Asymptomatic Rhabdomyoma of the Larynx: Case Report and Review of the Literature

Case Report

Ann Hematol Oncol. 2017; 4(2): 1135.

Asymptomatic Rhabdomyoma of the Larynx: Case Report and Review of the Literature

Ahmed HS¹, Parikh AS², Srikanth P², Tjoa T³, Faquin WC4 and Lin DT²*

¹Harvard Medical School, USA

²Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, USA

³Department of Otolaryngology, University of California Irvine, USA

4Department of Pathology, Massachusetts Eye and Ear Infirmary, USA

*Corresponding author: Lin DT, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA

Received: January 09, 2017; Accepted: February 13, 2017; Published: February 17, 2017


Rhabdomyoma of the head and neck is an uncommon tumor. Tumor occurrence in the larynx is particularly rare, with only 53 reported cases. We describe the case of a 59-year-old woman with an asymptomatic, incidentally discovered adult type rhabdomyoma of the left aryepiglottic fold, successfully treated with endoscopic resection. We also conduct a comprehensive review of the literature, including operative management and surgical outcomes. Of 53 cases reviewed, the mean age at diagnosis was 33.8 years and a 2.4:1 male predominance was observed. The most common presenting symptom was hoarseness, and the most common site of origin was the true vocal fold. Endoscopic and open resection were used at similar rates, and there were no obvious differences in patient characteristics by surgical approach. However, a higher recurrence rate and shorter time to recurrence were observed with endoscopic resection, as compared with open resection. Thus, we suggest the potential downside of endoscopic management that must be balanced with the potential for significantly lower morbidity and ease of re-resection when considering the appropriate surgical approach for a given patient.

Keywords: Larynx; Rhabdomyoma; Laryngeal neoplasms


Rhabdomyomas are extremely rare benign neoplasms of striated muscle and comprise less than 2% of all skeletal muscle tumors [1]. Most extracardiac rhabdomyomas occur in the head and neck, specifically the oropharynx, larynx, pharyngeal constrictor muscles, submandibular region, base of the tongue, and less commonly lip, lateral neck, soft palate, uvula, cheek, and orbit [2-6].

Rhabdomyomas of the head and neck are slow-growing and wellcircumscribed; malignant transformation is rare [4,7]. Treatment generally involves surgical excision of the lesion, preventing invasion of surrounding tissues and obstruction of the airway or esophageal inlet. Although treatment is typically curative, these lesions do occasionally recur. Histologically, rhabdomyomas are divided into two types: neoplastic and hamartoma [8]. Neoplastic rhabdomyomas are further classified into adult, fetal, and genital (or vaginal) subtypes [8]. Adult type rhabdomyomas are characterized by sheets of closelypacked polygonal vacuolated (glycogen-containing) cells with granular eosinophilic cytoplasm, with occasional cross-striations and prominent nucleoli [2,3]. Fetal cellular type is characterized by immature skeletal muscle elements at varying stages of differentiation [2], while genital cellular type appears as a mixture of fibroblast-like cells with clusters of mature cells with cross-striations and collagenand mucoid-rich matrix [3].

Clinically, adult type rhabdomyomas occur in the soft tissues of the head and neck in 70 to 93% of cases; the fetal subtype is most prevalent in children, and the genital (also known as vaginal) type is typically a polypoid mass found in the vagina and vulva of middle-aged women [3,4,6]. The most common presenting symptoms for the adult type are hoarseness, dyspnea, and dysphagia [3,4]. Fetal type rhabdomyomas present with obstructive and constrictive oropharyngeal symptoms, and the genital type is typically asymptomatic but presents with dyspareunia [3,9].

Here, we describe the case of an incidentally discovered adult rhabdomyoma of the larynx in a 59-year-old woman. We also systematically review 53 cases of laryngeal rhabdomyoma reported in the literature, which to our knowledge, has not been done previously.

Case Presentation

The patient is 59-year-old woman with a 40 pack year smoking history who presented to her primary care physician for routine care. Given her extensive smoking history, a screening chest CT was ordered and demonstrated a left upper lobe lung mass. Subsequent PET-CT showed this lung mass, as well as an incidental 3.2 cm left supraglottic laryngeal mass, and the patient was referred to otolaryngology. Fiberoptic laryngoscopy showed a large obstructing left arytenoid mass with normal overlying mucosa. Dedicated neck CT showed a well-circumscribed, oval, left supraglottic mass measuring 3.3x2.8x1.8 cm, centered in the left piriform sinus and along the left aryepiglottic fold, without involvement of the true vocal folds (Figure 1).