Recurrent Rhabdomyoma of the Parapharyngeal Space

David Goldfarb, DO, Louis D. Lowry, MD, and William M. Keane, MD

Benign skeletal muscle neoplasms are rare, especially when compared with their malignant counterparts. Rhabdomyomas have been reported to account for only approximately 2% of all striated-muscle neoplasms.1 Although it is difficult to know the true incidence of this rare tumor, up to 76% of reported non-cardiac cases have been found to occur in the head and neck region.2

Rhabdomyomas can be subdivided into different groups, based on clinical and morphological differences. The purpose of this paper is to share a unique case presentation of a man who developed a recurrence of a parapharyngeal rhabdomyoma 25 years after primary surgical excision. This paper will review the literature and discuss presenting symptoms, differential diagnosis, histopathological findings, and treatment.

Case Report

The patient is a 64-year-old white man, status post-resection of a parapharyngeal space mass 25 years before our meeting him. The patient presented with a chief complaint of a mass growing in the left soft palate region, progressive in size over the last 6 months. The patient in our case report was 39 years of age when he was originally treated. He originally told us that he had a benign tumor removed from the floor of his mouth 25 years ago. We were considering a salivary gland tumor as the most likely culprit, because they are fairly common neoplasms of the parapharyngeal space.3 After checking the patient's records from another hospital, we learned that a rhabdomyoma was removed from his left parapharyngeal space. Our patient was free of symptoms until 6 months before our evaluation, when he noticed a mass in his left soft palate region. During the physical examination, a large mass was found deviating the parapharyngeal space, soft palate, and tonsil. The tongue base, vallecula, and hypopharynx were clear. Nasopharyngoscopy showed no lesions in the nasopharynx or larynx; the vocal folds moved well. The epiglottis, pyriform sinuses, and hypopharynx were clear. The neck had an old, healed tracheotomy scar and labiomandibulotomy scar. No evidence of cervical adenopathy, bruits, or tenderness was found. Neurologically, the patient had no focal signs and cranial nerves 2 through 12 were intact. The rest of his physical exam was unremarkable.

A magnetic resonance imaging (MRI) scan was carried out with MR angiography. Intravenous gadolinium was administered, and Tl-weighted axial and coronal images were obtained using fat suppression. The MRI scan showed a large, left-sided mass in the parapharyngeal space (Fig 1). with extension to the tongue base and left tonsilar fossa. The MR angiography did not show any major blood supply. Both the carotid arteries were shown to be patent.

The patient underwent a tracheotomy, labioman dibulotomy, and resection of the tumor. The mass was 13- to 14-cm in diameter and partially obstructed the oropharynx. The whole tumor was sent to pathology as one large bulk specimen. The surgical pathology report confirmed the diagnosis of adult rhabdomyoma.

Pathology

Gross Specimen

The specimen was brown, soft, and nodular. Sections were sent for histological examination. The brown color is thought to represent the high content of mitochondria.

58 American Journal of Otolaryngology, Vol 17, No 1 (January-February), 1996: pp 58-60

From the Department of Otolaryngology-Head and Neck

Surgery, Medical Center at Princeton, Princeton, NJ; and the Department of Otolaryngology-Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, PA.

Address reprint requests to David Goldfarb, DO