTH secreting neuroendocrine tumour should also be considered in patients with ectopic ACTH syndrome, who have positive 111In-octreotide scans.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
2/30. paraganglioma of the thyroid: two cases that clarify and expand the clinical spectrum.BACKGROUND: Paragangliomas (PGs) can, on rare occasions, arise within the thyroid parenchyma presumably from displaced laryngeal paraganglia. On the basis of a limited number of reported cases, thyroid PGs invariably affect women, they are always benign, and they are usually mistaken for some other more common thyroid lesion. methods: We describe the histopathologic features, immunohistochemical findings, and clinical characteristics of two thyroid PGs. RESULTS: One tumor was incidentally discovered in a 55-year-old man during evaluation of a carotid bruit. The other tumor aggressively invaded the trachea and esophagus of a 52-year-old woman with a presumed long-standing nodular goiter. In both cases, the initial pathologic evaluation suggested medullary thyroid carcinoma. Both patients are alive without recurrent disease after surgical resection. CONCLUSIONS: These cases emphasize the need to consider PG in the differential diagnosis of neuroendocrine thyroid tumors, even in those tumors involving men or behaving in a locally aggressive fashion. Failure to do so carries grave implications regarding patient prognosis and management.- - - - - - - - - - ranking = 0.50390344429208keywords = neuroendocrine, carcinoma (Clic here for more details about this article) |
3/30. A case of Cushing's syndrome in ACTH-secreting mediastinal paraganglioma.Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.- - - - - - - - - - ranking = 0.5keywords = neuroendocrine (Clic here for more details about this article) |
4/30. A diagnostic and therapeutic approach to paragangliomas of the larynx.BACKGROUND: Differentiating paragangliomas from moderately differentiated neuroendocrine carcinoma in the larynx is a difficult management problem. As the biological behavior of these 2 entities is different, we developed an algorithm for the preoperative diagnosis and treatment of this disease. DESIGN: The sample case from which the algorithm was developed consisted of a 69-year-old man who was transferred to us after tracheostomy and an attempt at biopsy for airway obstruction secondary to a vascular mass. biopsy resulted in substantial bleeding. Flexible laryngoscopy showed a vascular mass of the supraglottis. A computed tomographic scan showed 2 vascular masses at the carotid bifurcation and in the larynx. An arteriogram confirmed synchronous vascular tumors. RESULTS: The arteriogram showed the superior thyroid artery to be the major feeder vessel to this mass, a situation commonly seen in paragangliomas but not other neuroendocrine tumors. The presence of synchronous lesions and a vascular mass based on the superior thyroid artery helped differentiate paraganglioma from the other neuroendocrine tumors. As the biological behavior of paragangliomas is relatively benign, we performed a conservative supraglottic laryngectomy and excision of the carotid body tumor. Histologic diagnosis and immunohistochemical analysis confirmed the diagnosis of paraganglioma. CONCLUSIONS: The vascular nature of neuroendocrine tumors prevents preoperative pathological diagnosis. Radiologic features demonstrating a vascular mass with a dominant feeder vessel by the superior or inferior thyroid artery may help in the clinical diagnosis of paragangliomas of the larynx. Since paragangliomas are rarely malignant, a conservative surgical procedure should suffice.- - - - - - - - - - ranking = 2.0039034442921keywords = neuroendocrine, carcinoma (Clic here for more details about this article) |
5/30. Clear-cell odontogenic carcinoma with pulmonary metastases resembling pulmonary meningothelial-like nodules.Clear-cell odontogenic carcinoma (CCOC) is a rare neoplasm with malignant potential and unknown cytogenetic alterations. We describe the case of a 43-year-old woman who presented with an unusual odontogenic epithelial tumor. Histologically, the tumor was composed of clear-cell areas and exhibited a squamous pattern with little nuclear pleomorphism similar to benign squamous odontogenic tumor. Multiple small pulmonary nodules occurring 3 years after primary surgical treatment histologically closely resembled benign minute pulmonary meningothelial-like nodules (MPMN) with clear-cell features. comparative genomic hybridization (CGH) and immunohistochemistry, performed as diagnostic adjuncts, revealed in the odontogenic tumor and the pulmonary lesions a very similar pattern of chromosomal aberrations (loss of 9, gains of 14q, 19 and 20 in both, and additional loss of 6 in the odontogenic tumor) and the same pattern of expression (positive for cytokeratin 5, 6, 8, 19 and negative for cytokeratin 18, epithelial membrane antigen, and vimentin), differing from that of MPMN. These findings confirmed the final diagnosis of metastasizing CCOC with partial squamous differentiation, substantiated the unfavorable prognosis of the clear-cell component, and highlighted the diagnostic impact of CGH and immunohistochemistry for classification of these morphologically peculiar pulmonary CCOC metastases.- - - - - - - - - - ranking = 0.019517221460389keywords = carcinoma (Clic here for more details about this article) |
