Endometrial stromal sarcoma of the vulva arising in extraovarian endometriosis: A case report and literature review

Background. Extraovarian malignant transformation of endometriosis is a rare event; 52 cases have been reported. We describe the first published account in the world literature of an endometrial stromal sarcoma (ESS) arising in the vulva from a focus of endometriosis. Case. A 34-year-old G4 P3 morbidly obese white female was seen with a 1-year history of a palpable lesion arising within the superior aspect of the left labium majus. Radical left hemivulvectomy with clitorectomy was performed. The histopathologic findings were consistent with ESS arising from endometriosis associated with the extrapelvic portion of the round ligament within the canal of Nuck. Twenty months later metatastatic ESS developed in the upper lobe of the right lung and the patient underwent wedge resection. Nine months later she developed a recurrent pulmonary nodule at the site of the previous wedge resection and underwent successful right pulmonary lobectomy. The patient was last seen in October 1995 and had no evidence of disease at that time. Conclusions. Since approximately 0.7-1.0% of patients with endometriosis have lesions that will ultimately undergo malignant transformation, consideration should be given to bilateral oophorectomy at the time of abdominal surgery in patients with significant endometriosis. (C) 1998 Academic Press.