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Low-grade myofibroblastic sarcoma of the distal femur.

AbstractINTRODUCTION:
Low-grade myofibroblastic sarcoma (myofibrosarcoma) is described to be a distinct atypical myofibroblastic tumor often with fibromatosis-like features and predilection for head and neck. Low-grade myofibroblastic sarcoma of bone is extremely rare.
PRESENTATION OF CASE:
A 50-year-old woman was admitted to our hospital because she had experienced right knee pain for 2 years. Plain radiography showed a honeycombed lesion on the right distal femur, and computed tomography showed a bone tumor with cortex destruction invading the soft tissue. A biopsy specimen from the intraosseous lesion showed a hypocellular area of spindle cell proliferation with dense collagen deposition, which is reminiscent of a histological feature of desmoplastic fibroma. However, histological examination of the extraosseous lesion indicated a slightly hypercellular area containing scattered spindle-shaped atypical cells with enlarged nuclei, suggestive of low-grade sarcoma. Spindle-shaped atypical cells were immunohistochemically positive for SMA. A final diagnosis of low-grade myofibroiblastic sarcoma of the bone was made from a surgically resected specimen.
DISCUSSION:
The patient was alive and well with no evidence of disease at 15 months after the surgery without any additional therapy.
CONCLUSION:
Extensive sampling of a tumor may be necessary to determine the true nature of the tumor and to make an accurate diagnosis.
AuthorsTsuyoshi Saito, Hiroyuki Mitomi, Aiko Kurisaki, Tomoaki Torigoe, Tatsuya Takagi, Yoshiyuki Suehara, Taketo Okubo, Kazuo Kaneko, Takashi Yao
JournalInternational journal of surgery case reports (Int J Surg Case Rep) Vol. 4 Issue 2 Pg. 195-9 ( 2013) ISSN: 2210-2612 [Print] Netherlands
PMID23276766 (Publication Type: Journal Article)
CopyrightCopyright © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.

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