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1/154. Management of incidentally discovered cervical paragangliomas: report of two cases and review of current issues.

    Paragangliomas of the head and neck are uncommon neoplasms arising from the extra-adrenal paraganglia and include carotid body and glomus vagale tumors. These lesions may be discovered incidentally by imaging studies performed to evaluate carotid atherosclerotic occlusive disease. Incidental paragangliomas of the head and neck may be smaller than those discovered due to symptoms. Although surgical resection remains the definitive treatment for head and neck paragangliomas, important issues of management arise when such lesions are discovered. Two recent cases are reported. epidemiology, pathophysiology, diagnostic evaluation, and issues of management of head and neck paragangliomas are discussed.
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2/154. MR appearance of paraganglioma of the cauda equina. case reports.

    PURPOSE: To investigate the value of MR imaging for preoperative diagnosis of paraganglioma of the cauda equina. MATERIAL AND methods: A retrospective review of 2 cases of paraganglioma of the cauda equina examined with MR imaging was undertaken. Features assessed included the homogeneity of the lesions, presence or absence of serpiginous flow void and thin hypointense margins. RESULTS: In case 1, the tumor was hyperintense on the postcontrast examination and serpiginous flow void suggested vessels in the upper pole of the tumor. In case 2, the tumor was encapsulated by a thin hypointense margin on both T1- and T2-weighted images, which suggested hemosiderin. CONCLUSION: The MR appearance may be of great value in the preoperative diagnosis of paraganglioma of the cauda equina.
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keywords = ear
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3/154. Extra-adrenal pheochromocytoma (paraganglioma).

    The authors describe the case of a 19-year-old female patient with an abdominal paraaortic extra-adrenal pheochromocytoma (paraganglioma), presenting arterial hypertension. The predominant catecholamine produced by the tumor was norepinephrine (4110 pg/ml; normal < 450 pg/ml). 131I metaiodobenzylguanidine (131I-MIBG), computed tomography and magnetic resonance imaging allowed location and characterization of the tumor. Histologically the tumor (weight = 34.2 g; 5.8 x 4 x 3 cm) was a typical pheochromocytoma.
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ranking = 0.3189911007799
keywords = ear
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4/154. 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.
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ranking = 0.63798220155979
keywords = ear
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5/154. Surgical resection of jugulare foramen tumors by juxtacondylar approach without facial nerve transposition.

    PRESENTATION: Jugulare foramen tumors (JFT) remain a difficult challenge especially in the forms extending extradurally and invading the petrous bone. In the standard technique, facial nerve function is placed at risks because of its transposition. We report on 31 extradural (N = 11) or intra-extra dural (N = 20) JFT resected surgically using the juxtacondylar approach alone or in combination with the infratemporal approach and without facial nerve transposition. RESULTS: The juxtacondylar approach permits the opening of the JF on its posteroinferior aspect and thus reduces the extent of petrous bone drilling. In tumors strictly located in the JF (N = 11), no petrous bone drilling is necessary and the facial nerve is never exposed. In tumors extending into the petrous bone (N = 20), the facial nerve was never transposed and moreover was kept in its bony canal in 15 cases. In only 5 cases, was the fallopian canal opened as the tumor was invading its bony wall. Similarly hearing function, when pre-operatively intact was always preserved and a better preservation of the lower cranial nerves could be achieved. Whatever the tumoral extent along the petrosal carotid artery, a radical resection could be realized in 30 cases. CONCLUSION: Therefore, we consider the juxtacondylar approach a useful adjunct to increase the possibilities of resection of JFT; it allows a better preservation of the neurovascular structures, especially the facial nerve which is kept in place and moreover kept in its bony canal when it is not invaded by the tumor.
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keywords = ear
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6/154. 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.
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ranking = 0.3189911007799
keywords = ear
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7/154. Malignant carotid body tumors: report of three cases.

    The aim of the current report was to describe 3 cases of malignant carotid body tumor (CBT) and to review the literature. My clinical records of 87 CBTs in 81 patients (6 bilateral) were reviewed, 79 of which were operated on. Three malignant cases were found. The first was in a 40-year-old man who presented with pulmonary metastases 6 years after resection of a CBT. He was treated with chemotherapy and interferon, but died with disseminated disease 2 years later. The second case was in a 56-year-old woman who had a 5-cm, fixed, hard mass in the upper aspect of the neck and a paralysis of the left vocal cord. This lesion was completely resected, and a shunt and reconstruction with a saphenous vein graft were performed. pathology revealed a malignant chemodectoma with invasion to 2 of the 5 lymph nodes removed. radiotherapy (50 Gy) was given after the operation. She is well and free of disease 68 months after the resection. The third case was in a 61-year-old woman who presented with an 8-cm nontender, hard, immobile mass in the left upper neck that displaced the left wall of the oropharynx toward the midline. A carotid arteriogram showed a CBT. On computed tomography, the tumor extended to the infratemporal fossa with no bone involvement. The lesion was embolized with a 40% reduction in vascularity. At surgical exploration, the tumor involved the sternocleidomastoid muscle and the lymph nodes at levels II and III, and the internal carotid artery could not be dissected free at the skull base, so only a partial resection was performed. This patient was lost to follow-up. These 3 cases are in agreement with the literature. Locoregional control is usually obtained with complete primary tumor resection and lymphadenectomy and eventual radiotherapy. Surgery with radiotherapy seems to be effective for isolated metastases. Current multidisciplinary treatments have been unsuccessful in controlling disseminated disease.
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ranking = 1.5949555038995
keywords = ear
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8/154. 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.
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ranking = 0.3189911007799
keywords = ear
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9/154. 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.
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ranking = 4.5089021085788
keywords = neoplasm, ear
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10/154. 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.
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ranking = 0.3189911007799
keywords = ear
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