Research

Paraganglioma of the endolarynx: a rare tumor in an uncommon location

Joseph R Smolarz¹ , Ehab Y Hanna² , Michelle D Williams³ and Michael E Kupferman²

¹  Department of Otorhinolaryngology, Head and Neck Surgery/Otolaryngology, The University of Texas Health Science Center at Houston, 6431 Fannin, Suite MSB 5.036, Houston, TX 77030, USA

²  Department of Head and Neck Surgery, The University of Texas M.D. Anderson Cancer Center, 1400 Pressler, Unit 1445, Houston, TX 77030, USA

³  Department of Pathology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd, Unit 0085, Houston, TX 77030, USA

author email corresponding author email

Head & Neck Oncology 2010, 2:2doi:10.1186/1758-3284-2-2

Published:	19 January 2010

Abstract

Background

Less than 80 reported cases of paragangliomas of the larynx are reported in the literature. A role for external beam radiation in this disease has not yet been explored. We present four cases of laryngeal paragangliomas treated at a large tertiary-care cancer center over a 35-year period.

Methods

124 cases of head and neck paragangliomas treated at a single institution from 1970 to 2005 were retrospectively studied. Patients with laryngeal paragangliomas were identified, and a comprehensive clinico-pathological review was undertaken.

Results

We identified 4 patients with tumors arising in the larynx at the following subsites: supraglottis (2), glottis (1), and subglottis (1). Three patients were treated with surgery and one with definitive radiation alone.

Conclusions

Laryngeal paragangliomas are rare tumors and are adequately treated with surgical resection. We also present one patient who was treated with radiation and had disease stabilization. Accurate histological classification is critical, and the role of genetic testing is emerging.