Case for diagnosis

Franco, Joanna Pimenta de Araujo; Barbosa, Caroline Cruz; Fonseca, Bárbara Ferreira Saraiva da; Lima, Ricardo Barbosa; D'Ácri, Antônio Macedo; Martins, Carlos José

doi:10.1590/abd1806-4841.20142696

An. Bras. Dermatol.

2014

DOI: 10.1590/abd1806-4841.20142696

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Case for diagnosis

Joanna Pimenta de Araujo Franco

Caroline Cruz Barbosa

Bárbara Ferreira Saraiva da Fonseca

et al.

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. W… Show more

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Dermatofibrosarcoma Protuberans of Distal Extremities and Acral Sites

Shah¹,

McHugh²,

Folpe³

et al. 2018

American Journal of Surgical Pathology

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Dermatofibrosarcoma protuberans (DFSP) of the distal extremities and acral sites are extremely rare and incompletely characterized. Twenty-seven DFSP occurring in these sites were retrieved from our collective archives and reevaluated. Tumors occurred in 16 males and 11 females. Median age at presentation was 42.5 years (range, 7 to 78 y). Lesions involved the foot (18 with 6 in the toes and 2 on the plantar foot), distal ankle (4), hand (4 with 2 in the thumbs), and wrist (1). All cases showed predominantly classic DFSP morphology and were diffusely CD34 positive. Myxoid change, melanin pigmented, and giant cell fibroblastoma foci were each present in 1 case, respectively. Fibrosarcomatous change was present in 3 cases. Fluorescent in situ hybridization demonstrated PDGFB gene rearrangement in 9 of 10 tested cases. Clinical follow-up was available in 21 cases (median, 36.1 mo; range, 1 to 152 mo) and revealed 4 local recurrences. Four patients underwent digital amputation for unresectable recurrent disease. An additional patient underwent multiple resections with positive margins and elected to receive imatinib mesylate therapy. After a 2-year course, the patient has no evidence of residual disease (40 mo). No metastases were documented in any of the cases studied. The natural history of DFSP of distal extremities and acral sites is similar to that of its counterparts elsewhere. A high index of suspicion, careful morphologic examination for key histologic features of DFSP, and in selected cases, molecular studies to identify the pathognomonic COL1A1-PDGFB gene fusion should facilitate the distinction of these rare, locally aggressive neoplasms from morphologic mimics that may arise in distal extremities and acral sites.

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Dermatofibrosarcoma Protuberans of Distal Extremities and Acral Sites

Shah¹,

McHugh²,

Folpe³

et al. 2018

American Journal of Surgical Pathology

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Recurrent Dermatofibrosarcoma Protuberance: A Case Report

et al. 2021

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Dermatofibrosarcoma protuberance represents less than 0.1% of all tumors, treatment of which requires wide local excision (≥5cm) but recurrence is not rare. Here we present a 32-year male presented with a swelling of 15 x 6cm over the left lumbar region for which he underwent excision three years ago, the histopathological examination of the swelling, showed a malignant mesenchymal tumor and Immunohistochemistry features were suggestive of Dermatofibrosarcoma protuberance. After three years of interval, he again presented with complaints of swelling in the previously operated site for nine months and underwent excision of the mass with Split Thickness Skin Graft. Although the tumor was confined to the skin and subcutaneous tissue in the present case, the patient didn’t undergo any adjuvant radiotherapy to avoid a possible relapse that would infiltrate deeper structures for the first time. Being a recurrent tumor, long-term follow-up is strongly recommended.

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Case for diagnosis

Cited by 2 publications

References 2 publications

Dermatofibrosarcoma Protuberans of Distal Extremities and Acral Sites

Dermatofibrosarcoma Protuberans of Distal Extremities and Acral Sites

Recurrent Dermatofibrosarcoma Protuberance: A Case Report

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