Extracardiac Rhabdomyoma of the Larynx

Case Presentation and Discussion

A 66-year-old man with a history of chronic obstructive pulmonary disease, migraines, tobacco use, and nonsmall-cell lung cancer presented with dysphagia, dysphonia, and shortness of breath. He reported a 30-pound weight loss and worsening dyspnea over the preceding month. Flexible nasolaryngoscopic examination demonstrated a large submucosal mass of the right hypopharynx, which was displacing the right arytenoid medially (Figure 1). Furthermore, the patient had reduced abduction of the right vocal fold and a mildly impaired glottic airway. Computed tomography (CT) of the neck showed an enhancing mass occupying the right pyriform sinus and posterior paraglottic space with extralaryngeal extension through the thyrohyoid space without cartilage destruction (Figure 2). There were no suspicious lymph nodes identified on this CT scan. Due to clinical concern for a primary laryngeal malignancy or lung cancer metastasis, the patient underwent triple endoscopy with biopsy of the laryngeal lesion. Final pathology showed polygonal eosinophilic tumor cells with vacuolated areas, consistent with adult-type extracardiac rhabdomyoma (Figure 3).

Figure 1.

Endoscopic view of patient’s larynx. Endoscopy demonstrates a large submucosal mass of the right hypopharynx. This mass displaces the right arytenoid medially and causes mild narrowing of the airway. Ar indicates arytenoid; PS, pyriform sinus; T, tumor.

Figure 2.

Computed tomography scan of the neck. An axial section through this CT neck with contrast shows an enhancing mass occupying the right pyriform sinus and posterior paraglottic space with extralaryngeal extension through the thyrohyoid space. There does not appear to be any evidence of cartilage destruction nor suspicious appearing lymph nodes on this scan.

Figure 3.

Hematoxylin and eosin stain slide from the biopsy of this patient’s laryngeal lesion. This pathology slide depicts polygonal eosinophilic tumor cells with vacuolated areas. These characteristics are further magnified within the inset of this figure. These pathologic features are consistent with adult-type extracardiac rhabdomyoma.

Extracardiac rhabdomyoma is a rare, benign mesenchymal tumor originating from striated muscle. It comprises 2% of neoplasms of striated muscle origin and is categorized into adult, fetal, and genital forms based on the degree of skeletal muscle differentiation. ^1,2 Although a rare neoplasm of the head and neck overall, the head and neck is the most common site for extracardiac rhabdomyomas with case reports describing these tumors in the larynx, pharynx, parapharyngeal space, and submandibular region. ^{3 -6} Symptoms are typically insidious and related to local progression including airway obstruction, stridor, and dysphagia. Recommended treatment is complete surgical resection, and a 10% to 40% risk of recurrence is reported in the literature. ⁷ Unfortunately, the patient presented in this case died of complications related to acute pancreatitis before operative intervention could be considered.