A Clinicopathologic Review of a Case Series of Dermatofibrosarcoma Protuberans with Fibrosarcomatous Differentiation

Kuzel, Paul; Mahmood, Muhammad Nateque; Metelitsa, Andrei I.; Salopek, Thomas G.

doi:10.2310/7750.2014.13192

Cited by 17 publications

(9 citation statements)

References 23 publications

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“…Fine-needle aspiration cytology has low diagnostic accuracy for mesenchymal breast tumor. Core biopsy is considered the optimal procedure for diagnosing such tumors, though adjunctive immunohistochemical analysis is often required (18–21). However, a diagnosis from a core biopsy is often difficult.…”

Section: Discussionmentioning

confidence: 99%

“…It was concluded that primary or recurrent treatment of DFSP should be excision with negative margins, which should result in low recurrence rates and infrequent metastatic spread. Multimodality treatment, particularly the use of tyrosine kinase inhibitors, could be effective, but should not be considered as curative (5,18,21,24–29). DFSP follow-ups subsequent to surgery are recommended for a minimum of 3 years, in 6-month intervals (18).…”

Section: Discussionmentioning

confidence: 99%

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Dermatofibrosarcoma protuberans of the breast: A case report

et al. 2017

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Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumor of subcutaneous tissue characterized by slow infiltrative growth. The tumor occurs in patients of all ages, with the highest frequency occurring between the second and the fifth decades of age. Genetically, DFSP is characterized by a reciprocal translocation t(17;22)(q22;q13), or more often, as a supernumerary ring chromosome involving chromosomes 17 and 22. Standard treatment of a localized tumor is surgical excision with wide margins. In the present study, a case report of a 43-year-old woman with a growing tumor in the left breast is discussed. The patient underwent breast-conserving surgery. Histological and cytogenetic examinations of the tumor resulted in a diagnosis of DFSP. The clinical and morphological characteristics of the tumor, in addition to the treatment options, were also evaluated.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Dermatofibrosarcoma protuberans of the breast: A case report

et al. 2017

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“…Fibrosarcomatous components are indistinguishable from a highgrade fibrosarcoma. This can be compared to the uniform, low-grade DFSP characterized by a storiform or cartwheel pattern [13]. The myxoid changes seen in our patient are rare in classic DFSP but seem to be more commonly found in FS-DFSP [4].…”

Section: Discussionmentioning

confidence: 52%

“…These tumors may be local or found invading local tissues, including subcutaneous tissue and skeletal muscle. While FS-DFSPs show considerable variability in presentation, consistently they demonstrate an association with higher rate of metastasis (10-15% vs. less than 1%) and poorer prognosis compared to low-grade DFSP [4,5,13,14].…”

Section: Discussionmentioning

confidence: 99%

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

Pryor

Sinkler

Ullah

et al. 2021

Cureus

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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 66year-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.

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Fibrous, Fibrohistiocytic, and Myofibroblastic Tumors of Intermediate Malignancy

Singh

Udager

Billings

2018

Soft Tissue Tumors of the Skin

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A Clinicopathologic Review of a Case Series of Dermatofibrosarcoma Protuberans with Fibrosarcomatous Differentiation

Abstract: DFSP-FS represents a more aggressive subtype of DFSP. Several features of DFSP-FS may impart a higher risk of metastasis.

Cited by 17 publications

References 23 publications

Dermatofibrosarcoma protuberans of the breast: A case report

Dermatofibrosarcoma protuberans of the breast: A case report

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

Fibrous, Fibrohistiocytic, and Myofibroblastic Tumors of Intermediate Malignancy

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