8/9/2019 Dermatofibrosarcoma Protuberans. M 62, Abdomen.

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Dermatofibrosarcoma protuberansDermatofibrosarcoma protuberans

Deba P Sarma, MDDeba P Sarma, MD

OmahaOmaha

8/9/2019 Dermatofibrosarcoma Protuberans. M 62, Abdomen.

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M 62, mid abdomen papuleM 62, mid abdomen papule

8/9/2019 Dermatofibrosarcoma Protuberans. M 62, Abdomen.

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8/9/2019 Dermatofibrosarcoma Protuberans. M 62, Abdomen.

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8/9/2019 Dermatofibrosarcoma Protuberans. M 62, Abdomen.

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CD 34: Positive

8/9/2019 Dermatofibrosarcoma Protuberans. M 62, Abdomen.

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DFSPDFSP

Dermal spindle cell neoplasm, strongly positive forDermal spindle cell neoplasm, strongly positive for

CD34CD34

..

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low grade,Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low grade,locally aggressive soft tissue tumor with high recurrence rate but rarelocally aggressive soft tissue tumor with high recurrence rate but raremetastasis.metastasis.

DFSP arises from the dermis and invades subcutaneous tissueDFSP arises from the dermis and invades subcutaneous tissue

Age: Adults, 20Age: Adults, 20--50 yrs.50 yrs. Location: Commonly trunk.Location: Commonly trunk.

Cellular origin of DFSP is not known, could be undifferentiatedCellular origin of DFSP is not known, could be undifferentiatedmesenchymal cells.mesenchymal cells.

Infiltrative spindle cell proliferation with a storiform nodular growth patternInfiltrative spindle cell proliferation with a storiform nodular growth patternoccupies the entire dermis and extends into the suncutaneous tissue andoccupies the entire dermis and extends into the suncutaneous tissue andthe fascial plane showing no defined border between the normal tissue andthe fascial plane showing no defined border between the normal tissue and

the tumor cells.the tumor cells.The tumor cells are strongly positive with CD 34 immunostain.The tumor cells are strongly positive with CD 34 immunostain.

A rare pigmented variant of DFSP, called Bednar tumor shows melaninA rare pigmented variant of DFSP, called Bednar tumor shows melanincontaining dendritic cells scattered between the neoplastic spindle cells.containing dendritic cells scattered between the neoplastic spindle cells.