High grade undifferentiated uterine sarcomas (HGUS) are rare, aggressive malignancies. Data regarding management are limited. We aimed to describe disease stage, response to treatment, and survival outcomes among patients with HGUS at our institution.

We identified all patients with HGUS who received treatment at our institution from 1/2000 to 3/2011. Demographics, surgical procedures, disease stage, treatment response, and survival outcomes were abstracted from the medical records.

Twenty-one patients were identified. FIGO 2008 stage distribution was: I-7 (33%), II-1 (5%), III-2 (10%), IV-11 (52%). Eighteen of 21 patients (86%) undergoing primary surgical resection achieved a complete gross resection; however, progression within the abdominal cavity was identified in 11 patients (61%) by the time they underwent postoperative imaging. Of 13 patients who received first-line chemotherapy for measurable disease, the overall response rate was 62%. Responses were observed in patients treated with gemcitabine/docetaxel (6 of 8) and doxorubicin-based regimens (2 of 5). Progression-free and overall survivals for the entire cohort were 7.3 months and 11.8 months, respectively. In 14 patients with measurable disease at the time of treatment, 1-year survival was 35.7% versus 80% in 5 patients without measurable disease at time of treatment (P=0.112). Nine patients received second- or additional chemotherapy for progressive disease, with response rate of 19%. Time to progression was short among responders.

HGUS represents a distinct histologic subtype of uterine sarcoma with poor outcomes regardless of stage at diagnosis. A majority rapidly develops distant metastases despite surgical resection. Gemcitabine-docetaxel and doxorubicin-based treatment achieved objective, but short-lived, responses in patients with measurable disease.