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2008, Volume 24, Number 1, Page(s) 044-049     
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Acral myxoinflammatory fibrablastic sarcoma: Report of six cases
Esen Gül UZUNER1, Misten DEMİRYONT1, Bilge BİLGİÇ1, Harzem ÖZGER2
1İstanbul Üniversitesi, İstanbul Tıp Fakültesi, Patoloji Anabilim Dalı, İSTANBUL
2İstanbul Üniversitesi, İstanbul Tıp Fakültesi, Ortopedi ve Travmatoloji Anabilim Dalı, İSTANBUL
Keywords: Acral myxoinflammatory fibroblastic sarcoma, distal extremity, low-grade sarcoma

Acral myxoinflammatory fibroblastic sarcoma was first described in 1998. The tumor typically involves distal extremities; especially fingers and toes. It is usually located in subcutaneous tissue. The age range is broad (mean age 40). The size of tumor varies between 1-8 cm. Microscopically, myxoid and hyaline zones are characteristic. Inflammatory cells are abundant and there are large cells resembling Reed-Sternberg or ganglion cells and multivacuolated cells having lipoblast-like appearance.

We present 6 cases of acral myxoinflammatory fibroblastic sarcoma, locations were ankle, 1st toe and hand. The most presenting symptoms were swelling with or without pain and they have lasted for 3,5 months-4 years.

Immunohistochemical studies of our cases, revealed vimentin (5/5), CD68 (5/5), CD34 (1/3) positivity. Ki-67 proliferation indices varied between 2-10%. Five patients underwent tumor excision, while in one patient amputation had been performed.

This tumor is a low grade sarcoma but has the potential of recurrence and even metastasis, though it is rare. In one of our cases, recurrence was observed; in none of our cases metastasis has been determined.

It included many tumoral and non-tumoral lesions in differential diagnosis.


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