Failed organ preservation strategy for adult laryngeal embryonal rhabdomyosarcoma

To present a case of embryonal rhabdomyosarcoma of the intrinsic laryngeal musculature and discuss the treatment of this rare tumor.

A 45year-old male presented with an eleven month history of hoarseness. A mass of the posterior glottis was noted on fiberoptic laryngoscopy. Computed tomography indicated a 1.5×2.5cm laryngeal mass without cartilage involvement. Direct laryngoscopy and biopsy were consistent with embryonal rhabdomyosarcoma involving the interarytenoid muscle. A multidisciplinary tumor board recommended multimodality therapy including total laryngectomy. His case was reviewed along with the available English language literature.

The patient refused surgery, and he qualified for a pediatric rhabdomyosarcoma treatment protocol with induction chemotherapy followed by combined chemoradiation. There was no noted response and the patient was taken off protocol to increase the radiation dose without chemotherapy. Fifteen weeks following radiation, a repeat biopsy revealed viable tumor. The patient elected further experimental chemotherapy at an outside hospital. The patient currently is alive at 34months post-treatment with suspected persistent disease and continues to refuse surgery.

Embryonal rhabdomyosarcoma involving the larynx is an extremely rare tumor usually seen in children. There have been few previous adult cases reported and treatment options are not well described. We present a case of chemoradiation failure in an adult with embryonal rhabdomyosarcoma refusing surgical intervention. Although pediatric tumors can be effectively treated with organ preservation strategies, adult tumors may have a poorer response. Based upon our experience and existing literature regarding adult embryonal rhabdomyosarcoma of the larynx, multimodality therapy including surgical resection should be the treatment of choice.