Ovarian Fibrosarcoma: 5 Case Reports and Review of Literature

Abstract

Ovarian fibrosarcoma is an extremely rare and malignant sex-cord stromal tumor. Due to its low incidence and poor prognosis, till now very few cases have been reported and most of them are sporadic. The treatments and prognostic factors of ovarian fibrosarcoma are still under discussion. Here we report 5 cases of ovarian fibrosarcoma in Peking Union Medical College Hospital during the past 20 years. The 5 patients were 41, 51, 54, 76, 76 years old with initial symptoms of pelvic mass or pain. Manifestations on ultrasound are usually as unilateral pelvic masses, within which are uneven echo enhancement and some blood flow signals. There was no significant increase in preoperative tumor markers such as serum CA125 and sex hormones. Final diagnosis depends on postoperative histopathological results since these tumors were easily misdiagnosed by intraoperative frozen sections. Pathological examinations showed the tumor cells were spindle shaped with moderate to severe atypia and high mitotic counts. Immunohistochemistry is not specific but the positive rate of Ki-67 was consistent with the degree of malignancy and prognosis of the tumor. In addition, Vimentin, α-inhibin, SMA, estrogen receptor and progesterone receptor could also be positive. There were significant differences in surgical methods and there is also no unified chemotherapy. The overall survival was >15, >7, >6, <1, <1 year for each patient. After reviewing the literature, there is a lack of evidence-based large-scale case study. As for treatments, complete cytoreductive surgery plus regimens of malignant cord stromal tumors in NCCN guidelines are recommended. Due to its low incidence, multi-center clinical studies and molecular studies are required to give gynecologists a better understanding and guidance for future managements of ovarian fibrosarcoma.

A giant primary ovarian fibrosarcoma in a South Sudanese patient

“…It has been observed that patients normally present with non-specific symptoms such as pelvic mass or abdominal pain. [5] In this case, the patient presented with a painless, large abdominal mass causing abdominal distension. Eventually, as the mass grew into the epigastric region, she started to feel the discomfort and disfiguration which prompted her to seek medical attention.…”

“…[5] In this case, we presented a 20-year-old woman with primary ovarian fibrosarcoma. This seems to be the youngest in comparison to other case reports [5] where the reported ages range from 41to 76 years. Ray S et al reported [6] a case in a patient who was 23 years old.…”

“…Sex-cord stromal tumours contribute to 4.3% of ovarian tumours. [5] Most of the ovarian fibrosarcomas are seen at peri and post-menopausal women with a mean age of 49 years. [5] In this case, we presented a 20-year-old woman with primary ovarian fibrosarcoma.…”

“…[5] Most of the ovarian fibrosarcomas are seen at peri and post-menopausal women with a mean age of 49 years. [5] In this case, we presented a 20-year-old woman with primary ovarian fibrosarcoma. This seems to be the youngest in comparison to other case reports [5] where the reported ages range from 41to 76 years.…”

“…The sizes of ovarian fibrosarcomas vary from one case report to another. In the literature review conducted by Tingting et al, [5] ovarian fibrosarcoma sizes ranged from 5 to 23cm. However, Daramola et al [2] presented a case of size 13.6x8.1cm.…”