Department of Pediatric Surgery, University Hospital of Saint-Etienne, Faculty of Medicine Jacques Lisfranc, PRES Lyon 42023, Jean Monnet University, Saint-Etienne, France.
Department of Pediatric Surgery, University Paris 7 Denis Diderot, Hôpital, Robert Debré, Assistance Publique-Hôpitaux de Paris, Paris, France.
Pediatr Blood Cancer. 2018 Apr;65(4). doi: 10.1002/pbc.26934. Epub 2017 Dec 29.
Efficacy and role of cytoreductive surgery (CRS) and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) remain poorly documented in pediatric tumors.
This retrospective national study analyzed all pediatric patients with peritoneal tumor spread treated by CRS and HIPEC as part of a multimodal therapy in France from 2001 to 2015.
Twenty-two patients (nine males and 13 females) were selected. The median age at diagnosis was 14.8 years (4.2-17.6). Seven had peritoneal mesotheliomas; seven, desmoplastic small round cells tumors (DSRCT); and eight, other histologic types. A complete macroscopic resection (CC-0, where CC is completeness of cytoreduction) was achieved in 16 (73%) cases. Incomplete resections were classified as CC-1 in four (18%) cases and CC-2 in two (9%) cases. Fourteen (64%) patients had complications within 30 days from HIPEC, requiring an urgent laparotomy in eight (36%) cases. Thirteen (59%) patients received adjuvant chemotherapy and four (18%) received total abdominal radiotherapy after surgery. Sixteen (72%) patients had relapse after a median time of 9.6 months (1.4-86.4) and nine (41%) eventually died after a median time of 5.3 months (0.1-36.1) from relapse. Six (27%) patients (four mesotheliomas, one pseudopapillary pancreatic tumor, and one DSRCT) were alive and in complete remission after a median follow-up of 25.0 months (5.3-78.2). The mean overall survival (OS) and disease-free survival (DFS) were 57.5 months (95% CI [38.59-76.32]) and 30.9 months (95% CI [14.96-46.77]). Patients with a peritoneal mesothelioma had a significantly better OS (p = 0.015) and DFS (p = 0.028) than other histologic type.
In this national series, outcomes of HIPEC are encouraging for the treatment of peritoneal mesothelioma in children.
细胞减灭术（CRS）和腹腔热灌注化疗（HIPEC）的疗效和作用在儿科肿瘤中仍记录不佳。
本回顾性全国性研究分析了 2001 年至 2015 年期间在法国，作为多模式治疗的一部分，通过 CRS 和 HIPEC 治疗的腹膜肿瘤播散的所有儿科患者。
选择了 22 名患者（9 名男性和 13 名女性）。诊断时的中位年龄为 14.8 岁（4.2-17.6）。7 例为腹膜间皮瘤；7 例为促纤维增生性小圆细胞肿瘤（DSRCT）；8 例为其他组织学类型。16 例（73%）患者达到完全宏观切除（CC-0，其中 CC 为细胞减灭术的完整性）。4 例（18%）和 2 例（9%）患者分别为不完全切除 CC-1 和 CC-2。14 例（64%）患者在 HIPEC 后 30 天内出现并发症，8 例（36%）需要紧急剖腹手术。13 例（59%）患者术后接受辅助化疗，4 例（18%）接受全腹部放疗。中位时间为 9.6 个月（1.4-86.4）后，16 例（72%）患者复发，9 例（41%）患者从复发后中位时间 5.3 个月（0.1-36.1）后最终死亡。6 例（27%）患者（4 例间皮瘤、1 例胰腺假乳头状瘤和 1 例 DSRCT）在中位随访 25.0 个月（5.3-78.2）后存活且完全缓解。总生存（OS）和无病生存（DFS）的平均值分别为 57.5 个月（95%CI[38.59-76.32]）和 30.9 个月（95%CI[14.96-46.77]）。腹膜间皮瘤患者的 OS（p=0.015）和 DFS（p=0.028）明显优于其他组织学类型。
在本项全国性研究中，HIPEC 治疗儿童腹膜间皮瘤的结果令人鼓舞。
