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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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C. Low-grade fibromyxoid sarcoma

Primary sarcomas of the head and neck are unusual, with a prevalence of 1% to 2%.1,2 Of these, low-grade fibromyxoid sarcomas (LGFMS) of the head and neck are exceedingly rare with only 26 cases described in literature.2 Low-grade fibromyxoid sarcomas often present in superficial subcutaneous tissue in children rather than the deep tissues of the proximal extremities commonly seen in young adults.3 Although multiple case series have described the indolent nature of LGFMS, there is a predilection for late recurrence after 15 years (median, 3.5 years) and pulmonary metastasis at a median of 5 years. Low-grade fibromyxoid sarcomas have a protracted clinic course with disease-specific survival of up to 70 years.4

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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.

References
1.
Peng  KA , Grogan  T , Wang  MB .  Head and neck sarcomas: analysis of the SEER database.   Otolaryngol–Head Neck Surg. 2014;151(4):627-633. doi:10.1177/0194599814545747Google ScholarCrossref
2.
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
3.
Sargar  K , Kao  SC , Spunt  SL ,  et al.  MRI and CT of low-grade fibromyxoid sarcoma in children: a report from children’s oncology group study ARST0332.   AJR Am J Roentgenol. 2015;205(2):414-420. doi:10.2214/AJR.14.13972PubMedGoogle ScholarCrossref
4.
Evans  HL .  Low-grade fibromyxoid sarcoma: a clinicopathologic study of 33 cases with long-term follow-up.   Am J Surg Pathol. 2011;35(10):1450-1462. doi:10.1097/PAS.0b013e31822b3687PubMedGoogle ScholarCrossref
5.
Doyle  LA , Möller  E , Dal Cin  P , Fletcher  CDM , Mertens  F , Hornick  JL .  MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma.   Am J Surg Pathol. 2011;35(5):733-741. doi:10.1097/PAS.0b013e318210c268PubMedGoogle ScholarCrossref
6.
Panagopoulos  I , Storlazzi  CT , Fletcher  CDM ,  et al.  The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma.   Genes Chromosomes Cancer. 2004;40(3):218-228. doi:10.1002/gcc.20037PubMedGoogle ScholarCrossref
7.
Chamberlain  F , Engelmann  B , Al-Muderis  O ,  et al.  Low-grade fibromyxoid sarcoma: treatment outcomes and efficacy of chemotherapy.   In Vivo. 2020;34(1):239-245. doi:10.21873/invivo.11766Google ScholarCrossref
8.
Sangkhathat  S .  Current management of pediatric soft tissue sarcomas.   World J Clin Pediatr. 2015;4(4):94-105. doi:10.5409/wjcp.v4.i4.94PubMedGoogle ScholarCrossref
9.
Maretty-Nielsen  K , Baerentzen  S , Keller  J , Dyrop  HB , Safwat  A .  Low-grade fibromyxoid sarcoma: incidence, treatment strategy of metastases, and clinical significance of the FUS gene.   Sarcoma. 2013;2013:256280. doi:10.1155/2013/256280PubMedGoogle Scholar
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