Ovarian fibrosarcoma: A single-institution experience and a review of the literature.

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, <1, and < 1 year for each patient. After reviewing the literature, evidence-based large-scale case studies were lacking. For treatments, complete cytoreductive surgery plus regimens typically used against malignant sex cord-stromal tumors, as described in the NCCN guidelines, are recommended. Due to its low incidence, both multicenter clinical studies and molecular studies are required to provide gynecologists with a better understanding and guidance for future management of patients with ovarian fibrosarcoma.

Nuclear Atypia Is a Necessary Factor for Diagnosis of Primary Ovarian Fibrosarcoma: A Case Report and Literature Review.

INTRODUCTION: Primary ovarian fibrosarcoma is a rare malignant tumor with few case reports. The current opinion in diagnosis preferring mitotic activity was questioned as there is a large amount of heterogeneity in prognosis between cases. We report a case of primary ovarian fibrosarcoma, and a literature review was performed according to the latest diagnostic trends. This is the first study to review the prognostic factors of primary ovarian fibrosarcoma according to the latest trend in diagnosis. CASE PRESENTATION: A 50-year-old woman with primary ovarian fibrosarcoma is reported. The patient was admitted to the hospital due to increasing abdominal girth. Physical examination and CT scan showed a giant mass from the suprapubic area to the xiphoid region which filled her entire abdominopelvic cavity. Frozen sections were performed during an operation to resect, and the pathology report indicated a high degree of malignancy. Because of the degree of malignancy, a bilateral salpingo-oophorectomy and omentectomy were performed. Histopathological examination and immunohistochemical staining confirmed the diagnosis of primary ovarian fibrosarcoma. During the operation, the tumor ruptured, which placed the patient in a FIGO stage IC1. After surgery, the patient received 6 courses of combination chemotherapy, consisting of etoposide, bleomycin, and cisplatin. The patient has been free from disease without evidence of recurrence at 5 months after the initial diagnosis. CONCLUSIONS: Herein we report a case of primary ovarian fibrosarcoma and reviewed all published cases in English. Following the latest trend of diagnosis, multiple prognostic factors were used to determine survival prognosis. These included nuclear atypia, FIGO stage, mitotic counts, treatment methods, and tumor size. Only nuclear atypia was found to be associated with patient prognosis (p < 0.05). FIGO stage (p = 0.383), mitotic count per 10 hpfs (p = 0.155), treatment methods (p = 0.185), and tumor size (p = 0.972) were not found to be associated with survival prognosis. Nuclear atypia was an important factor in prognosis of patients. Using surgery with adjuvant medical treatment was inconclusive regarding patient survival.

Primary Ovarian Fibrosarcoma-An Unusual Tumor in an Adolescent: Case Report and Review of the Pediatric Literature.

BACKGROUND: Primary ovarian fibrosarcomas are rare and usually observed in perimenopausal and postmenopausal women. To our knowledge, to date, there are only 3 reports of ovarian fibrosarcoma in childhood and adolescence in the English-language literature. In this report, we aimed to present to our knowledge, the first pediatric case with advanced stage primary ovarian fibrosarcoma and to compare with previous cases. CASE: A 14-year-old teenage girl was admitted because of a giant abdominal mass. Imaging techniques revealed a giant heterogeneous and vascular ovarian mass. Salpingo-oophorectomy along with staging procedures were performed. Pathologic examination confirmed grade III ovarian fibrosarcoma with severe nuclear atypia and high mitotic count. Because of pulmonary metastasis, she received 4 courses of adjuvant combination chemotherapy (Ewing protocol). The patient withstood the treatment well and is disease-free at 2 years of follow-up. CONCLUSION: Fibrosarcoma of the ovary is an uncommon neoplasm with poor prognosis. To our knowledge, the presented patient is the first pediatric case with advanced staged primary ovarian fibrosarcoma to be treated with surgery and adjuvant chemotherapy.

Primary ovarian fibrosarcoma: a case report and review of the literature.

