Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature.
Low-grade fibromyxoid sarcoma (LGFMS) is a deceptively bland malignancy with potential for late recurrence and metastasis, which usually occurs in the deep soft tissues of the extremities and trunk. Most LGFMSs harbor a characteristic gene fusion of FUS-CREB3L2, and recently MUC4 immunostaining has been found to be highly sensitive and specific for the diagnosis. We present a dedicated series of head and neck LGFMS, including the first reported laryngeal case, as well as a review of reported head and neck cases. The surgical pathology archives of our three institutions were searched for cases of LGFMS arising within the head and neck, and four cases were identified. The H&E slides were reviewed, and immunohistochemistry were performed for pancytokeratin, p63, p40, EMA, S100 protein, β-catenin, actin, CD34, and MUC4. The patients were 6, 43, 45, and 73 years old (mean 41.8 years) and included three males and one female. The tumors were located in the posterior cervical spine, facial skin, mandible, and larynx. The tumors were treated with surgical excision, and all four had histologic features typical for LGFMS including alternating myxoid and fibrous areas with prominent curvilinear vasculature. All tumors were MUC4 positive (100%), 2/4 (50%) were p63 positive, 1/4 (25%) showed focal EMA positivity; all 4 were negative for pancytokeratin, p40, S100 protein, β-catenin, actin, and CD34. LGFMS is a low grade sarcoma that rarely develops in the head and neck. Due to its rarity, a pathologist may not consider LGFMS in the differential diagnosis of spindle cell neoplasms within the head and neck. Immunohistochemical staining is helpful, but stains should be selected carefully to avoid misdiagnosis.
Department of Pathology, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Pathology 401, Baltimore, MD, 21287, USA. mcowan6@jhmi.edu.
Morgan L
http://orcid.org/0000-0003-1568-193X
ML
Cowan
Department of Pathology, Southern California Permanente Medical Group, Woodland Hills, CA, USA.
Lester D
LD
Thompson
Department of Anatomic Pathology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.
Marino E
ME
Leon
Department of Pathology, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Pathology 401, Baltimore, MD, 21287, USA.|Department of Otolaryngology-Head and Neck Surgery, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Pathology 401, Baltimore, MD, 21287, USA.
Justin A
JA
Bishop
D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D005260:Female; D005354:Fibrosarcoma / Q000473:pathology*; D006258:Head and Neck Neoplasms / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading
D014408:Biomarkers, Tumor
Head and neck sarcoma; Low-grade fibromyxoid sarcoma; MUC4; p40; p63