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An Aggressive Sinonasal Mass With Parameningeal Extension

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 60s presented with a 3-month history of right-sided sinus pressure progressing to dental pain. She was prescribed antibiotics and steroids by her family physician, which initially provided intermittent, symptomatic improvement; however, she ultimately experienced a progressive decrease in tactile sensation on her face, anosmia, and changes in her right peripheral vision. She had a remote 2 pack-year smoking history. Sinus computed tomography (CT) and neck magnetic resonance imaging (MRI) revealed a large expansile mass arising from the right side of the skull base and nasopharynx with extension into the pterygopalatine fossa, masticator, parapharyngeal, retropharyngeal, and carotid spaces. In addition, imaging showed involvement of the right posterior orbit with erosion of the posterior maxillary sinus wall, anterior and middle cranial fossa with erosion through the cribriform plate, and cavernous sinus. Physical examination revealed slight right-sided facial fullness, grossly intact extraocular motion, decreased vision in the right lateral field, and palpable bilateral level 2 neck lymph nodes. Flexible laryngoscopy revealed a right-sided lobulated mass involving the medial middle turbinate, posterior septum, and nasal floor, as well as the posterior aspect of the maxillary sinus extending superiorly to the nasopharynx and skull base posteriorly. The mass extended into the left superior nasopharynx and superior septum. In-office biopsy specimens were obtained and revealed a primitive small round cell tumor with the following immunohistochemical (IHC) staining profile: diffusely positive for desmin, myogenin, and CD56; focally positive for low-molecular-weight kininogen (LMWCK), p63, and S-100; and negative for AE1/AE3, CK 5/6, CK7, CK20, p40, chromogranin, SOX10, HMB45, CD99, and CD20 (Figure 1).

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C. Alveolar rhabdomyosarcoma

These histologic and IHC findings are consistent with the alveolar subtype of rhabdomyosarcoma (RMS). Although it is the most common soft-tissue sarcoma in children, RMS represents only 2% to 5% of adult soft-tissue tumors.1 RMS is divided into 3 main histologic subtypes: embryonal, alveolar, and pleomorphic.1 The embryonal subtype is more common in children and frequently occurs in the genitourinary tract and the head and neck region. The alveolar and pleomorphic subtypes are more common in adults and often occur in the extremities, as well as in the head and neck region.2 Although alveolar RMS is more commonly seen in adults, this diagnosis is often unexpected in the older age group.3

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Article Information

Corresponding Author: Wojciech K. Mydlarz, MD, Department of Otolaryngology–Head and Neck Surgery, Johns Hopkins University School of Medicine, 6420 Rockledge Dr, Ste 4920, Bethesda, MD 20817 (mydlarz@jhmi.edu).

Published Online: March 15, 2018. doi:10.1001/jamaoto.2017.3446

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest, and none were reported.

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