Prognostic value of the diagnostic criteria distinguishing endometrial stromal sarcoma, low grade from undifferentiated endometrial sarcoma, 2 entities within the invasive endometrial stromal neoplasia family


The World Health Organization (WHO 2003) recognizes 3 endometrial stromal neoplasms: noninvasive endometrial stromal nodule and the 2 invasive neoplasms, endometrial stromal sarcoma (ESS), low grade and undifferentiated endometrial sarcoma (UES). It is important to note that the WHO 2003 does not define moderate atypia (an important differentiating diagnostic criterion for ESS, low grade and UES), nor does it discuss its significance. Moreover, studies on reproducibility and additional prognostic value of other diagnostic features in large are lacking. Using strict definitions, we analyzed the agreement between routine and expert-review necrosis and nuclear atypia in 91 invasive endometrial stromal neoplasias (IESN). The overall 5-year and 10-year recurrence-free survival rate estimates of the 91 IESN patients were 82% and 75%, respectively. Necrosis was well reproducible, and nuclear atypia was reasonably well reproducible. The 10-year recurrence-free survival rates for necrosis absent/inconspicuous versus prominent were 89% and 45% (P<0.001) and those for review-confirmed none/mild, moderate, severe atypia were 90%, 30%, and <20% (P<0.00001). Therefore, cases with moderate/severe atypia should be grouped together. Nuclear atypia and necrosis had independent prognostic values (Cox regression). Once these features were taken into account, no other feature had an independent additional prognostic value, including mitotic count. Using "none/mild atypia, necrosis absent/inconspicuous" as ESS, low grade versus "moderate/severe atypia present or necrosis present" as UES resulted in 68 ESS, low grade and 23 UES cases with disease-specific overall mortality-free survival of 99% versus 48% (P<0.00001, hazard ratio=45.4). When strictly defined microscopic criteria are used, the WHO 2003 diagnoses of ESS, low grade and UES are well reproducible and prognostically strong. © 2012 International Society of Gynecological Pathologists.






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Feng, Weiwei, Anais Malpica, Stanley J Robboy, Einar Gudlaugsson, Keqin Hua, Xianrong Zhou and Jan PA Baak (2012). Prognostic value of the diagnostic criteria distinguishing endometrial stromal sarcoma, low grade from undifferentiated endometrial sarcoma, 2 entities within the invasive endometrial stromal neoplasia family. International Journal of Gynecological Pathology, 31(2). pp. 151–158. 10.1097/PGP.0b013e318229adfb Retrieved from

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Stanley J. Robboy

Professor Emeritus of Pathology

My research program, largely histopathological, concerns definitions of criteria, biological properties, differential diagnosis, and survival associated with pathological lesions in the female genital tract. With the gynecologic oncologists at Duke, we have reviewed the Institution's long term experience of endometrial cancer and with the International Collaborative Group on Endometrium, we have developed a new classification system for endometrial hyperplasia that better differentiates precancerous from benign lesions. In a nationwide NIH study in which I head the pathology review, long term complications in both sons and daughters from prenatal exposure to DES are being assessed. A major initiative is a study of vulvar dermatoses and unusual vulvar neoplasms. A final clinical pathologic correlation study, ongoing for over 40 years, has been the long term follow-up of a very rare tumor (malignant struma ovarii). Long term study has been required to identify those features that will ultimately prove to be of biologic significance for defining cases which will likely recur.

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