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Case for diagnosis. Dermatofibrosarcoma protuberans.

Franco JP, Barbosa CC, Fonseca BF, Lima RB, D'Acri AM, Martins CJ - An Bras Dermatol (2014 Mar-Apr)

Bottom Line: Histopathology reveals spindle cells with storiform pattern and cartwheel-like or whirlwind-like aspect.Immunohistochemistry shows positivity for CD34.The treatment is surgical.

View Article: PubMed Central - PubMed

Affiliation: State of Rio de Janeiro Federal University, Rio de Janeiro, RJ, Brazil.

ABSTRACT
Dermatofibrosarcoma protuberans is a fibrohistiocytic tumor of intermediate malignancy with aggressive localized growth, high recurrence rate, but low metastatic potential. It appears as a hardened plaque, with slow growth, upon which the development of nodules occurs. It predominates in the trunk and is unusual in acral locations. Histopathology reveals spindle cells with storiform pattern and cartwheel-like or whirlwind-like aspect. Immunohistochemistry shows positivity for CD34. The treatment is surgical. We report a case of long evolution, with an unusual location, that relapsed after surgery, to emphasize the importance of early diagnosis and proper treatment, avoiding aggressive resections with increased morbidity.

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Post-operatory image showing amputation of the fourth left finger
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f03: Post-operatory image showing amputation of the fourth left finger

Mentions: A new biopsy was performed and histopathology demonstrated proliferation of spindlecells with storiform pattern and areas with "cartwheel" aspect (Figure 2A). Immunohistochemistry showed positivity for CD34 (Figure 2B). Radiography revealed increase in softtissue volume without bone involvement and ultrasonography showed a hypoechoic solidimage. Upon diagnosis, the patient underwent surgical treatment, as described below inthe discussion section (Figure 3).


Case for diagnosis. Dermatofibrosarcoma protuberans.

Franco JP, Barbosa CC, Fonseca BF, Lima RB, D'Acri AM, Martins CJ - An Bras Dermatol (2014 Mar-Apr)

Post-operatory image showing amputation of the fourth left finger
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4008077&req=5

f03: Post-operatory image showing amputation of the fourth left finger
Mentions: A new biopsy was performed and histopathology demonstrated proliferation of spindlecells with storiform pattern and areas with "cartwheel" aspect (Figure 2A). Immunohistochemistry showed positivity for CD34 (Figure 2B). Radiography revealed increase in softtissue volume without bone involvement and ultrasonography showed a hypoechoic solidimage. Upon diagnosis, the patient underwent surgical treatment, as described below inthe discussion section (Figure 3).

Bottom Line: Histopathology reveals spindle cells with storiform pattern and cartwheel-like or whirlwind-like aspect.Immunohistochemistry shows positivity for CD34.The treatment is surgical.

View Article: PubMed Central - PubMed

Affiliation: State of Rio de Janeiro Federal University, Rio de Janeiro, RJ, Brazil.

ABSTRACT
Dermatofibrosarcoma protuberans is a fibrohistiocytic tumor of intermediate malignancy with aggressive localized growth, high recurrence rate, but low metastatic potential. It appears as a hardened plaque, with slow growth, upon which the development of nodules occurs. It predominates in the trunk and is unusual in acral locations. Histopathology reveals spindle cells with storiform pattern and cartwheel-like or whirlwind-like aspect. Immunohistochemistry shows positivity for CD34. The treatment is surgical. We report a case of long evolution, with an unusual location, that relapsed after surgery, to emphasize the importance of early diagnosis and proper treatment, avoiding aggressive resections with increased morbidity.

Show MeSH
Related in: MedlinePlus