Extrauterine Endometrial Stromal Sarcoma: A Clinicopathologic Study of 63 Cases
Ramya P Masand, Elizabeth D Euscher, Michael T Deavers, Anais Malpica. The University of Texas MD Anderson Cancer Center, Houston, TX
Background: Extrauterine endometrial stromal sarcoma (EESS) is an uncommon tumor that occurs in patients (pts) over a wide age range. The extrauterine location, non-gynecologic symptoms/signs at presentation and confounding histologic features can pose a diagnostic challenge. In this study, we present the clinicopathologic features of 63 such cases.
Design: 63 cases of EESS from a period of 20 yrs were retrieved. Clinical information was obtained from the pts' charts and questionnaires sent to physicians. The following clinical parameters were recorded: pts' age, clinical presentation, treatment (tx), recurrences and current status. In 46 cases slides were re-reviewed. The following pathologic parameters were recorded: size and location, gross and microscopic features, and evidence of endometriosis.
Results: The pts' ages ranged from 27 to 87 yrs (median 50). The most common symptoms/signs were: abdominal/pelvic mass or pain, vaginal bleeding, and gastrointestinal symptoms. Tumor size ranged from 1.2 to 24.5 cm. The most common sites of involvement included ovaries (24), bowel wall (27), abdomen/peritoneum (37), pelvis (20) and vagina (6). Multiple sites of involvement were present in 34 cases. 42/45 cases had a classic microscopic pattern and 1 had dedifferentiation; 16 had vascular invasion. Sex-cord elements were seen in 8, smooth muscle differentiation in 3, myxoid change in 4, and hyaline plaques in 23 cases. Stromal fibrosis was present diffusely in 5 and focally in 21 cases. Endometriosis was noted in 29/63 cases. CD10 was diffusely (+) in 26 and focally in 3 of 29 cases. Desmin was focally (+) in 7/15 cases. ER was (+) in 27/28 cases; PR was (+) in 33/34 cases. In 25% of cases an initial diagnosis other than ESS was made: GIST, leiomyosarcoma, liposarcoma, synovial sarcoma, MPNST and various sex cord stromal tumors. 61/63 pts had cytoreductive surgery, 31/48 had hormonal tx, 13/45 had chemotx, and 7/45 had radiation tx. Follow up ranged from 5 to 336 months; 14 cases were lost to follow up. 29 pts had recurrent disease; the time to recurrence ranged from less than 12 mos to 192 mos (median 42 mos). 7 pts died of disease (DOD) with a median of 70 mos from dx to death, 14 pts are alive with disease, and 27 pts are alive without disease.
Conclusions: EESS is commonly associated with endometriosis and tends to be indolent with a propensity for recurrence. 6/7 pts who DOD had bowel involvement and 1 had dedifferentiation. Unusual histologic features or presentation and the extrauterine location lead to an inaccurate dx in a significant number of cases.
Category: Gynecologic & Obstetrics
Monday, March 19, 2012 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 185, Monday Morning