IMR Press / EJGO / Volume 31 / Issue 6 / pii/1630990943898-1204947689

European Journal of Gynaecological Oncology (EJGO) is published by IMR Press from Volume 40 Issue 1 (2019). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with S.O.G.

Open Access Case Report
Dermatofibrosarcoma protuberans with areas of giant cell fibroblastoma in the vulva: a case report
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1 Department of Pathology, Hospital Universitario Ramón y Cajal, Madrid
2 Department of Gynecology, Hospital Universitario Ramón y Cajal, Madrid
3 Department of Dermatology, Hospital Universitario Ramón y Cajal, Madrid (Spain)
Eur. J. Gynaecol. Oncol. 2010, 31(6), 685–689;
Published: 10 December 2010
Abstract

Objective: To review the clinical, morphologic, immunohistochemical, and histogenetic characteristics of dermatofibrosarcoma protuberans with areas of giant cell fibroblastoma and explore current treatment options. Methods: We describe the case of a 38- year-old patient with a tumor measuring 5.7 cm on the right labium majus of the vulva. Serial sections stained with hematoxylineosin were examined and immunohistochemical staining was performed for CD34 and PDGF receptor alpha and beta (PDFGRA and PDGFRB). Results: The histologic study showed spindle-cell proliferation typical of dermatofibrosarcoma protuberans and other areas containing fibrosis and giant cells lining pseudovascular spaces. Both tumor areas expressed CD34, PDGFRA, and PDGFRB. Conclusions: Only two cases of dermatofibrosarcoma protuberans with areas of giant cell fibroblastoma in the vulva have been reported to date. Both dermatofibrosarcoma protuberans and giant cell fibroblastoma are characterized by the translocation t (17;22) (q22;q13). The fact that PDGFRA and PDGFRB are overexpressed in these tumors opens new treatment options with imatinib. Surgical excision with wide margins or Mohs micrographic surgery continues to be the treatment of choice.
Keywords
Dermatofibrosarcoma protuberans
Giant cell fibroblastoma
Imatinib
COL1A1-PDGFB
Mohs micrographic surgery
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