Ovarian fibrosarcoma: A single-institution experience and a review of the literature
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RESEARCH
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Ovarian fibrosarcoma: A single-institution experience and a review of the literature Ting-Ting Sun, Ning-Hai Cheng, Dong-Yan Cao and Peng Peng*
Abstract Ovarian fibrosarcoma is an extremely rare and malignant sex cord-stromal tumor. Due to its low incidence and poor prognosis, until now, very few cases have been reported, and most of the reported cases have been sporadic. Therefore, the treatments and prognostic factors of ovarian fibrosarcoma are still debatable. Here, we report 5 cases of ovarian fibrosarcoma that presented at Peking Union Medical College Hospital over the past 20 years. The 5 patients were 41, 51, 54, 76, and 76 years of age when initial symptoms of pelvic mass or pain appeared. On ultrasound, this disease usually manifests as unilateral pelvic masses, within which uneven echo enhancement and some blood flow signals are observed. No significant increase was observed in the levels of preoperative tumor markers, such as serum CA125 and sex hormones. The final diagnosis depends on postoperative histopathological results since these tumors are easily misdiagnosed when intraoperative frozen sections are used for examination. Pathologic examinations showed that the tumor cells were spindle-shaped with moderate to severe atypia and high mitotic counts. The immunohistochemistry profile is not specific, but the positive rate of Ki-67 was consistent with the degree of malignancy and the prognosis of patients with this tumor. In addition, the tumor may also be positive for Vimentin, α-inhibin, SMA, estrogen receptor and progesterone receptor. Significant differences were observed in the surgical methods used, and no unified chemotherapy regimen has been established. The overall survival was > 15, >7, > 6, 10 times in 10 highpower fields (HPFs). The immunohistochemical analysis showed AE1/AE3 (-), Calretinin (-), Ki-67 (index 5%), p53 (-), α-inhibin (-). After surgery, the serum CA125 level was 27.1 U/ml. This case was diagnosed as FIGO stage I A. The patient refused chemotherapy and remained diseasefree with normal ovarian function during the 7-year follow-up. Case 3
A 76-year-old woman was admitted with abdominal pain and fever. She had been postmenopausal for 28 years without any vaginal bleeding after the onset of menopause. Fourteen years ago, the patient underwent an exploratory laparotomy due to acute abdomen at another hospital. During the operation, 2800 ml of intraabdominal blood clots and non-clotted blood was removed, and a cauliflower-like tumor 3*3*2 cm in diameter with active bleeding was seen around the left tubal umbrella. Complete hysterectomy and bilateral salpingo-oophorectomy and omentectomy were performed. The postoperative pathology report described a diffuse granular follicular cell tumor of the left ovary with invasion of the left fallopian tube. The clinical FIGO stage was II C. After surgery, she received 3 cycles of systemic chemotherapy consisting of Cisplatin + Cyclophosphamide (PC), after which the patient did not return for follow-up. Four years later
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