Paraganglioma

ICD-O:8680/1

Paragangliomas are rare benign tumours, originating from the anatomically dispersed neuroendocrine system (paraganglia), characterised by similar neu-rosecretory cells derived from the neural crest and associated with autonomic ganglia. The extra-adrenal portions of the paraganglia are divided into four groups according to their distribution and innervations: branchiomeric, intravagal, aortosympathetic and visceral autonomic. The paraganglia of the larynx are part of the branchiomeric paraganglionic system, together with the jugulotympanic, carotid body, subclavian, coronary, aorticopulmonary, pulmonary and orbital paraganglia [18].

Laryngeal paragangliomas (LP) are extremely rare, almost always benign tumours originating in the superior and inferior paired paraganglia. The former are localised in the false vocal cords, the latter in the vicinity of the cricoid cartilage [18, 255]. Patients are usually of middle age with a median age of 47 years. Surprisingly, compared with other neuroendocrine tumours, the LPs are three times more common in women. The predominant site is the supraglottic (82%), followed by the subglottic in 15% and the glottic area in 3% of cases. Signs and symptoms are mainly related to the localisation and size of the tumour [45, 289]. LPs are rarely functional, multicentric or associated with other head and neck paragangliomas.

Macroscopically, the tumour usually presents as a rounded submucosal mass with an intact covering mucosa, ranging in size from 0.5 to 6 cm [18]. The tumours are firm; on the cut surface they may be homogenous or nodular, from pink to tan and dark red in colour. Prominent vascularity of the tumour may cause abundant bleeding during biopsy.

Histologically, LPs are composed of two cell types: chief and sustentacular or supporting cells. The chief cells of epithelioid appearance are packed into round nests showing an organoid pattern, surrounded by highly vascular fibrous tissue (i.e. Zellballen; Fig. 7.11a). However, the characteristic cell nests may be squeezed and not apparent in a small biopsy specimen. The chief

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