Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature

Morgan L. Cowan, Lester D. Thompson, Marino E. Leon, Justin A. Bishop

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
JournalHead and Neck Pathology
DOIs
StateAccepted/In press - Aug 15 2015

Fingerprint

Sarcoma
Neck
Head
Catenins
S100 Proteins
Actins
Neoplasms
Surgical Pathology
Gene Fusion
Head and Neck Neoplasms
Larynx
Diagnostic Errors
Mandible
Spine
Differential Diagnosis
Coloring Agents
Extremities
Immunohistochemistry
Staining and Labeling
Neoplasm Metastasis

Keywords

  • Head and neck sarcoma
  • Low-grade fibromyxoid sarcoma
  • MUC4
  • p40
  • p63

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Oncology
  • Otorhinolaryngology

Cite this

Low-Grade Fibromyxoid Sarcoma of the Head and Neck : A Clinicopathologic Series and Review of the Literature. / Cowan, Morgan L.; Thompson, Lester D.; Leon, Marino E.; Bishop, Justin A.

In: Head and Neck Pathology, 15.08.2015.

Research output: Contribution to journalArticle

Cowan, Morgan L. ; Thompson, Lester D. ; Leon, Marino E. ; Bishop, Justin A. / Low-Grade Fibromyxoid Sarcoma of the Head and Neck : A Clinicopathologic Series and Review of the Literature. In: Head and Neck Pathology. 2015.
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abstract = "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.",
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