Unraveling the Complexities of Proximal-Type Epithelioid Sarcoma of the Vulva: A Rare Case of Diagnosis and Multidisciplinary Management

Proximal-type epithelioid sarcoma (PES) is one of the most aggressive malignancies having a high rate of recurrence with improper prognosis. Loss of SMARCB1 is a hallmark that plays a crucial role in pathogenesis and instructing therapeutic approaches. Constrained literature on PES underscores the requirement to have a thorough, multidimensional strategy of management. A 41-year-old woman exhibited having a mass without pain in the right labia majora. Modalities of imaging like MRI and PET/CT along with histopathology and immunohistochemistry affirmed PES with the loss of SMARCB1. The patient received an extended local excision accompanied by another excision and inguinal lymphadenectomy to have local control. Adjuvant radiotherapy was performed but was disrupted at 46 Gy because of severe adverse effects such as reactions of skin and dehiscence of the wound, both of Grade II.PET/CT scans that were executed after the treatment presented challenges in differentiating recurrence from therapy-associated changes. However, two years after the follow-up, the patient was free of disease. This case insists on the significance of a multidimensional strategy incorporating surgery, radiotherapy, and advanced imaging to manage vulvar PES. Challenges like radiation-produced side effects and the molecular pathology role, particularly the loss of SMARCB1, in the decision-making of therapy, are underscored. The results emphasize the requirement for strategies of personalized treatment and follow-up that is long-term in non-common malignancies such as PES. This case adds a contribution to the narrow literature on PES, giving insights into the advancements in diagnosis and treatment. It highlights the importance of resolving the challenges of treatment and offering individualized care for uncommon and aggressive types of cancer.