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

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.

Figure 1

Case No. 3 (A) Pretreatment stroboscopy revealed tumor involvement in the right AE fold, extending down into the right piriform sinus (B) Post-radiation - Only a small remnant of tumor remained after radiation and continued to be stable throughout follow-up.

Figure 2

Case No. 4 (A) A large laryngeal paraganglioma is noted in the left arytenoid area. (B) There is involvement of the laryngeal and lingual surfaces of the epiglottis.

Figure 3

Case No. 4 - (A) Epiglottic extension (B) Tumor pushing the airway to the right (C) Near-total laryngeal obstruction (D) Extension of tumor is found in the left anterior subglottis.

Figure 4

Case No. 4 (A) Overlying squamous epithelium with the tumor in the submucosa. The circular focus is a post-embolized vessel. (B) Higher power 400× - Zellballen nests of tumor cells with thin supporting sustenacular cells around each nest.