Low-grade Fibromyxoid Sarcoma Arising in the Big Toe

Znati Kaoutar, MD; Amal Benlemlih, MD; Harmouch Taoufiq, MD; Chbani Laila, MD; ElFatemi Hinde, MD; Sanae Bennis, PhD; Amarti Afaf, MD


South Med J. 2011;104(3):241-243. 

In This Article

Abstract and Introduction


Low-grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm commonly affecting young adults and typically arising in the somatic soft tissue of the proximal extremities. Its occurrence within the big toe is exceedingly rare. A 43-year-old man had surgery on a mass located in the big toe, which was first noted 6 months previously. Histological examination revealed LGFMS. One year after surgery, the patient is alive with no evidence of disease. Low-grade fibromyxoid sarcoma is a rare neoplasm that should be considered in the differential diagnosis of spindle cell neoplasms of the foot.


Low-grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm that occurs primarily in young male adults. Although LGFMS has a deceptively benign histologic appearance, local recurrence and late metastases have frequently been reported. Diagnosis of LGFMS is still difficult because of its characteristic bland-looking histologic features that can be confused with other benign or low-grade fibromyxoid lesions.[1–4] It was recently recognized that most cases of LGFMS exhibit the FUS/CREB3L2 fusion gene, and that rare cases may display a variant FUS/CREB3L1 fusion gene.[5–7]

Low-grade fibromyxoid sarcoma typically affects deep soft tissues including the thigh, inguinal area, shoulder, axilla, perineum, neck, and buttock.[1] It is rarely seen in the distal extremities, especially in fingers or toes. This paper reports a case of LGFMS arising in the big toe, and discusses the histological features and differential diagnosis.


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