Cluster of Uterine Mullerian Adenosarcoma in the Washington, DC Metropolitan Area With High Incidence of Sarcomatous Overgrowth

Abstract

Mullerian adenosarcoma is an uncommon variant of uterine sarcoma. Twelve uterine adenosarcomas were diagnosed during a 42-month period at the Washington Hospital Center in Washington, DC. Based on estimated incidence data derived from the US Department of Defense beneficiary population, an estimated relative risk of 15.4 (95% confidence interval, 7.7-31.0) was calculated, indicating a significantly increased incidence of adenosarcoma in the population studied (p<0.0000001). Among 10 patients who underwent hysterectomy, six (60%) of their tumors had sarcomatous overgrowth. In comparison with the previously reported proportion of adenosarcomas with sarcomatous overgrowth, approximately 16%, the proportion with sarcomatous overgrowth was significantly higher than expected (p<0.01). Mullerian adenosarcoma with sarcomatous overgrowth was first described in 1989 and suggests that the cluster of adenosarcomas reported herein may be due in part to the current classification of some uterine tumors as adenosarcoma with sarcomatous overgrowth that previously would have been classified as other types of uterine sarcoma. Nonetheless, even after reviewing and updating the classification of all sarcomas diagnosed at the Washington Hospital Center from 1985 to 1998, the ratio of adenosarcomas to uterine adenocarcinomas during the 1994-1998 period was 4.7 times (p<0.005) that of the 1985-1993 period, suggesting a more modest but real increase in the occurrence of this tumor. Correct classification of mullerian adenosarcomas with sarcomatous overgrowth is important because the limited available data suggest that the prognosis is notably worse than that for adenosarcomas without sarcomatous overgrowth.

Mullerian adenosarcoma with sarcomatous overgrowth of the cervix presenting as cervical polyp: a case report and review of the literature

“…A classification of MASO is made when a pure sarcoma is present in more than 25% of the tumor, and when this sarcoma is in a grade similar to or higher than that of the underlying adenosarcoma ( 17,20 ) . Most cases that present as MASO originated from the uterine corpus; about 49 cases have been described as MASO of the uterus in the English literature ( 2,4,5,14,17,21 ) . No report has been described about the MASO of uterine cervix.…”

“…No report has been described about the MASO of uterine cervix. According to the Gynecologic Oncology Group clinicopathologic study in 31 cases of MA of uterus ( 2 ) , the rate of MASO in total MA of uterus is approximately 16% (5/31) at 1992 ( 2 ) , but during 1994–98, it was presented that the proportion of sarcomatous overgrowth was 60% (6/10) among the population diagnosed with uterine adenosarcoma by Seidman ( 21 ) . These data suggest that an important contributory factor in the increased incidence of adenosarcoma reported herein is the high proportion of cases displaying sarcomatous overgrowth.…”

Sonography and MRI of Tamoxifen-Associated Müllerian Adenosarcoma of the Uterus

“…Microscopically, the adenosarcoma presents a glandular epithelial component lined by a benign-appearing epithelium. The sarcomatous component contains oval to spindle-shaped cells with mild to moderate atypia [7,10]. To the best of B A E D Ascher et al [11,12] described two distinct MRI patterns in women receiving tamoxifen: homogeneous high signal intensity on T2-weighted images and enhanced endometrial-myometrial interface, or heterogeneous signal intensity on T2-weighted images and lattice-like enhancement traversing the endometrial canal on contrast-enhanced images.…”

Mullerian Adenosarcoma Of The Uterus With Sarcomatous Overgrowth

“…The term "Mullerian adenosarcoma with sarcomatous overgrowth (MASO) was first used by Clemant and Scully 8 in 1989 for those tumours in which a pure sarcoma, similar to or of a higher grade than that of the underlying adenosarcoma accounts for atleast one-quarter of the tumour after thorough histological sampling. Seidman et al 9 in their study have noted that tumours with sarcomatous overgrowth occurred in younger women, were larger and were more likely to have heterologous elements as compared with those without sarcomatous overgrowth. The main differential diagnosis of MASO is typical adenosarcoma with stromal predominant areas.…”