Gustave Roussy, Université Paris-Saclay, Department of Children and Adolescent oncology, Villejuif, France.
Institut Multisite de Pathologie, Hospices civils de Lyon, Bron, France.
Pediatr Blood Cancer. 2020 Apr;67(4):e28186. doi: 10.1002/pbc.28186. Epub 2020 Jan 25.
To describe characteristics and outcome of pediatric ovarian immature teratomas (IT) to better define the place of chemotherapy.
Children with ovarian IT enrolled in TGM95 and TGM2013 studies were analyzed. Norris grading and International Federation of Gynecology and Obstetrics staging system were used.
Thirty-six cases were identified with a median age of 11 years (range = 1-18): 35 of 36 stage I (17 stage IA, 13 stage IC, and 5 stage IX), including seven patients with gliomatosis peritonei (GP), and 1 stage IIIB (IT peritoneal implants). Centrally reviewed Norris grading was performed in 31 cases: 14 grade I and 17 grade II/III tumors. All patients underwent upfront surgery: 19 unilateral oophorectomy, 14 unilateral adnexectomy, 2 unilateral cystectomy, and 1 bilateral cystectomy. No extensive GP surgery was performed. Six patients received adjuvant vinblastin, bleomycin, and cisplatinum because of tumor rupture (n = 5, including two patients with GP) or stage III (n = 1). After a median follow-up of 39.5 months (range = 6-238), two events occurred 10 and 11 months after diagnosis: one bilateralization (initial stage IX, grade I) and one IT peritoneal relapse (initial stage IA, grade II), respectively. Both were successfully rescued by platinum-based chemotherapy and delayed surgery. No stage IC patients treated without adjuvant chemotherapy relapsed (four grade I and three grade III). None of the seven patients with GP progressed. Five-year event-free survival and overall survival were 94% (95% CI = 81-98%) and 100%.
The current series confirms the excellent prognosis of pediatric ovarian IT, arguing for conservative surgical approach in GP and against systematic adjuvant chemotherapy, even in ruptured tumors.
描述儿童卵巢未成熟畸胎瘤（IT）的特征和结局，以更好地确定化疗的地位。
对参加 TGM95 和 TGM2013 研究的儿童卵巢 IT 患者进行分析。采用诺里斯分级和国际妇产科联合会分期系统。
共确定 36 例病例，中位年龄为 11 岁（范围为 1-18 岁）：36 例均为Ⅰ期（17 例 IA 期、13 例 IC 期和 5 例 IX 期），包括 7 例腹膜胶质瘤病（GP）患者，1 例ⅢB 期（IT 腹膜种植）。对 31 例病例进行了中心审查诺里斯分级：14 例为Ⅰ级，17 例为Ⅱ/Ⅲ级肿瘤。所有患者均接受了初始手术：19 例行单侧卵巢切除术，14 例行单侧附件切除术，2 例行单侧囊肿切除术，1 例行双侧囊肿切除术。未行广泛 GP 手术。由于肿瘤破裂（n=5，包括 2 例 GP 患者）或Ⅲ期（n=1），6 例患者接受了辅助长春碱、博来霉素和顺铂治疗。诊断后 10 个月和 11 个月各发生 1 例事件：1 例双侧化（初始 IX 期，Ⅰ级）和 1 例 IT 腹膜复发（初始 IA 期，Ⅱ级）。两者均通过铂类化疗和延迟手术成功挽救。未接受辅助化疗的 IC 期患者均无复发（4 例Ⅰ级和 3 例Ⅲ级）。7 例 GP 患者均未进展。5 年无事件生存率和总生存率分别为 94%（95%CI=81-98%）和 100%。
本研究证实了儿童卵巢 IT 具有良好的预后，支持在 GP 中采用保守的手术方法，且不主张对破裂肿瘤进行系统辅助化疗。
