Adult-type rhabdomyoma of the larynx: clinicopathologic study of an uncommon tumor in a rare location

Rhabdomyoma is an uncommon benign mesenchymal tumor with skeletal muscle differentiation that may occur either in the heart or in extra-cardiac sites. Even though the head and neck region is the most common area of extra-cardiac rhabdomyoma, the larynx is rarely involved. We present the case of an 85-year-old woman who reported a 10-day history of breathing difficulties, dysphagia and dysphonia. A computed tomography scan of the head and neck showed a contrast-enhanced, solid hypopharyngeal-laryngeal neoplasm with well-defined margins causing subtotal obliteration of the right pyriform sinus and a reduction in air lumen of the laryngeal vestibule. The patient underwent complete endoscopic removal of the lesion; histologic examination revealed an adult- type rhabdomyoma based on the histologic features and the immunoreactivity of the neoplastic cells for desmin, myoglobin and muscle-specific actin but not for cytokeratin, S-100, CD68R, chromogranin-A and synaptophysin. Since clinical and imaging features are not specific for rhabdomyoma, histologic examination and immunohistochemical analyses play a central role in the differential diagnosis of the adult-type rhabdomyoma from other laryngeal neoplasms. A correct diagnosis is mandatory to avoid inappropriate treatment.