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11/41. Bilateral chemodectoma in the neck.

    A 55-year-old man with bilateral cervical chemodectoma is reported. The differences between tumours of the carotid body and those of the glomus intravagale are described and a brief review of the investigation and management of such tumours is given.
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12/41. Chemodectoma of the larynx. A clinico-pathological study.

    The present case report is concerned with a clinico-pathological study, including ultrastructural investigation, of a rare and uncommon laryngeal tumour, a chemodectoma, in a 62 year old patient. There have been 23 cases of laryngeal chemodectomas reported in the literature, and only three of them, including our own report, were investigated by electron microscopy. The tumours arise from the superior and inferior larynegeal nonchromaffin paraganglia or possibly from Kultschitzky-cells of the normal bronchial mucosa. Ultrastructurally they have all the characteristics of apudomas whose parent cells (APUD-cells), usually show endocrine function and probably have their origin in the neural crest. The tumours show an aggressive type of behaviour, despite usually benign histological features when compared to chemodectomas at other sites in the head and neck region. Surgery is thus the therapy of choice.
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13/41. 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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14/41. Scintigraphic evaluation of glomus tumours.

    The current investigations of choice for a suspected glomus tumour are either direct or indirect angiography to include digital subtraction followed by computerized tomography (CT) or magnetic resonance imaging (MRI) or, if available, CT and MRI with gadolinium alone. Although these modalities confirm the diagnosis and give anatomical information to facilitate accurate staging, they do not provide functional data. The use of radionuclide scintigraphy can add an extra physiological dimension to glomus tumour imaging. iodine-131/123 metaiodobenzylguanidine (MIBG) is a tumour imaging agent which has been used to diagnose head and neck neuroendocrine tumours to include paragangliomata and medullary carcinoma of the thyroid (MCT). However, it is expensive and the new head and neck tumour imaging agent technetium-99 m (Tc99m) (v) dimercaptosuccinic acid (DMSA) has superceded it as the imaging agent of choice to evaluate MCT. We report a patient with a glomus jugulare tumour which was evaluated with I131/I123-MIBG and Tc99m (v) DMSA. The tumour was functional and is the first reported case exhibiting positive accumulation of both I131-MIBG and Tc99m (v) DMSA. The patient was subsequently treated with a therapeutic dose of I131-MIBG. The significance of these results is discussed.
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15/41. Chemodectoma in the mid-thyroid region.

    A chemodectoma (non-chromafin paraganglioma), which presented as a neck mass in the mid-thyroid region, is reported in a 36 year old female. Clinically, it resembled a thyroglossal duct cyst and on frozen section it looked like a thyroid adenoma.
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16/41. Catecholamine-secreting paraganglioma of glomus jugulare region.

    A 43-year-old woman had clinical and biochemical evidence of a secreting paraganglioma of the glomus jugulare region. Catecholamine secretion was exacerbated during embolization of the tumor before surgery and resulted in a life-threatening vasomotor attack. Preoperatively, pharmacologic blockade of excessive catecholamine secretion with prazocin controlled her blood pressure, tachycardia, and symptoms. The tumor was resected and its catecholamine content measured. This case is reported to stress the importance of adequate preoperative assessment of patients with paragangliomas of the head and neck. The extreme rarity of catecholamine-secreting tumors of this region should not lead us to underestimate the morbidity and mortality of such patients undergoing surgery or any other invasive procedure whether the diagnosis is confirmed or only suspected.
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17/41. Multicentric chemodectoma in the head and neck.

    A case of a multicentric chemodectoma, with the unusual combination of glomus vagale and glomus tympanicum, is presented. The patient, though asymptomatic, showed elevated levels of urinary catecholamines, suggesting some biochemical activity of the tumour. Multicentricity was unsuspected prior to selective carotid arteriography. Intra-operative haemostasis was assisted by the utilization of pre-operative selective embolization of the tumour mass. Subsequently, the injected Ivalon was confirmed histologically in the specimen. The asymptomatic small glomus tympanicum tumour was treated using embolization alone but only time will determine the effectiveness of this method of management. The vagus nerve was anatomically spared, but complete function had not returned after 2 years of follow-up.
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keywords = neck
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18/41. 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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19/41. Familial malignant retroperitoneal paraganglioma.

    Paragangliomas are neuroendocrine tumours and those occurring in the head and neck have well recognized familial association. Retroperitoneal paragangliomas are uncommon and we present two cases of familial malignant retroperitoneal paraganglioma. review of the literature revealed marked differences in the incidence and malignant potential of familial and non-familial paraganglioma. In contrast to the cases reported here, familial tumours are generally benign, though they may occur at multiple sites. Familial and non-familial paragangliomas may indeed be different disease entities.
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20/41. radionuclide angiography and surgery for familial bilateral chemodectomas.

    carotid body tumours, or chemodectomas, can occur on both sides of the neck (5%), and this bilaterality increases sixfold (30%) where a familial pattern is evident. The known pathological behaviour of chemodectomas makes early recognition and management desirable, especially when an inherited predisposition can be anticipated. Difficulties in clinical diagnosis and the hazards of blind exploration have imposed a reliance on contrast angiography, which is a potentially dangerous procedure. The family tree of a patient with bilateral chemodectomas was traced and 28 individuals were studied using radionuclide angiography (99mTechnetium gluconate), a quick, safe technique with a high yield. Bilateral chemodectomas were observed in five siblings, establishing conclusively the existence of a dominant hereditary trait. Excision of newly discovered tumours was accomplished without complication. The techniques which permitted such a favourable surgical outcome are discussed, emphasising the view that these tumours are best dealt with by vascular surgeons.
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