Kurtmen Bade T, Dokumcu Zafer, Divarci Emre, Serin Gürdeniz, Ergun Orkan, Ozok Geylani, Celik Ahmet
Department of Pediatric Surgery, Ege University Faculty of Medicine, İzmir, Turkey.
Department of Pathology, Ege University Faculty of Medicine, İzmir, Turkey.
J Pediatr Adolesc Gynecol. 2017 Dec;30(6):655-658. doi: 10.1016/j.jpag.2017.05.003. Epub 2017 May 11.
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.
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.
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.
原发性卵巢纤维肉瘤罕见，多见于围绝经期和绝经后女性。据我们所知，迄今为止，英文文献中仅有3例关于儿童及青少年卵巢纤维肉瘤的报道。在本报告中，我们旨在介绍我们所知的首例晚期原发性卵巢纤维肉瘤的儿科病例，并与既往病例进行比较。
一名14岁少女因腹部巨大肿块入院。影像学检查显示卵巢有一个巨大的异质性血管性肿块。实施了输卵管卵巢切除术及分期手术。病理检查确诊为III级卵巢纤维肉瘤，伴有严重核异型性和高有丝分裂计数。由于发生肺转移，她接受了4个疗程的辅助联合化疗（尤因方案）。患者对治疗耐受性良好，随访2年无疾病复发。
卵巢纤维肉瘤是一种罕见肿瘤，预后较差。据我们所知，该病例是首例接受手术及辅助化疗的晚期原发性卵巢纤维肉瘤儿科病例。
