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An Osseous Destructive Mass of the Infratemporal Fossa

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

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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Article Information

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 (marlan-hansen@uiowa.edu).

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.

References
1.
Parham  D. Skeletal-muscle tumours. In: Fletcher  CDM, Bridge  JA, Hogendoorn  P, Mertens  F, eds.  WHO Classifications of Tumours of Soft Tissue and Bone. 4th ed. Lyon, France: International Agency for Research on Cancer; 2013:130-132.
2.
Smith  MH, Atherton  D, Reith  JD, Islam  NM, Bhattacharyya  I, Cohen  DM.  Rhabdomyosarcoma, spindle cell/sclerosing variant: a clinical and histopathological examination of this rare variant with three new cases from the oral cavity.  Head Neck Pathol. 2017;11(4):494-500. doi:10.1007/s12105-017-0818-xPubMedGoogle ScholarCrossref
3.
Turner  JH, Richmon  JD.  Head and neck rhabdomyosarcoma: a critical analysis of population-based incidence and survival data.  Otolaryngol Head Neck Surg. 2011;145(6):967-973. doi:10.1177/0194599811417063PubMedGoogle ScholarCrossref
4.
Goto  TK, Yoshiura  K, Tanaka  T,  et al.  A follow-up of rhabdomyosarcoma of the infratemporal fossa region in adults based on the magnetic resonance imaging findings: case reports.  Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998;86(5):616-625. doi:10.1016/S1079-2104(98)90356-0PubMedGoogle ScholarCrossref
5.
Thiagarajan  A, Iyer  NG.  Radiation-induced sarcomas of the head and neck.  World J Clin Oncol. 2014;5(5):973-981. doi:10.5306/wjco.v5.i5.973PubMedGoogle ScholarCrossref
6.
Yeang  MS, Tay  K, Ong  WS,  et al.  Outcomes and prognostic factors of post-irradiation and de novo sarcomas of the head and neck: a histologically matched case-control study.  Ann Surg Oncol. 2013;20(9):3066-3075. doi:10.1245/s10434-013-2979-5PubMedGoogle ScholarCrossref
7.
Hansen  MR, Moffat  JC.  Osteosarcoma of the skull base after radiation therapy in a patient with McCune-Albright syndrome: case report.  Skull Base. 2003;13(2):79-83. doi:10.1055/s-2003-40597PubMedGoogle ScholarCrossref
8.
Henderson  TO, Whitton  J, Stovall  M,  et al.  Secondary sarcomas in childhood cancer survivors: a report from the Childhood Cancer Survivor Study.  J Natl Cancer Inst. 2007;99(4):300-308. doi:10.1093/jnci/djk052PubMedGoogle ScholarCrossref
9.
Shamim  T.  The spindle cell neoplasms of the oral cavity.  Iran J Pathol. 2015;10(3):175-184.PubMedGoogle Scholar
10.
Wu  Y, Li  C, Zhong  Y, Guo  W, Ren  G.  Head and neck rhabdomyosarcoma in adults.  J Craniofac Surg. 2014;25(3):922-925. doi:10.1097/SCS.0000000000000704PubMedGoogle ScholarCrossref
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