Primary vulvovaginal choriocarcinoma: a case report of unusual presentation and literature review

Abstract

Only one case of primary extra uterine vaginal choriocarcinoma and one case of primary vulvar choriocarcinoma have been reported in literature. This is a case of 27 year old lady who presented with a 10cm × 7cm× 5cm vulvar mass with pain abdomen since 1 month, to the Gynecologic oncology outpatient. The mass was smooth, hard and fixed to underlying structures. Multiple bilateral inguinal lymph nodes were enlarged. Vulvar biopsy with Immunohistochemistry proved it to be choriocarcinoma. CT scan thorax, abdomen and pelvis showed multiple bilateral lung metastases, empty uterine cavity and normal sized uterus with a vaginal mass extending up to introitus encasing urethra and anal canal with multiple enlarged pelvic & inguinal lymph nodes. Final diagnosis of Primary Vulvovaginal choriocarcinoma FIGO stage III and WHO score-12 was made. Multidrug chemotherapy with Etoposide, Methotrexate, Actinomycin-D, Folinic Acid, Cyclophosphamide and Vincristine (EMA-CO) was started then shifted to Etoposide, Methotrexate, Actinomycin-D, Folinic Acid and Cisplatin (EMA-EP) regimen followed by Paclitaxel & Carboplatin, because of poor response. Patient’s βHCG became 1.57IU/L with resolution of all lesions after 5 three weekly cycles of Paclitaxel & Carboplatin. Now she is planned for three more cycles of chemotherapy. This case highlights another atypical presentation of choriocarcinoma.