Clinico-Pathological Challenges in Managing Rare Cancers of the Vulva: Experience at a Tertiary Cancer Center in South India

IntroductionCarcinoma of the vulva constitutes 4-5% of gynecologic malignancies with squamous cell carcinoma being the commonest. Rare histological types can cause diagnostic dilemmas and pose treatment challenges.AimClinico-pathological analysis of rare types of carcinoma vulva cases treated in our hospital and their follow-up.MethodologyCases diagnosed and treated at the hospital for ca vulva from January 2015 through December 2022 were included. Diagnosis, treatment details and follow-up was obtained.ResultsThe rare types of cancers of the vulva during the study period included spindle cell carcinoma, neuroendocrine carcinoma, sebaceous carcinoma, metastasis from breast cancer, melanoma of vulva and adenocarcinoma of intestinal type. Spindle cell carcinoma and neuroendocrine carcinoma had poor outcomes in-spite of multi-modality treatment. Sebaceous carcinoma patient was disease free after primary radiotherapy and died due to other causes during follow-up. The patient with malignant melanoma progressed after radiotherapy, chemotherapy and immunotherapy. The patient with metastasis from breast cancer also had further disease progression leading to fatality. The young patient with adenocarcinoma of intestinal type underwent surgery and brachytherapy and is currently on follow-up.ConclusionAwareness of rare tumors and IHC utilization will aid in diagnosis of rare cancers of the vulva. Extrapolating management of similar tumors arising in non-gynecological areas is currently followed. Reporting rare tumors helps in further research. SCC vulva can give good survival whereas the rare tumors may present with early recurrences and progression despite multi-modality approach.