J. Piura et al., PRIMARY SARCOMA OF THE OVARY - REPORT OF 5 CASES AND REVIEW OF THE LITERATURE, European journal of gynaecological oncology, 19(3), 1998, pp. 257-261
Primary ovarian sarcomas are rare and usually behave very aggressively
. Over a ten year period (1987-1996) five cases of primary ovarian sar
coma were managed at the Soroka Medical Center, Beer-Sheva, Israel. Fo
ur patients had malignant mullerian-mixed mesodermal tumor (MMMMT): tw
o had Stage IIIC tumor with chondrosarcoma being the predominant sarco
matous element, one had Stage IIIC tumor with high-grade endometrioid
stromal sarcoma (ESS) being the predominant sarcomatous element and on
e had Stage IC tumor with rhabdomyosarcoma being the predominant sarco
matous element. One patient had Stage LA leiomyosarcoma (LMS). All fou
r patients with MMMMT received postoperative adjuvant chemotherapy, wh
ereas the patient with LMS did not. The four patients with MMMMT died
of their disease 10, 10, 13 and 25 months, respectively, after initial
surgery. The patient with LMS died of intercurrent disease 21 months
after initial surgery. It is concluded that most patients with primary
ovarian sarcoma present with extraovarian disease and the prognosis i
s poor. The mainstay of treatment is debulking surgery consisting of t
otal abdominal hysterectomy, bilateral salpingo-oophorectomy and extir
pation of tumor masses. The benefit of postoperative adjuvant chemothe
rapy and/or radiotherapy is still a subject of debate and has yet not
been established.