Sclerosing rhabdomyosarcoma: a rare variant with predilection for the head and neck

Sclerosing rhabdomyosarcoma is a newly described variant of rhabdomyosarcoma with a predilection for the head and neck. Little has been written on the topic, because of the scarcity of the disease and its recent recognition as a distinct entity. The present report describes the fifth confirmed case of sclerosing rhabdomyosarcoma and is the first report in the otolaryngology literature.

Case report.

The authors have reported the case of a 66-year-old woman with a 35-year history of heavy cigarette smoking and daily alcohol consumption and a 2-month history of progressive dysphagia and dysarthria secondary to an enlarging tongue mass. Urgent tracheotomy was performed for impending respiratory embarrassment. Direct laryngoscopy revealed a bulky, exophytic mass involving the base of tongue. Specimens were obtained and submitted for analysis.

Initial frozen-section analysis of the specimens favored carcinoma, although subsequent immunohistochemical analysis disproved this. The diagnosis of sclerosing rhabdomyosarcoma was based on microscopic appearance and patterns of gene expression, including the expression of desmin and myogenin. A search of the literature revealed only four confirmed cases of sclerosing rhabdomyosarcoma. With the inclusion of the oropharyngeal tumor in the present report, three of the five confirmed cases have occurred in the head and neck.

Sclerosing rhabdomyosarcoma is a rare variant of rhabdomyosarcoma that has a predilection for the head and neck. The clinical presentation may mimic carcinoma. The otolaryngologist-head and neck surgeon must be familiar with this disease entity.