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Uterine sarcomas are rare tumors that account for 3% of uterine cancers. Their histopathologic
classification was revised by the World Health Organization (WHO) in 2003. A new staging
system has been recently designed by the International Federation of Gynecology and
Obstetrics (FIGO). Currently, there is no consensus on risk factors for adverse outcome.
This review summarizes the available clinicopathological data on uterine sarcomas
classified by the WHO diagnostic criteria.
Medline was searched between 1976 and 2009 for all publications in English where the
studied population included women diagnosed of uterine sarcomas.
Since carcinosarcomas (malignant mixed mesodermal tumors or MMMT) are currently classified
as metaplastic carcinomas, leiomyosarcomas remain the most common uterine sarcomas.
Exclusion of several histologic variants of leiomyoma, as well as "smooth muscle tumors
of uncertain malignant potential," frequently misdiagnosed as sarcomas, has made apparent
that leiomyosarcomas are associated with poor prognosis even when seemingly confined
to the uterus. Endometrial stromal sarcomas are indolent tumors associated with long-term
survival. Undifferentiated endometrial sarcomas exhibiting nuclear pleomorphism behave
more aggressively than tumors showing nuclear uniformity. Adenosarcomas have a favorable
prognosis except for tumors showing myometrial invasion or sarcomatous overgrowth.
Adenofibromas may represent well-differentiated adenosarcomas. The prognosis of carcinosarcomas
(which are considered here in a post-script fashion) is usually worse than that of
grade 3 endometrial carcinomas. Immunohistochemical expression of Ki67, p53, and p16
is significantly higher in leiomyosarcomas and undifferentiated endometrial sarcomas
than in endometrial stromal sarcomas.
Evaluation of H&E stained sections has been equivocal in the prediction of behavior
of uterine sarcomas. Immunohistochemical studies of oncoproteins as well as molecular
analysis of non-random translocations will undoubtedly lead to an accurate and prognostically
relevant classification of these rare tumors.