Dermatofibrosarcoma Protuberans of the Vulva – A Case Report Jamil Omar, Fauziah Shari, Wan Shahrul Liza Saharan and Muralitharan Ganesalingam Department of Obstetric & Gyneacology , Hospital Ampang , Selangor Darul Ehsan , Malaysia. INTRODUCTIONS.
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DermatofibrosarcomaProtuberans of the Vulva – A Case Report
Jamil Omar, Fauziah Shari, Wan Shahrul Liza Saharan and MuralitharanGanesalingamDepartment of Obstetric & Gyneacology, Hospital Ampang, Selangor DarulEhsan, Malaysia
MRI pelvis showed there were two well – defined, solid, avascular, subcutabeous perineal masses with well – defined capsule measuring about 5 x 4cm on the right and 4 x 4cm on the left.She underwent excision of the vulval tumor. Both tumor enucleated out and sent for histopathology examination.
Radiation has occasionally been used as a primary therapeutic modality for DFSP, but it is more commonly used as adjuvant therapy after surgery.
Post – operative radiation therapy or imatinib mesylate should be considered for positive surgical margins if further resection is not feasible. If negative margin is achieved, no adjuvant treatment is necessary.
Dermatofibrosarcoma protuberans (DFSP) is rare malignant dermal neoplasm characterized by slow infiltrative growth, little metastatic potential but a high tendency to recur locally after surgery excision. The estimated incidence is 0.8 to 5 cases per 1 million persons per year. And its location on the vulva is uncommon.
Dermatofibrosarcoma protuberans of the vulva is uncommon. The aim of this case report is to highlight the importance of the surgical intervention in managing this disease.
DFSP is rarely metastasizes. The local recurrence rate ranges from 0 – 60%, whereas the rate of development of regional or distant metastatic disease is only 1% and 4 – 5%, respectively.
The initial treatment of DFSP is surgical. If initial surgery yields positive margins, re – resection is recommended whenever possible, with the gold of achieving clear margins.
Imatinib mesylate, a protein tyrosine kinase inhibitor, has shown clinical activity against localized and metastatic DFSP tumors containing t(17:22) and has been approved for the treatment of unresectable, recurrent and / or metastatic DFSP.
A 49 year old Indian woman presented with slow growing tumor on her vulva for 1 year. Upon examination, there were 2 masses: 1 immediately above the clitoris and another mass on the left which is more deep seated, difficult to differentiate from the surrounding tissue and appears continuous in the right upper region towards the superficial inguinal lymph node.
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Ohlinger R, Kuhl A, Schwesinger G, et al. Dermatofibrosarcomaprotuberans of the vulva. ActaObstetGynecol Scand 2004; 83: 685–6. [PubMed].
Leake JF, Buscema J, Cho KR, et al. Dermatofibrosarcomaprotuberans of the vulva. GynecolOncol 1991; 41: 245–9. [PubMed]
The histology study showed intradermal tumors extending and infiltrating the subcutaneous fat. They were cellular and arranged in storiform pattern composed of monomorphic spindle shaped cells with hyperchromatic nuclei. Both tumors expressed CD 34. The tumor is seen at its excision margin.
For the completion of the treatment of this patient, radiotherapy is required to irradicates the tumor and she will be under our regular and long term follow up.