Myssiorek David, Rinaldo Alessandra, Barnes Leon, Ferlito Alfio
Department of Otolaryngology, Long Island Jewish Medical Center The Long Island Campus of the Albert Einstein College of Medicine, NY, USA.
Acta Otolaryngol. 2004 Nov;124(9):995-9. doi: 10.1080/00016480410017576.
Laryngeal paragangliomas are rare submucosal lesions that arise from paraganglion cells located in the false vocal fold and subglottic larynx. To date, 76 recognized cases have been reported in the world literature. Symptoms arise when the lesions become large enough to impair function. Supraglottic paragangliomas cause hoarseness and deglutition disorders, whereas subglottic tumors become symptomatic when they obstruct the airway. Evaluation of these tumors includes obtaining a complete history. Familial paragangliomas and hypertension should be sought but are rarely, if ever, associated with laryngeal paragangliomas. MRI can detect these lesions and permit characterization of the vascularity of the lesion. Adding 111In pentetreotide scanning can distinguish neuroendocrine tumors from other submucosal laryngeal lesions, making the preoperative diagnosis clearer and obviating the need for biopsy. The biggest dilemma regarding laryngeal paragangliomas is making the correct pathologic distinction between paraganglioma, typical carcinoid, atypical carcinoid and medullary thyroid cancer. Immunohistochemical markers, supplementing standard histopathologic evaluation, can distinguish paragangliomas from the aforementioned tumors. This distinction is critical as the prognosis for treated paragangliomas is excellent compared to that for other neuroendocrine neoplasms. Almost all alleged malignant paragangliomas of the larynx are in reality atypical carcinoid tumors that have been misdiagnosed. Treatment should always comprise excision. Thyrotomy has the best chance of achieving a sustained cure without damaging phonation or deglutition. Laser excision has been used successfully but there is no great experience with this modality. Surgery is preferable to radiation for paragangliomas in all locations but especially so in the larynx, due to issues such as swelling, airway protection and destruction of cartilage. With increased clinical suspicion and the use of modern imaging techniques, laryngeal paragangliomas should be routinely diagnosed and treated without loss of laryngeal functions.
喉副神经节瘤是一种罕见的黏膜下病变，起源于位于假声带和声门下喉的副神经节细胞。迄今为止，世界文献中已报道76例确诊病例。当病变大到足以损害功能时就会出现症状。声门上副神经节瘤会导致声音嘶哑和吞咽障碍，而声门下肿瘤在阻塞气道时会出现症状。对这些肿瘤的评估包括获取完整病史。应排查家族性副神经节瘤和高血压，但它们与喉副神经节瘤很少（如果有的话）相关。磁共振成像（MRI）可以检测到这些病变，并能对病变的血管情况进行特征描述。添加铟-111奥曲肽扫描可以将神经内分泌肿瘤与其他喉黏膜下病变区分开来，使术前诊断更清晰，无需进行活检。关于喉副神经节瘤最大的难题是在副神经节瘤、典型类癌、非典型类癌和甲状腺髓样癌之间做出正确的病理区分。免疫组化标志物辅助标准组织病理学评估，可以将副神经节瘤与上述肿瘤区分开来。这种区分至关重要，因为与其他神经内分泌肿瘤相比，经治疗的副神经节瘤预后良好。几乎所有所谓的喉恶性副神经节瘤实际上都是误诊的非典型类癌肿瘤。治疗应始终包括切除。甲状软骨切开术在不损害发声或吞咽功能的情况下实现持续治愈的机会最大。激光切除已成功应用，但对此方法经验不多。对于所有部位的副神经节瘤，手术优于放疗，在喉部尤其如此，因为存在肿胀、气道保护和软骨破坏等问题。随着临床怀疑增加以及现代成像技术的应用，喉副神经节瘤应常规诊断和治疗，同时不丧失喉功能。
