The high-grade endometrial sarcoma: a rare entity.
BACKGROUND
With an estimated incidence of one to two per one million women, the endometrial stromal sarcoma (ESS) is a rare disease. It is subclassified into a high-grade and a prognostically better low-grade type. Evidence-based data for a standardized therapy is lacking.


METHODS
A 32-year-old obese nulligravida presented with persistent vaginal bleeding after the operation of an acute adnextorsion at another hospital. The repeat gynecological ultrasound examination showed a 5.3x5.3x3.6 cm vascularized, partially inhomogeneous mass in the uterus. A fractioned curettage yielded a differential diagnosis of malignant muellerian mixed tumor or a non-differentiated endometrial sarcoma. For completion of the operative treatment, laparotomy with hysterectomy, adnexectomy, and pelvine lymphonodectomy were performed. The final histological report described a 7 cm non-differentiated endometrial sarcoma with infiltration of the left ovary and 25 tumor-free lymph nodes.


CONCLUSIONS
Standard therapy for resectable sarcoma is abdominal hysterectomy and bilateral adnexectomy. So far, there is little data from studies reporting radio- or chemotherapy treatment of small patient numbers in an adjuvant setting.


CONCLUSIONS
The ESS is a very rare disease of the uterus. Due to missing clinical data, it remains a multidisciplinary therapeutic challenge requiring individual decisions. To receive more information on this rare disease, treatment should be performed according to international protocols.
Lenhard
S M
SM
Department of Obstetrics and Gynecology, Ludwig-Maximilians-University Munich, Campus Grosshadern, Marchioninistrasse 15, 80337, Munich, Germany. miriam.lenhard@med.uni-muenchen.de
Untch
M
M
Himsl
I
I
Ditsch
N
N
Bittmann
I
I
Friese
K
K
Bauerfeind
I
I
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016889:Endometrial Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007044:Hysterectomy; D008796:Metrorrhagia / Q000209:etiology; D018203:Sarcoma, Endometrial Stromal / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery