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Neck Mass in an Adolescent

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

A 13-year-old male presented to the pediatric otolaryngology clinic with a 2-year history of a right neck mass that had slowly increased in size. He denied any associated symptoms of pain, fevers, chills, malaise, night sweats, unintentional weight loss, prior history of neck masses, recent upper respiratory tract infections, or skin lesions. He was the product of a full-term pregnancy with up-to-date immunizations. Physical examination revealed a 2.5-cm firm, ovoid, mobile, and nontender mass at the apex of the posterior triangle of the right neck without any associated overlying skin changes. The remainder of the head and neck examination was unremarkable. Doppler ultrasonography revealed a 1.9 × 1.8 × 0.9-cm hypoechoic mass, and subsequent fine-needle aspirates demonstrated cells with elongated nuclei and eccentric blue cytoplasm in a background of myxoid stroma. The mass was excised in entirety without issue. At the time of surgery, the deep surface of the mass was found to be adherent to the sternocleidomastoid muscle. The lesion was resected with a cuff of muscle and sent for permanent histopathological examination. This revealed proliferation of bland spindle cells with plump nuclei and eosinophilic cytoplasm arranged as loose fascicles in a background of myxoid stroma (Figure 1). These cells were positive for mucin 4 (MUC4), epithelial membrane antigen (EMA), and transducing-like enhancer of split 1 (TLE1) immunohistochemical stains. Fluorescence in situ hybridization (FISH) revealed a positive FUS (16p11) gene rearrangement.

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A 13-year-old male presented to the pediatric otolaryngology clinic with a 2-year history of a right neck mass that had slowly increased in size. He denied any associated symptoms of pain, fevers, chills, malaise, night sweats, unintentional weight loss, prior history of neck masses, recent upper respiratory tract infections, or skin lesions. He was the product of a full-term pregnancy with up-to-date immunizations. Physical examination revealed a 2.5-cm firm, ovoid, mobile, and nontender mass at the apex of the posterior triangle of the right neck without any associated overlying skin changes. The remainder of the head and neck examination was unremarkable. Doppler ultrasonography revealed a 1.9 × 1.8 × 0.9-cm hypoechoic mass, and subsequent fine-needle aspirates demonstrated cells with elongated nuclei and eccentric blue cytoplasm in a background of myxoid stroma. The mass was excised in entirety without issue. At the time of surgery, the deep surface of the mass was found to be adherent to the sternocleidomastoid muscle. The lesion was resected with a cuff of muscle and sent for permanent histopathological examination. This revealed proliferation of bland spindle cells with plump nuclei and eosinophilic cytoplasm arranged as loose fascicles in a background of myxoid stroma (Figure 1). These cells were positive for mucin 4 (MUC4), epithelial membrane antigen (EMA), and transducing-like enhancer of split 1 (TLE1) immunohistochemical stains. Fluorescence in situ hybridization (FISH) revealed a positive FUS (16p11) gene rearrangement.

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Article Information

Corresponding Author: Saikrishna C. Gourishetti, MD, Department of Otorhinolaryngology–Head and Neck Surgery, University of Maryland School of Medicine, 16 S Eutaw St, Ste 500, Baltimore, MD 21201 (sgourishetti@som.umaryland.edu).

Published Online: September 9, 2021. doi:10.1001/jamaoto.2021.2314

Conflict of Interest Disclosures: No disclosures.

Additional Contributions: We thank the patient’s guardian for granting permission to publish this information.

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Cowan  ML , Thompson  LD , Leon  ME , Bishop  JA .  Low-grade fibromyxoid sarcoma of the head and neck: a clinicopathologic series and review of the literature.   Head Neck Pathol. 2016;10(2):161-166. doi:10.1007/s12105-015-0647-8PubMedGoogle ScholarCrossref
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AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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