6/30. paraganglioma manifesting as shock: a case report.paraganglioma is a rare neuroendocrine tumor in children that rarely manifests as shock. We describe the case of a 12-year-old girl with paraganglioma who developed impaired cardiac function, pulmonary edema, and shock at the time of admission. Her blood pressure stabilized after intravenous normal saline rescue and dopamine treatment. However, hypertension was noted thereafter. After a series of examinations, paraganglioma was diagnosed and excision of the tumor was performed. After surgery, blood pressure stabilized and her cardiac function had fully recovered at 4 months' follow-up.- - - - - - - - - - ranking = 0.5keywords = neuroendocrine (Clic here for more details about this article) |
7/30. Primary paraganglioma strictly confined to the liver and mimicking hepatocellular carcinoma: an immunohistochemical and in situ hybridization study.We describe a case of primary nonfunctioning paraganglioma that, unlike any other previously reported case, was strictly confined to the liver and must therefore have arisen on liver parenchyma. An asymptomatic 46-year-old man was referred to us for laparotomy and a right hemihepatectomy after a preoperative diagnosis of fibrolamellar hepatocellular carcinoma, based on a fine-needle biopsy. An 8-cm resiliently firm, pale gray nodule with a large central area of fibrosis and a thin fibrous capsule was resected. The polygonal eosinophilic tumor cells containing round nuclei lacking nucleoli were arranged in small nests set in a vascularly rich stroma. At immunohistochemistry neoplastic cells were strongly positive for chromogranin a, neuron-specific enolase, synaptophysin, and IGF-II protein; they were negative for keratin, S-100 protein, CD10, vimentin, and smooth muscle actin. in situ hybridization confirmed that, as in other sites, liver paraganglioma can express IGF-II gene. Conversely (and unlike hepatocellular carcinomas), the neoplastic cells did not express albumin mRNA, which was detected only in surrounding hepatocytes. The clinical course was benign and the patient is well and free of neoplastic disease 9 years after surgery. knowledge of the entity should avoid possible confusion with hepatocellular carcinoma, especially of the fibrolamellar variety.- - - - - - - - - - ranking = 0.027324110044545keywords = carcinoma (Clic here for more details about this article) |
8/30. neuropeptide y secretion from a malignant extraadrenal retroperitoneal paraganglioma.A patient with a malignant extraadrenal retroperitoneal paraganglioma had elevated levels of immunoreactive neuropeptide y (NPY) in the peripheral blood (5988 pg/ml; normal, 123 /- 30 pg/ml [mean /- standard error of the mean]). A 6-month course of chemotherapy allowed surgical removal of the previously unresectable primary tumor. Postoperatively, the plasma NPY level initially fell to 1089 pg/ml; continued chemotherapy caused an additional decrease to 440 pg/ml. Four months after surgery, the plasma NPY level increased to 940 mg/ml, coincident with hepatic metastases. This case is the first report of a NPY-secreting clinically nonfunctional malignant extraadrenal paraganglioma. Determination of circulating NPY levels may be useful in the diagnosis and follow-up of patients with neuroendocrine tumors.- - - - - - - - - - ranking = 0.5keywords = neuroendocrine (Clic here for more details about this article) |
9/30. Pitfalls in fine needle aspiration cytology of extraadrenal paraganglioma. A report of 2 cases.BACKGROUND: Fine needle aspiration (FNA) is commonly used as an initial diagnostic tool in the evaluation of mass lesions. However, extraadrenal paragangliomas (EAPs) are not commonly seen on FNA and, when encountered, can pose a diagnostic challenge since they have a wide variety of morphologic patterns. CASES: Two intraabdominal EAPs were misdiagnosed as an anaplastic adenocarcinoma of the pancreas with giant cell features and papillary adenocarcinoma of the pancreas. The sources of the pitfalls were inaccurate radiologic localization, misinterpretation of vascular-rich tissue fragments as papillary structures, presence of acinarmicroglandular structures, marked cellular pleomorphism (including multinucleation), prominent nucleoli and some cells with "squamoid" cytoplasm. CONCLUSION: The cytologic features of EAP, although suggestive, are not specific. A high index of suspicion and knowledge of clinical information, exact anatomic location and cytologic morphology combined with appropriate ancillary studies are the key to an accurate diagnosis.- - - - - - - - - - ranking = 0.0078068885841557keywords = carcinoma (Clic here for more details about this article) |
10/30. Neuroendocrine differentiation in hyalinizing trabecular tumor of the thyroid.The presence of neuroendocrine cells is putative in thyroid hyalinizing trabecular tumor (HTT), although this entity requires a differential diagnosis from paraganglioma or medullary carcinoma of the thyroid, due to similarity of the growth pattern of the cells. Here, we present a case of HTT with a mixture of endocrine cells stained positive for somatostatin, chromogranin a and Grimelius' silver impregnation. The histology and results of other immunostainings were consistent with the features typical for HTT. In the world literature to date, only two cases of HTT with endocrine cells, including the current case, have been reported. Nevertheless, the cases may indicate the diversified differentiation of cells in HTT and may, in part, account for the resemblance of HTT to paraganglioma and medullary carcinoma.- - - - - - - - - - ranking = 0.50780688858416keywords = neuroendocrine, carcinoma (Clic here for more details about this article) |
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