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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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Irregular, nodular, indurate lesion with erythematous surface withteleangiectasias. A) Overview B) Lesion detail
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f01: Irregular, nodular, indurate lesion with erythematous surface withteleangiectasias. A) Overview B) Lesion detail

Mentions: Physical examination revealed an irregular and indurated nodular lesion, measuring 5centimeters, with an erythematous surface with telangiectasias, located in the dorsalaspect of the proximal phalanx of the fourth left finger extending to themetacarpophalangeal joint (Figure 1).


Case for diagnosis. Dermatofibrosarcoma protuberans.

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

Irregular, nodular, indurate lesion with erythematous surface withteleangiectasias. A) Overview B) Lesion detail
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f01: Irregular, nodular, indurate lesion with erythematous surface withteleangiectasias. A) Overview B) Lesion detail
Mentions: Physical examination revealed an irregular and indurated nodular lesion, measuring 5centimeters, with an erythematous surface with telangiectasias, located in the dorsalaspect of the proximal phalanx of the fourth left finger extending to themetacarpophalangeal joint (Figure 1).

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