Abstract
Primary malignant melanoma of vagina is a rare variant of melanoma and usually associated with a grave prognosis. Radical surgery is the only treatment option with reasonable loco regional control. A case of primary malignant melanoma involving whole of vagina infiltrating urethra and reaching up to vulva was treated by surgery and postoperative radiotherapy. The tumor was infiltrating bladder and rectum reaching the anal sphincter. Total pelvic exenteration was done to achieve tumor-free surgical margins. One year after treatment, patient is disease free.
Electronic supplementary material
The online version of this article (doi:10.1007/s12262-015-1349-x) contains supplementary material, which is available to authorized users.
Keywords: Malignant melanoma vagina, Total pelvic exenteration, Vaginal cancers, Mucosal melanoma, Radical surgery, Primary vaginal melanoma
Background
Primary vaginal melanoma is a rare and highly malignant disease. It constitutes less than 0.3 % of all melanomas and less than 3 % of all malignant vaginal tumours. It presents usually in late stages and the prognosis is very poor, 5 year survival rates ranging from 5 to 25 %. Surgery is advocated as the curative treatment with procedures ranging from local excision to radical extirpation of vagina.
Case Report
A case of primary malignant melanoma of vagina involving whole of vagina infiltrating urethra and reaching vulva was treated by total pelvic exenteration and postoperative radiotherapy. The tumor was infiltrating bladder, urethra and rectum up to the anal sphincter. Total exenteration with radical vulvectomy was done to achieve tumor-free surgical margins. Surgical reconstruction was done by wet colostomy. Patient received adjuvant pelvic radiation. One year after treatment, patient is disease free.
Discussion
Vaginal melanoma is associated with poor clinical outcome for majority of patients. Because of the rich lymphatic and vascular supply of the vagina, both local and distant spread is common in this biologically aggressive malignancy. Although the majority of patients with vaginal melanoma will die of their disease, surgical therapy remains the only chance for long-term survival and is associated with increased overall survival. The goal of operative intervention should be complete resection of gross disease. Considering the rarity of the disease, there are no prospective studies or validated recommendations in treating vaginal melanomas.
Electronic supplementary material
Below is the link to the electronic supplementary material.
Total exenteration specimen showing uterus with urinary bladder anterior to it. Tumour is seen extending from vagina to vulva and infiltrating urethra (JPEG 121 kb)
Preoperative picture of patient with malignant melanoma vagina. The tumour is seen protruding through vagina infiltrating urethra and reaching up to rectum (JPEG 76 kb)
Acknowledgments
Conflict of interest
The authors declare that they have no competing interests.
Associated Data
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Supplementary Materials
Total exenteration specimen showing uterus with urinary bladder anterior to it. Tumour is seen extending from vagina to vulva and infiltrating urethra (JPEG 121 kb)
Preoperative picture of patient with malignant melanoma vagina. The tumour is seen protruding through vagina infiltrating urethra and reaching up to rectum (JPEG 76 kb)