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儿童腹膜恶性肿瘤的细胞减灭术及腹腔热灌注化疗:单中心初步经验

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal malignant tumors in children: Initial experience in a single institution.

作者信息

Zhu Zhiyun, Chang Xiaofeng, Wang Jiarong, Yang Shen, Qin Hong, Yang Wei, Cheng Haiyan, Meng Deguang, Wang Huanmin

机构信息

Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.

Department of Surgical Oncology, Baoding Branch of Beijing Children's Hospital, Baoding Children's Hospital, Baoding, China.

出版信息

Front Surg. 2023 Jan 11;9:1078039. doi: 10.3389/fsurg.2022.1078039. eCollection 2022.

Abstract

BACKGROUND

Peritoneal malignant tumors in children are rare but commonly associated with disease progression and poor outcome. The successful treatment experience of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in adult peritoneal carcinoma has been applied to pediatric peritoneal malignancy in recent years. However, patients with desmoplastic small round cell tumor (DSRCT) accounted for the majority of patients treated with CRS and HIPEC in previous studies. The role of CRS and HIPEC remains controversial due to the rarity of the disease and the limited sample size of studies. Additionally, the cases using CRS and HIPEC except DSRCT were mainly small case reports with unclear outcomes. We present our experience in the treatment of pediatric peritoneal malignancies using CRS and HIPEC, with more emphasis on the safety, feasibility, and short-term outcome.

METHODS

A retrospective query from December 2019 to February 2022 identified 19 children with peritoneal malignancies who underwent CRS and HIPEC in our institution. Clinical characteristics, therapies, and outcomes were summarized and analyzed.

RESULTS

The median age of the patients was 6.4 years (range, 0.7-13.9 years). The histologic types included rhabdomyosarcoma (7), Wilms tumor (2), clear cell sarcoma of the kidney (2), undifferentiated sarcoma (2), immature teratoma (1), peritoneal serous carcinoma (1), malignant rhabdoid of the kidney (1), malignant germ cell tumor (1), neuroblastoma (1), and epithelioid inflammatory myofibroblast sarcoma (1). Seven patients underwent initial operation, and 12 patients received reoperation for tumor recurrence. The median peritoneal carcinomatosis index was 5 (range, 2-21). There were no perioperative deaths or life-threatening complications of CRS and HIPEC. Two patients had grade 3 complications of wound infection and wound dehiscence. With a median follow-up time of 14 months (range, 1.5-31 months), 14 patients were alive, and 5 died of tumor recurrence. Of the 14 patients who were alive, 2 relapsed after CRS and HIPEC and then received radiotherapy and molecular-targeted therapy or chemotherapy.

CONCLUSIONS

CRS and HIPEC are safe and feasible in children, without increasing serious complications in the peri- and postoperative periods. The complication is acceptable. The short-term outcome shows possible effectiveness in pediatric peritoneal malignant tumors. The long-term effectiveness needs to be verified by additional cases and long-term follow-ups.

摘要

背景

儿童腹膜恶性肿瘤较为罕见,但通常与疾病进展及不良预后相关。近年来,减瘤手术(CRS)及腹腔内热灌注化疗(HIPEC)在成人腹膜癌中的成功治疗经验已应用于儿童腹膜恶性肿瘤。然而,在既往研究中,促结缔组织增生性小圆细胞肿瘤(DSRCT)患者占接受CRS和HIPEC治疗患者的大多数。由于该疾病罕见且研究样本量有限,CRS和HIPEC的作用仍存在争议。此外,除DSRCT外,使用CRS和HIPEC的病例主要是小样本病例报告,结局尚不清楚。我们介绍了我们使用CRS和HIPEC治疗儿童腹膜恶性肿瘤的经验,更侧重于安全性、可行性和短期结局。

方法

回顾性查询2019年12月至2022年2月期间在我院接受CRS和HIPEC的19例儿童腹膜恶性肿瘤患者。总结并分析其临床特征、治疗方法及结局。

结果

患者的中位年龄为6.4岁(范围0.7 - 13.9岁)。组织学类型包括横纹肌肉瘤(7例)、肾母细胞瘤(2例)、肾透明细胞肉瘤(2例)、未分化肉瘤(2例)、未成熟畸胎瘤(1例)、腹膜浆液性癌(1例)、肾恶性横纹肌样瘤(1例)、恶性生殖细胞肿瘤(1例)、神经母细胞瘤(1例)和上皮样炎性肌成纤维细胞肉瘤(1例)。7例患者接受了初次手术,12例患者因肿瘤复发接受了再次手术。中位腹膜癌指数为5(范围2 - 21)。CRS和HIPEC围手术期无死亡病例或危及生命的并发症。2例患者出现3级伤口感染和伤口裂开并发症。中位随访时间为14个月(范围1.5 - 31个月),14例患者存活,5例死于肿瘤复发。在14例存活患者中,2例在CRS和HIPEC后复发,随后接受了放疗及分子靶向治疗或化疗。

结论

CRS和HIPEC在儿童中是安全可行的,不会增加围手术期及术后严重并发症。并发症是可接受的。短期结局显示对儿童腹膜恶性肿瘤可能有效。长期有效性需要更多病例及长期随访来验证。

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