Identification, classification, treatment, and prognosis of laryngeal paraganglioma. Review of the literature and eight new cases.

This study details the clinicopathologic features of 62 cases of laryngeal paraganglioma (LP), including 54 acceptable cases identified in the literature (although clinical information is lacking on 7 of these) and 8 previously unpublished cases identified from the Registry of Otolaryngic-Endocrine Pathology at the Armed Forces Institute of Pathology. Demographic findings show that the overwhelming majority of cases affect women (41:14), mainly in the fourth to sixth decades of life (age range, 14 to 83 years; median, 44 years), with a prevalence in the supraglottic larynx. These neoplasms are treated by surgical resection and are benign. Despite the characteristic pathologic features associated with LP, it is sometimes confused with other neoplasms, particularly neuroendocrine carcinomas of the larynx, and this confusion leads to unfortunate designations such as malignant paraganglioma and metastasizing paraganglioma of the larynx. Judging from the cases reported in this study and those identified in the literature, we conclude that malignant biologic behavior associated with LP is extraordinarily rare (< 2%). Because of the misdiagnoses of LP, the prognosis associated with this entity has been skewed to suggest that LP may behave aggressively. This has led to the inappropriate classification of LP among the malignant categories of laryngeal neuroendocrine neoplasms. The goal of this study is to detail the features diagnostic of LP and to discuss the appropriate treatment, prognosis, and classification of these neoplasms.