Extragenital Müllerian adenosarcoma with pouch of Douglas location

A 49-year-old para-0 woman, was seen at our OB/GYN-UNIT because she complained vaguely of pelvic pain. She had a mass of undefined nature in the pouch of Douglas. A simple excision of the mass showed low-grade Müllerian adenosarcoma with areas of stromal overgrowth. One and a half year after surgery, at another hospital, a mass was detected in the patient's posterior vaginal fornix and removed surgically. Six months later she came back to our observation with vaginal bleeding and mass in the vaginal fornix. We performed radical surgery. The pathological examination showed recurrent adenosarcoma. Surgical treatment was supplemented by radiation therapy.The case of Müllerian adenosarcoma reported here is the third known so far in the literature that was located in the pouch of Douglas. To date, only two other such cases have been reported, including one resulting from neoplastic degeneration of an endometriotic cyst.Malignant stromal tumors account for 1-3% of all female genital tract tumors; 8-10% of these are Müllerian adenosarcomas. Adenosarcomas are rare tumors scarcely reported in the medical literature that associate benign glandular epithelium with a malignant endometrial stromal component of usually low histological grade. Occasionally, a high-grade malignant stromal component may raise a differential diagnosis issue with leiomyosarcomas and carcinosarcomas [1,2].Adenosarcomas arise more frequently in the uterus [3], but cases with extrauterine locations in the ovaries [4], cervix [5], vagina [6], and peritoneum [7] have also been reported. Only two cases arising in the pouch of Douglas are known so far [8]. The recurrence rate and the medium- to long-term survival rate depend on the grade and mitotic index of the stromal component [9,10] and on the presence of sarcomatous overgrowth [11]. Tumor location is also an important prognostic factor as extragenital tumors have proved to be more aggressive [8,12].Of great interest is the association between adenosarco