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A 69-year-old man with a history of metastatic squamous cell carcinoma in the left parotid gland had undergone left parotidectomy and modified neck dissection followed by adjuvant radiation therapy 12 years previously. Ten years later, he developed a second primary tumor in the hypopharynx requiring total laryngectomy and bilateral neck dissection. He presented with new-onset headaches, left ear pain, and persistent unsteadiness during the previous month. There was no palpable lymphadenopathy, neurological deficits, or mastoid tenderness, and otoscopic examination results of the tympanic membrane appeared normal.
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D. Spindle cell rhabdomyosarcoma
Rhabdomyosarcoma is a rare, aggressive, soft-tissue malignant tumor originating from undifferentiated striated skeletal muscle. Spindle cell/sclerosing rhabdomyosarcoma (SCSR) is one of 4 subtypes of rhabdomyosarcoma (embryonal, alveolar, pleomorphic, and SCSR) classified by the World Health Organization since 2013.1 Because SCSR is a newly classified and rare subtype, there is limited literature addressing risk factors, treatments, and prognosis. Spindle cell/sclerosing rhabdomyosarcoma makes up 5% to 13% of all rhabdomyosarcoma cases and is more common in adults, unlike embryonal and alveolar rhabdomyosarcoma, which are seen predominantly in children.2
The head and neck is the most common site of rhabdomyosarcoma, with an incidence rate of fewer than 1 case per 2 million people per year, making up 35% of all rhabdomyosarcoma cases.3 Within the head and neck, rhabdomyosarcoma can be classified into orbital, parameningeal (ie, ear, mastoid, nasal cavity, paranasal sinuses, infratemporal fossa, pterygopalatine fossa), and nonorbital nonparameningeal (ie, palate, parotid, tongue, any other head and neck sites) types.3 Parameningeal tumors, as in this patient, have the worst 5-year relative survival rate (49.1%) of any type and are more likely to present with regional or distant metastases, especially in adults.3 Parameningeal rhabdomyosarcoma, specifically of the infratemporal fossa in adults, has limited literature owing to its rarity.4
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Corresponding Author: Marlan R. Hansen, MD, Department of Otolaryngology–Head and Neck Surgery, Carver College of Medicine, University of Iowa, 200 Hawkins Dr, Iowa City, IA 52242 (email@example.com).
Published Online: November 27, 2019. doi:10.1001/jamaoto.2019.3390
Conflict of Interest Disclosures: Dr Hansen reports receiving grants from the National Institutes of Health and grant UL1TR002537 from the National Center for Advancing Translational Sciences. No other disclosures were reported.
Additional Contributions: We thank the wife of the patient for granting permission to publish this information. We also thank Andrew M. Bellizzi, MD (Department of Pathology, University of Iowa, Iowa City), for preparing the final pathologic report for the patient’s specimen. He received no compensation for his contributions.
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