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1/10. A new pattern of multiple endocrine adenomatosis: chemodectoma, bronchial carcinoid, GH-producing pituitary adenoma, and hyperplasia of the parathyroid glands, and antral and duodenal gastrin cells.

    A female patient was found to have a chemodectoma, a GH-producing pituitary tumour and a bronchial carcinoid combined with hyperplasia of the parathyroids and of antral and duodenal gastrin cells. This combination of endocrine tumours and hyperplasias does not fit with the two multiple endocrine adenomatosis syndromes recognized at present. The case stresses the importance of scanning the patient for other endocrine tumours, once one has been diagnosed.
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2/10. Prediction of malignant behavior of pheochromocytomas and paragangliomas using immunohistochemical techniques.

    Pheochromocytomas and paragangliomas arise from the adrenal glands and extraadrenal paraganglia, respectively. Malignant behavior of these tumors is uncommon and is, in part, dependent on their sites of origin, such as extraadrenal location. Morphologic criteria for malignancy of pheochromocytoma and paragangliomas have not been clearly defined. In this study, to clarify the histologic features that distinguish the benign from malignant pheochromocytomas and paragangliomas, we examined metastatic and nonmetastatic tumors using immunohistochemical techniques. A total of eight cases, five pheochromocytomas from the adrenal glands (four benign and one malignant tumor) and three paragangliomas with invasion or metastasis, were studied. The markers used in this study were chromogranin a, synaptophysin, NCAM (CD56), SNAP25, neuron-specific enolase, S-100 protein, and MIB-1. Our results suggest that MIB-1 immunostaining is a useful adjunct marker to predict malignant behavior in these tumors.
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3/10. 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.
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4/10. Nonchromaffin paraganglioma (chemodectoma) of thyroid region.

    A case of a paraganglioma located over the thyroid cartilage in the midline is reported. The tumor had all the appearances of a carotid body tumor, but its odd location could not be explained on the basis of previously known areas where paraganglionic tissues have been found. Since a paraganglioma has also hitherto been described within the thyroid gland, clinicians and pathologists alike are cautioned that unusually located paragangliomas may be expected in the future.
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5/10. A case of familial paraganglioma syndrome type 4 caused by a mutation in the SDHB gene.

    BACKGROUND: A 40-year-old man was referred to our clinic with recurrent paragangliomas. He had undergone resection of a paraganglioma superior to the right adrenal gland at 19 years of age, resection of two para-aortic paragangliomas at 39 years of age, and resection of a paraganglioma in the interatrial septum at 40 years. The patient's mother had died at age 39 years of metastases from a carotid body tumor. INVESTIGATIONS: MRI and CT scanning, 131I-labeled metaiodobenzylguanidine scanning, and genetic testing for a mutation in the succinate dehydrogenase complex, subunit B gene. diagnosis: Familial paraganglioma syndrome type 4 caused by a mutation in the succinate dehydrogenase complex, subunit B gene. MANAGEMENT: The patient underwent two surgical procedures in our clinic. The first was to remove two para-aortic paragangliomas, and the second to remove a paraganglioma that involved both atria. The patient is at high risk for malignant disease and should undergo an annual monitoring program that consists of physical examination and measurement of his blood pressure and levels of urinary catecholamines and metanephrines. If these procedures suggest a recurrence of paraganglioma, 123I-labeled metaiodobenzylguanidine scanning should be performed. As he might develop nonfunctional tumors, however, he should also undergo CT scanning, MRI scanning, or both, of the neck, thorax, abdomen, and pelvis every 6-12 months. genetic testing has been offered to family members.
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6/10. glomus tumor treated by prostaglandin inhibition. Report of a case.

    glomus tumor is a very painful pericytal lesion of the arteriovenous anastomotic complex that controls circulation in a limb. glomus tumor usually involves a digit. Prostaglandin inhibition may control the glomus tumor pain, but surgical removal is the cure. When the condition is discovered early, or when there is no gross evidence of tumor, thermography and localized anesthetic blocks are invaluable in arriving at the proper diagnosis.
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7/10. Approaches to the diagnosis and treatment of tumors of the parapharyngeal space.

    Tumors of the parapharyngeal space appear as painless massess bulging into the tonsillar, nasopharyngeal, or retromandibular area. Most are benign and represent a wide range of tumor growth, from parotid extension to intrinsic growth of nerves, blood vessels, and salivary gland tissues within or around the parapharyngeal area. The anatomy of this area and diagnostic procedures are discussed. Sinus films, tomograms of the skull base and lateral pharyngeal area, CT scans with simultaneous parotid sialograms, and angiograms are part of the evaluation of these rarely seen tumors. The approach to therapy is discussed.
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8/10. Angiographic detection of unsuspected carcinoma of the thyroid gland.

    angiography is seldom used to detect carcinoma of the thyroid. We have recently encountered four patients with hypervascular lesions of the neck demonstrated by angiography for evaluation of an unrelated medical problem. In each, carcinoma of the thyroid was detected and total thyroidectomy performed. Because of the possibility of carcinoma of the thyroid in patients with hypervascular lesions of the neck, we recommend exploration of the neck, regardless of symptoms, physical findings or scintiscan results.
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9/10. Multiple chemodectomas. carotid body tumor masked by salivary gland uptake on I-123 MIBG scintigraphy.

    Chemodectomas resemble pheochromocytomas in that they are derived from neural crest tissue. Their distribution in the body may be quite different, however, as chemodectomas are more frequently found in the aortic and carotid bodies, while pheochromocytomas are most common in the adrenal medulla. The authors present a case of a patient with a known history of chemodectoma, imaged with I-123 MIBG to rule out recurrent disease. Images of the chest revealed a thoracic tumor representing recurrent periaortic tumor, but an additional carotid body tumor (which was later demonstrated by angiography) was masked by salivary gland uptake. In patients with chemodectomas, SPECT imaging of the neck may be necessary to distinguish normal salivary gland uptake from tumoral uptake of MIBG. In general, knowledge of the characteristics of the individual neural crest derived tumor is mandatory for maximal effectiveness of I-123 MIBG scintigraphy.
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10/10. paraganglioma: an unusual tumour of the parathyroid gland.

    A case of paraganglioma arising within a parathyroid gland is reported. The lesion was an incidental finding in a block dissection of neck performed for squamous carcinoma of the pharynx. A well-circumscribed lesion, exhibiting the characteristic pathological features of a paraganglioma, was embedded within the right inferior parathyroid gland. Due to its location, the chief histological differential diagnosis was an unusual variant of parathyroid adenoma. immunohistochemistry and electron microscopy assisted in reaching a diagnosis. This, as far as we are aware, is the first reported case of a paraganglioma of the parathyroid gland.
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