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儿童恶性卵巢肿瘤:一家机构22年的经验

Malignant ovarian tumors in children: 22 years of experience at a single institution.

作者信息

Akyüz C, Varan A, Büyükpamukçu N, Kutluk T, Büyükpamukçu M

机构信息

Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, Ankara, Turkey.

出版信息

J Pediatr Hematol Oncol. 2000 Sep-Oct;22(5):422-7. doi: 10.1097/00043426-200009000-00007.

Abstract

PURPOSE

Malignant ovarian tumors of childhood are relatively rare and thus, management is still unclear. We reviewed our experience with these tumors to evaluate their histopathologic characteristics, treatment, and outcome.

PATIENTS AND METHODS

From January 1975 to December 1997, 56 patients had their malignant ovarian tumors diagnosed, treated, and followed-up in our institution. All tumors were completely excised when possible; otherwise, biopsy was performed. Staging was made according to Federation Internationale de Gynecologie Oncologique classification. Chemotherapy was recommended for all patients. Twelve cases were treated with vincristine, actinomycin, cyclophosphamide (VAC) before 1986; 12 with cisplatin, vinblastine, and bleomycin (PVB) from 1986 to 1989; and 23 with the bleomycin, etoposide, and cisplatin (BEP) regimen from 1989 to present. The Kaplan-Meier survival method was used to calculate the survival. The log-rank test was used to compare groups with respect to survival.

RESULTS

Age range was 0 to 16 years (median 11 yrs; average 9.8 yrs). Only two patients were younger than 1 year. The most common presenting symptom was abdominal pain, occurring in 27 patients (48.2%). Thirty-three patients (60%) had total one-sided salpingo-oophorectomy and three patients had bilateral salpingo-oophorectomy. Nineteen patients had stage I, 15 had stage II, 19 had stage III, and 3 had stage IV disease. Dysgerminoma was the most common type. Overall survival (OAS) and event-free survival were 68% (median follow-up time: 71 mos) and 57%, respectively, after 22 years. Histopathology was not correlated with survival. Two important predictors for survival are age (P < 0.0001) and treatment protocol (P = 0.013). The BEP protocol was superior to the other regimens. The OAS was 74.6% in BEP, 55% in PVB, and 63.6% in VAC regimens.

CONCLUSION

Although age at diagnosis and treatment with BEP regimen have major roles in determining prognosis of the ovarian tumors in childhood, for patients with advanced ovarian germ cell tumors, intensification of chemotherapy or the development of new approaches is necessary.

摘要

目的

儿童恶性卵巢肿瘤相对罕见,因此其治疗方案仍不明确。我们回顾了我们对这些肿瘤的治疗经验,以评估其组织病理学特征、治疗方法及预后。

患者与方法

1975年1月至1997年12月,我院共诊断、治疗并随访了56例儿童恶性卵巢肿瘤患者。所有肿瘤均尽可能完整切除;若无法完整切除,则进行活检。根据国际妇产科联盟(FIGO)的分类标准进行分期。所有患者均建议接受化疗。1986年前,12例患者采用长春新碱、放线菌素、环磷酰胺(VAC)方案治疗;1986年至1989年,12例患者采用顺铂、长春花碱、博来霉素(PVB)方案治疗;1989年至今,23例患者采用博来霉素、依托泊苷、顺铂(BEP)方案治疗。采用Kaplan-Meier生存分析法计算生存率,采用对数秩检验比较各治疗组的生存率。

结果

患者年龄范围为0至16岁(中位年龄11岁;平均年龄9.8岁)。仅2例患者年龄小于1岁。最常见的症状为腹痛,27例患者(48.2%)出现该症状。33例患者(60%)接受了单侧附件全切术,3例患者接受了双侧附件全切术。19例患者为Ⅰ期,15例为Ⅱ期,19例为Ⅲ期,3例为Ⅳ期。无性细胞瘤是最常见的病理类型。22年后,总生存率(OAS)和无事件生存率分别为68%(中位随访时间:71个月)和57%。组织病理学与生存率无关。两个重要的生存预测因素是年龄(P < 0.0001)和治疗方案(P = 0.013)。BEP方案优于其他方案。BEP方案的OAS为74.6%,PVB方案为55%,VAC方案为63.6%。

结论

尽管诊断时的年龄和采用BEP方案治疗对儿童卵巢肿瘤的预后有重要影响,但对于晚期卵巢生殖细胞肿瘤患者,仍需强化化疗或探索新的治疗方法。

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