Endometrial stromal sarcoma: a systematic review.
OBJECTIVE
To summarize available studies with respect to evaluation and management of patients with endometrial stromal sarcoma and undifferentiated endometrial sarcoma.


METHODS
We conducted an electronic search of research articles published in English between January 1, 1981, and January 1, 2013, using MEDLINE, PubMed, and ClinicalTrials.gov (www.clinicaltrials.gov) databases.


METHODS
Of the 115 studies initially identified, 86 were chosen after limiting the review to those articles focusing on endometrial stromal sarcoma and crossreferencing to eliminate duplication. Review articles were excluded. Of the 86 studies meeting eligibility criteria, 84 were retrospective, one was a prospective phase II trial, and one was a phase III randomized study. Data were extracted systematically. Each of the reviewers assessed the quality of each study independently.


RESULTS
Data were abstracted using standard abstraction templates to summarize study findings. Given the rarity of this tumor, we report available data with respect to epidemiology, pathogenesis, prognostic factors, and treatment. Endometrial stromal sarcoma and undifferentiated endometrial sarcoma comprise an estimated 1% of all uterine cancers and less than 10% of all uterine mesenchymal neoplasms. Hysterectomy and bilateral salpingo-oophorectomy is the cornerstone of treatment for early-stage (I or II) disease. Surgical resection when feasible may also be appropriate for patients presenting with advanced-stage tumors. The value of adjuvant therapy for early-stage disease remains unproven. Hormone therapy continues to be the most efficacious treatment modality for patients with advanced-stage or recurrent disease.


CONCLUSIONS
Endometrial stromal sarcoma and undifferentiated endometrial sarcoma are rare tumors. Surgical resection is appropriate for patients with early-stage (I or II) disease and those with resectable, advanced-stage (III or IV) tumors. Hormone therapy may be appropriate in treating advanced and recurrent disease.
Division of Gynecologic Oncology, Vincent Obstetrics and Gynecology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts 02114, USA.
J Alejandro
JA
Rauh-Hain
Marcela G
MG
del Carmen
D016889:Endometrial Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D005260:Female; D006801:Humans; D018203:Sarcoma, Endometrial Stromal / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*