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WHO Classification of Tumours
Low grade fibromyxoid sarcoma
Connective, subcutaneous and other soft tissues


Definition

Low grade fibromyxoid sarcoma is a rare variant of fibrosarcoma that arises subfascially, typically in the proximal extremities or trunk, but also in unusual locations (e.g. head or retroperitoneum). The tumours are histologically characterized by a mixture of hypocellular, heavily collagenized areas and more cellular myxoid zones, bland spindled cells with a whorled growth pattern, and arcades of curvilinear blood vessels. In approximately 40% of cases, prominent collagen rosettes, consisting of a core of hyalinized collagen surrounded by epithelioid fibroblasts, are present focally
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Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Low grade fibromyxoid sarcomas mainly occur in young adults (median age 34 years). Recent investigations indicate lower rates of recurrence (9%), metastases (6%), and death (2%) than had been observed in earlier, retrospective, studies

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Folpe AL, Lane KL, Paull G, Weiss SW (2000)
Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas.
Am J Surg Pathol 24: 1353-60



. However, the median follow-up was only slightly over 4 years, and low grade fibromyxoid sarcomas may metastasize many years after initial diagnosis
Click for details
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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