Success treatment of adult primary vulvar Langerhans cell histiocytosis with thalidomide

Due to rarity of primary vulvar Langerhans cell histiocytosis (LCH) and lack of randomized clinical trials, treatment of genital LCH is still non-standardized. We describe a case in which the LCH was confined to the vulva, with review of the literature. The review showed different response and recurrence rate of each therapy modality. First line treatment of isolated vulvar LCH included surgery, radiotherapy, chemotherapy and topical therapy. Overall outcome was good but the risk of recurrence was high (up to 42%–50%). Mean time to relapse was 18.3±12.4 months. In our case, a 59-year-old woman presented with pruritus and painful ulcerative lesions on her vulva. The biopsy was consistent with LCH. A metastatic work-up did not reveal any metastasis. The treatment was vulvectomy followed by radiotherapy. The patient was diagnosed with a local recurrent 3 years after the completion of primary therapy. For this reason, she underwent second line treatment with radical vulvectomy and followed by chemotherapy (methotrexate 10mg weekly). Fifteen months later, the patient had signs of a local recurrence. Because of co-morbidity and high risk of surgical treatment after radical vulvectomy, the patient received low-dose thalidomide (lenalidomide 10mg once daily for 21 days in a 28-day cycle). The pain became less after the first cycle. A complete remission was achieved within 6 months treatment. The patient reported minimal side effect of lenalidomide. In conclusion, isolated vulvar LCH should be treated with local therapy. In case of relapse, systemic therapy such as thalidomide or chemotherapy should be considered as maintenance treatment in appropriately selected patients in order to prolong the disease free interval.

vulvar Langerhans cell histiocytosis - vulvar malignancy - vulvar ulcers