<abstract xmlns="http://www.w3.org/1999/xhtml"><p> Dermatofibrosarcoma protuberans (DFSP) represents a low-grade cutaneous sarcoma which may have different histological aspects, presenting as a fibrosarcomatous, pigmented, juvenile, myxoid, atrophic, sclerosing or myoid lesion. Some of these subtypes may occur isolated or in association with one of the others creating hybrid lesions. We present the case of a 66 years old woman having a 4 cm diameter tumor located on the abdominal wall. Histopathological examination of the resection specimen revealed areas of typical DFSP associated with fibrosarcomatous transformation, myoid and myxoid areas. Also, focally, pleomorphic tumor cells and foreign-body type multinucleated giant cells were observed. Immunostains revealed CD34 positivity in typical DFSP and myxoid areas with negative staining of some of the tumor cells in fibrosarcomatous areas and negative staining of myoid areas. Smooth muscle actin was positive in myoid areas. The nature of myoid fascicles in DFSP is a matter of debate, being uncertain whether these represent a type of tumor differentiation or a reactive myoid proliferation. In this particular case, finding the association of myoid cells with blood vessel walls sustains their reactive nature. We present the morphological aspects of the different areas of the tumor with emphasis on differential diagnostic problems and clinical implications.</p></abstract>

Dermatofibrosarcoma protuberans (DFSP) represents a low-grade cutaneous sarcoma which may have different histological aspects, presenting as a fibrosarcomatous, pigmented, juvenile, myxoid, atrophic, sclerosing or myoid lesion. Some of these subtypes may occur isolated or in association with one of the others creating hybrid lesions. We present the case of a 66 years old woman having a 4 cm diameter tumor located on the abdominal wall. Histopathological examination of the resection specimen revealed areas of typical DFSP associated with fibrosarcomatous transformation, myoid and myxoid areas. Also, focally, pleomorphic tumor cells and foreign-body type multinucleated giant cells were observed. Immunostains revealed CD34 positivity in typical DFSP and myxoid areas with negative staining of some of the tumor cells in fibrosarcomatous areas and negative staining of myoid areas. Smooth muscle actin was positive in myoid areas. The nature of myoid fascicles in DFSP is a matter of debate, being uncertain whether these represent a type of tumor differentiation or a reactive myoid proliferation. In this particular case, finding the association of myoid cells with blood vessel walls sustains their reactive nature. We present the morphological aspects of the different areas of the tumor with emphasis on differential diagnostic problems and clinical implications.

Multiple Histological Subtypes of Dermatofibrosarcoma Protuberans Occurring in the Same Tumor

“Colentina” University Hospital, Department of Pathology, Bucharest, Romania

University of Medicine and Pharmacy, Department of Pathology, Bucharest, Romania

Romanian Journal of Internal Medicine

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Dermatofibrosarcoma protuberans (DFSP) represents a low-grade cutaneous sarcoma which may have different histological aspects, presenting as a...

Multiple Histological Subtypes of Dermatofibrosarcoma Protuberans Occurring in the Same Tumor

Published Online: Oct 07, 2015

Page range: 81 - 90

Received: Feb 10, 2015

DOI: https://doi.org/10.1515/rjim-2015-0011

Keywords
Dermatofibrosarcoma protuberans, fibrosarcomatous, juvenile, giant-cell fibroblastoma, myxoid, myoid

© 2015

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Multiple Histological Subtypes of Dermatofibrosarcoma Protuberans Occurring in the Same Tumor

Published Online: Oct 07, 2015

Page range: 81 - 90

Received: Feb 10, 2015

DOI: https://doi.org/10.1515/rjim-2015-0011

KeywordsDermatofibrosarcoma protuberans, fibrosarcomatous, juvenile, giant-cell fibroblastoma, myxoid, myoid

© 2015

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Keywords
Dermatofibrosarcoma protuberans, fibrosarcomatous, juvenile, giant-cell fibroblastoma, myxoid, myoid