Extragenital mullerian adenosarcoma with sarcomatous overgrowth arising in an endometriotic cyst in the pouch of Douglas.

Mullerian adenosarcoma is a neoplasm composed of benign mullerian epithelium and a sarcomatous stroma. This tumor classically occurs in the endometrium of postmenopausal women and less frequently in the extrauterine genital tract. Rare cases of extragenital adenosarcoma have been reported. We present the case of a 23-year-old female who presented with an extragenital adenosarcoma arising in an endometriotic cyst in the pouch of Douglas. The patient was treated with local excision, chemotherapy and radiotherapy. At 2 years follow-up she was disease-free. The literature on extragenital adenosarcoma is reviewed. These tumors are clinically more aggressive than their uterine counterparts. Surgical excision is the initial treatment modality for these patients. Little information is available regarding the efficacy of adjuvant chemotherapy or radiotherapy. This is an area that requires further study.