Introduction/Background To describe a clinical case and review the literature of a primary clear cell adenocarcinoma of the vulva originating from malignant endometrioma, diagnosed and treated at the Hospital Universitario San Ignacio, in Bogotá, Colombia
Methodology A review was made of all articles in English or Spanish in databases: Scielo, LILACS, PubMEd, Cochrane, Embase.
Results in our review of the literature, we only found nine (9) cases reported with this histological type, of which seven (7) are associated with malignant endometriosis and the age range is from 34 to70 years.
Conclusion Vulvar clear cell carcinoma is an extremely rare pathology, we only found nine (9) cases, this being the first in our country and in Latin America. Most are associated with malignant endometriosis. There is no standard treatment, surgical management being the main one, associated with chemotherapy or radiotherapy in some cases.
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