Radiation Oncology/Uterine Sarcoma/Overview

Uterine Sarcoma Overview


 * Comprise <1% of GYN malignancies and 2-5% of uterine malignancies
 * Three distinct tissues of origin:
 * Carcinosarcoma (mixed mullerian tumor): endometrium, accounts for 40-50%. High frequency of TP53 mutations.
 * Subtypes:
 * Heterologous type:
 * Sarcomatous components derived from skeletal muscle, cartilage, or bone; frequently rhabdomyosarcomas or chondrosarcomas
 * More aggressive, poorer prognosis, higher risk of recurrence
 * Homologous type: Sarcomatous component derived from endometrium, usually resemble high-grade undifferentiated sarcoma or fibrosarcoma
 * GOG 150: Whole Abdominal Irradiation (WAI) vs Cisplatin-Ifosfamide and Mesna (CIM) as post-surgical therapy
 * 206 patients with uterine carcinosarcoma
 * Stages I-IV eligible as long as no demonstrable parenchymal hepatic involvement or extra-abdominal distant disease
 * All patients underwent TAH, BSO, and maximal resection of all gross intra-abdominal/pelvic disease, including macroscopically involved pelvic and para-aortic nodes, leaving no residual disease any larger than 1 cm
 * WAI: 1 cm margins on the diaphragm superiorly, the inguinal ligament inferiorly, and the lateral aspect of the peritoneal margin laterally; AP/PA; total dose 30 Gy. 20 Gy 4-field boost to true pelvis.
 * CIM: cisplatin (20 mg/m2/day IV × 4 days) that was to be followed by a one hour IV administration of Ifosfamide (1.5 g/m2/day IV × 4 days) with mesna (120 mg/m2 IV bolus over 15 minutes on day one, followed by 1.5 g/m2/day IV continuous infusion × 4 days beginning with day one) every three weeks for three cycles.
 * Primary endpoints: Death and recurrence rates
 * No statistically significant difference in time to recurrence (at 5 years, 58% WAI vs 52% CIM)
 * No statistically significant difference in survival time (at 5 years, 35% WAI vs 45% CIM)
 * Slightly more vaginal recurrences in CIM group and more abdominal recurrences in WAI group, but not statistically significant
 * AEs: Higher grade 3 or 4 acute anemia and neuropathy in CIM group, higher grade 2-4 gastrointestinal events in WAI group. Two WAI patients died of radiation hepatitis. One CIM patient died of acute port infection complicated by neutropenia.


 * Leiomyosarcoma: myometrial muscle, accounts for ~30%
 * Endometrial stromal sarcoma: endometrial stroma, accounts for ~15%
 * Please see more detail at cancer.gov PDQ
 * Shueng et al. (2013) Unusual Oligometastases of Distant Nodes as the Initial Relapse Site of Uterine Sarcoma: A Case Report, Therapeut Radiol Oncol 2013; 20(4): 315-321.