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1/12. 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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2/12. Aortico-pulmonary paraganglioma associated with bilateral carotid body tumors. Diagnostic presentation and clinical implications.

    A case of mediastinal paraganglioma in association with bilateral carotid body tumors is presented. Characteristic radiological findings included a hypointense signal in T1-weighted, a hyperintense signal in T2-weighted magnetic resonance (MR) images and a vascular enhancement pattern in dynamic contrast enhanced MR imaging. Thus, feeding vessels could be depicted noninvasively. The importance of family screening in affected individuals is stressed, as a hereditary form of the disease exists in which multiple paragangliomas are common.
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3/12. 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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4/12. Aortico-pulmonary paraganglioma: case report and Japanese review.

    Aortico-pulmonary paraganglioma (APPG) is a rare middle mediastinal tumor. We experienced a case of APPG in a 52-year-old man. Chest computed tomography and magnetic resonance imaging revealed a multi-cystic mass in the subaortic area. A left thoracotomy was performed without definitive preoperative diagnosis. The tumor was strongly adherent to the pericardium and the surrounding large vessels, but a complete resection was undertaken. Histological and immunohistochemical examination revealed that it was a malignant paraganglioma with a microscopically positive surgical margin. After radiotherapy of 50 Gy for the mediastinum, the patient almost recovered from his hoarseness by thyroplasty. We also reviewed nine Japanese cases of APPG reported previously. Though APPG is rare, we must consider that a middle mediastinal tumor may be APPG, and preoperative examination and preoperative planning are necessary to prevent massive bleeding and microscopic residual tumor.
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5/12. Angiographic features of lateral cervical masses.

    An audible bruit may represent a clue to the vascular nature of the lesion. angiography is definitely indicated in the presence of pulsatile cervical masses with or without an associated bruit, and should be more widely utilized in the future for evaluation of cervical masses. In addition to the nature of the mass, angiography may also be of value in outlining the extent of the mass and its relationship to major vessels. Magnification and subtraction angiographic techniques with their improved detail may play an important role in clarifying the etiology of cervical masses, and hence facilitate the plan of therapy. If reasonable uncertainty as to the clinical diagnosis exists, angiography should be considered a diagnostic aid.
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6/12. angiography in the diagnosis and management of extracranial vascular lesions of the head and neck.

    The angiographic features of various lesions of the head and neck are presented. Angiographically, cavernous hemangiomas display large venous lakes with calcified phleboliths. arteriovenous malformations reveal massive tumor stain with well delineated feeding vessels from multiple systems. Chemodectomas and juvenile nasopharyngeal angiofibromas are clearly vascular with homogenous tumor staining in the capillary phase. angiography of cavernous hemangioma, AVM, chemodectoma, and angiofibroma is diagnostic and may preclude the need for tissue biopsy. Angiographically neurilemmomas are less vascular with non-homogenous tumor stain. Carcinomas are typically avascular. The use and benefits of arterial embolization in the management of these lesions is presented.
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7/12. Immediate postembolization excision of glomus jugulare tumors: advantages of new combined techniques.

    Preoperative percutaneous transfemoral catheter embolization of feeding vessels in glomus jugulare tumors, followed by immediate application of standard surgical techniques, presents the treatment of choice, allowing meticulous microsurgery with virtually complete hemostasis. Therefore, the surgeon can operate in a bloodless environment throughout the compressed and intricate anatomic field, amidst such important yet vulnerable structures as cranial nerves, inner ear, carotid artery, jugular bulb, venous sinuses, and dura, while reducing surgical error and functional deficit for the patient. review of the last 11 cases of glomus jugulare tumors at UCLA shows that even extensive Alford grade 2 tumors of the middle ear, jugular bulb, and mastoid had only minor blood losses with this combined technique of embolization-immediate surgery, as compared with earlier surgical methods. Pertinent literature on glomus jugulare and its treatment is reviewed. Combined embolization and immediate surgery offer the best approach for treatment of resectable glomus jugulare tumors.
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8/12. paraganglioma presenting with an aortic aneurysm.

    The concurrence of a paraganglioma of the organ of Zuckerkandl and an abdominal aortic aneurysm is reported in a 72-year-old caucasian male. Computerized tomography scan confirmed the aneurysm and noted a well-circumscribed tumour of the aneurysmal wall. At operation, the tumour was identified and excised with part of the aneurysmal wall. The tumour exhibited all the features of a paraganglioma while the vessel wall showed atherosclerosis and secondary degenerative changes. The features of the aortico-sympathetic group of paragangliomas are discussed.
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9/12. Orbital nonchromaffin paraganglioma. A case report and review of the literature.

    Nonchromaffin paraganglioma (NCP), also called glomus body tumor or chemodectoma, is rarely found in the orbit. The behavior of orbital nonchromaffin paraganglioma may potentially be more aggressive than in other head and neck locations. diagnosis depends on electron microscopic demonstration of membrane-bound neurosecretory granules. Results of histopathologic study show a well-circumscribed lesion without a true capsule with alveolar or organoid arrangements of epithelioid cells within a reticulin framework with thin-walled blood vessels. Cells are polygonal with round or oval nuclei containing rare mitotic figures and pale-staining cytoplasm. Differential diagnosis includes alveolar soft-part sarcoma, alveolar rhabdomyosarcoma, neuroblastoma, carcinoid, and granular cell tumor. Of 29 previously reported cases of orbital NCP, 16 have been reclassified as alveolar soft-part sarcoma. The authors report a patient with an electron microscopically established orbital NCP, with the history of a contralateral glomus jugulare tumor irradiated 14 years previously.
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10/12. Superselective embolization of glomus jugulare tumors.

    The purpose of this report is to introduce the technique of superselective embolization (SSE) and to review our experience in surgically treating glomus jugulare tumors with and without preoperative SSE. Retrospective chart review was performed to determine estimated blood loss and operative time, and illustrative case reports are presented. The technique of preoperative SSE represents an important advance in the surgical management of glomus jugulare tumors. In our experience, surgical ligation of tumor-feeding vessels or nonselective embolization of the external carotid system is unsatisfactory. Inadequate devascularization and excessive risk of complications have caused us and others to abandon these procedures. However, SSE performed by an experienced neuroradiologist can produce effective and safer tumor devascularization. Preoperative SSE results in shrinkage of tumor size and significantly decreases blood loss. The advantages to the surgeon include improved visualization and ease of dissection as well as increased confidence that complete tumor excision has been achieved.
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