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A 53-year-old African American man with a history of bipolar disorder presented to the emergency department with an enlarging right-sided tongue mass that had caused mild discomfort and halitosis over the course of 1 week. He denied recent injury, fever, chills, odynophagia, or difficulty breathing. Laboratory examination was significant for leukocytosis (white blood cell count, 15 700/μL) and hypercalcemia (total calcium level, 13.1 mg/dL) (to convert white blood cells to ×109 per liter, multiply by 0.001; to convert calcium to millimoles per liter, multiply by 0.25). Physical examination was notable for a 4- to 5-cm pedunculated, ulcerative mass on the dorsal oral aspect of the tongue near midline, anterior to the foramen cecum, without palpable cervical lymphadenopathy. Contrast-enhanced computed tomography (CT) demonstrated a rim-enhancing, centrally hypodense lesion in the right dorsal tongue abutting the lingual septum with a somewhat ill-defined border (Figure 1); there was no associated osseous erosion of the mandible or hard palate, no tongue prolapse to suggest hypoglossal nerve palsy, no involvement of the soft palate, and no cervical lymphadenopathy by radiological size criteria. An excisional biopsy of the anterior aspect of the mass was performed at bedside, and the patient was discharged home with instructions to follow up in the otolaryngology clinic.
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D. Pleomorphic rhabdomyosarcoma
Fluorodeoxyglucose F18–labeled positron-emission tomography demonstrated the lesion to be intensely avid (standardized uptake value, 16.4) (Figure 2).
Rhabdomyosarcoma (RMS) is an aggressive cancer of mesenchymal origin, most common in the pediatric population, with 35% occurring in the head and neck region1; the tongue is an uncommon site of involvement. World Health Organization classification of RMS is based on histologic findings and includes 4 groups: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing.2 Pleomorphic RMS is rare and is seen almost exclusively in adults.3 Wide surgical resection is the mainstay of treatment for localized pleomorphic RMS, followed by postoperative radiotherapy because this RMS subtype is sometimes regarded as chemoresistant.2,4 In patients with metastatic disease, palliative radiation therapy and chemotherapy may be attempted.2 Pleomorphic RMS heralds poor prognosis by virtue of its histologic characteristics as well as the affected age group.4 Overall survival rates in the adult population with RMS of all sites and all histologic traits range from 40% to 54% at 5 years.5
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Corresponding Author: Sara B. Strauss, MD, Montefiore Medical Center, 111 E 210th St, Bronx, NY 10467 (email@example.com).
Published Online: February 7, 2019. doi:10.1001/jamaoto.2018.4038
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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