OBJECTIVES: Primary ovarian fibrosarcomas are extremely rare neoplasms, and only 50 cases have been reported in the English literature. Diagnosis can be difficult because of this condition's rarity, and other similar appearing mesenchymal lesions should be ruled out. METHODS: A 50-year-old postmenopausal woman came to our hospital because of abdominopelvic pain. Ultrasonography revealed a 41x33 mm heterogeneous solid mass in the right ovary. Total blood counts, biochemical parameters, and tumor markers were within normal ranges. Total abdominal hysterectomy, and bilateral salpingo oophorectomy were performed. Examination of a frozen, specimen revealed fibroma; however, the final histopathological diagnosis was low grade fibrosarcoma of the ovary. Microscopic examination demonstrated densely cellular, spindle-shaped tumor cells with increased mitotic activity (5 to 6 mitoses per 10 high-power fields). RESULTS: Immunohistochemical analysis revealed that the tumor cells were positive for vimentin and negative for actin and desmin and that the Ki 67 proliferation index was 30% to 40%. The patient did not receive adjuvant treatment, and remained free of disease after a follow up of 6 months. CONCLUSIONS: Although ovarian fibrosarcomas are unusual causes of solid masses in postmenopausal women, they should be considered when adnexal masses are examined in these patients. Mitotic activity and Ki-67 positivity were identified as important diagnostic factors for ovarian fibrosarcoma.

Mitotically active cellular fibroma of ovary should be differentiated from fibrosarcoma: a case report and review of literature.

The clinicopathologic characteristic of mitotically active cellular fibroma is significantly different from the malignant behavior of ovarian fibrosarcoma. Therefore, it's very important to differentiate mitotically active cellular fibroma from ovarian fibrosarcoma. We report a case in which a 39-year-old woman was found with an ovarian tumor measuring 105 × 71 × 47 mm. The tumor ruptured and adhered to the peritoneum. Microscopic examination showed densely cellular spindle-shaped tumor cells. The cellular atypia was mild. The Ki-67 proliferation index was approximately 10%. The patient remained free of tumor for more than 66 months without any adjuvant chemotherapy after operation. After reviewing the literature, we diagnosed this case as mitotically active cellular fibroma rather than ovarian fibrosarcoma. It is very important to differentiate these two tumors because of the marked differences in treatment modalities and prognosis between them. The ovarian fibrous tumors with mitotic figures ≥ 4 per 10 high-power fields but no severe nuclear atypia should be mostly diagnosed as mitotically active cellular fibroma of ovary. The correct diagnosis is the key to avoid excessive treatments.

A case of ovarian fibrosarcoma with pregnancy / 대한산부인과학회지

Ovarian fibromas originating primarily from the ovarian stroma are rare, accounting for approximately 4% of all ovarian neoplasm. The ovarian fibromas are mostly benign tumors, but infrequently they have histologically malignant appearances such as hypercellularity, high mitotic rate, marked nuclear pleomorphism. Malignant fibromatous tumor can be categorized on the basis of prognosis into two separate types of tumors, one of which is cellular fibroma (one to three miotic counts per 10 high power fields) and the other of which is fibrosarcoma (over four miotic counts per 10 high power fields) that has extremely poor prognosis. We report a case of large ovarian fibrosarcoma accompanied with pregnancy and have a brief review of literatures.

A Case of Primary Ovarian Fibrosarcoma / 대한산부인과학회지

Fibrosarcoma of the ovary is an exceedingly rare primary ovarian stromal tumor, which has a poor prognosis. Fibrosarcoma may arise de novo or as a result of malignant change in a benign fibromatous or fibrothecomatous tumor of the ovary. There are only about 30 cases in the literature reported within past three decades, showing the extreme rarity of these tumors. We report a case of primary ovarian fibrosarcoma with a review of the available literature.

A Case of Ovarian Fibrosarcoma / 대한산부인과학회잡지

Fibromatous tumors of the ovary are considered to originate from specialized ovarianstromal cells and account for approximately 4% of all ovarian neoplasms. Most ovarianfibromatous tumors are benign, but infrequently there are histologically malignant appearances.Malignant fibromatous tumor can be categorized into two separate types of tumorsrelating to prognosis, cellular fibroma and fibrosarcoma. The cellular fibroma has one tothree mitotic counts per 10 high power fields(HPF). Unless an adhesion or rupture appearson this tumor, recurrence should not happen after operation. Furthermore, where themitotic count per 10 HPF is over four, it is classified as a very bad fibrosarcoma case interms of prognosis.We have met a case of large ovarian fibrosarcoma with has ten mitotic counts per10 HPF. We hereby report this case with the brief review of literatures.