Rare Lower Extremity Fibrosarcomatous Variant of Dermatofibrosarcoma With Myxoid Features Treated With Transtibial Amputation.

Cureus 2021 Vol.13(9) p. e18079

Pryor EK, Sinkler MA, Ullah A, Martin E, Homlar K

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Abstract

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor originating from the dermis, with high rates of local recurrence and invasive growth but low likelihood of distant metastasis. Fibrosarcomatous transformation (FS-DFSP) of DFSP accounts for approximately 5-15% of DFSP tumors, is a higher-grade tumor, with higher chances of metastasis and poorer prognosis. We present a case of a 66-year-old female presented with a large fungating mass on the left dorsal foot. Ultrasound-guided core needle biopsy with immunohistochemistry suggested a spindle cell neoplasm, favoring myxofibrosarcoma with intermediate grade. The patient elected for below-knee amputation over limb salvage with wide resection and free flap reconstruction. Based on clinical presentation, radiologic, histologic features and fluorescence in situ hybridization (FISH) studies confirmed the diagnosis of fibrosarcomatous variant of dermatofibrosarcoma protuberans with myxoid change. FS-DFSP with myxoid change is a rare soft tissue tumor that requires aggressive treatment due to its high rates of recurrence. This case presents a rare tumor in a unique location that was successfully treated with limb amputation, which is not documented in current literature.

추출된 의학 개체 (NER)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
시술 free flap 피판재건술 dict 1
해부 soft tissue tumor scispacy 1
해부 dermis scispacy 1
해부 limb scispacy 1
해부 flap scispacy 1
합병증 below-knee scispacy 1
질환 Dermatofibrosarcoma C0206647
Dermatofibrosarcoma
scispacy 1
질환 Dermatofibrosarcoma protuberans C0206647
Dermatofibrosarcoma
scispacy 1
질환 DFSP → Dermatofibrosarcoma protuberans C0206647
Dermatofibrosarcoma
scispacy 1
질환 tumor C0027651
Neoplasms
scispacy 1
질환 Fibrosarcomatous transformation scispacy 1
질환 FS-DFSP C3665732
Metastatic Dermatofibrosarcoma Protuberans
scispacy 1
질환 DFSP tumors scispacy 1
질환 neoplasm C0027651
Neoplasms
scispacy 1
질환 myxofibrosarcoma C3714524
Fibromyxosarcoma
scispacy 1
질환 below-knee amputation C0002692
Amputation of leg through tibia and fibula
scispacy 1
질환 Fibrosarcomatous scispacy 1
질환 higher-grade tumor scispacy 1
질환 fungating scispacy 1
질환 spindle cell neoplasm scispacy 1
기타 female scispacy 1
기타 left dorsal scispacy 1
기타 patient scispacy 1

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