Sinichenkova Ksenia, Yasko Ludmila, Akhaladze Dmitry, Petrushin Anton, Konovalov Dmitry, Abasov Ruslan, Mareeva Yulia, Melekhina Olga, Usman Natalia, Karachunsky Alexander, Novichkova Galina, Litvinov Dmitry, Druy Alexander
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
Anatoly S. Loginov Moscow Clinical Scientific Center, Moscow, Russia.
Front Oncol. 2022 Oct 24;12:981230. doi: 10.3389/fonc.2022.981230. eCollection 2022.
In this report we firstly describe undifferentiated embryonal sarcoma of the liver (UESL) in a patient with neurofibromatosis type 1 (NF1), fatally complicated by synchronous malignant peripheral nerve sheath tumor (MPNST) with a highly aggressive metastatic course. The case also represents our first experience of chemoperfusion involving the transcatheter arterial chemoembolization (TACE) in a pediatric patient, applied as a treatment for UESL.
A 13-year-old girl was diagnosed with NF1 and presented with a liver tumor identified as UESL by histological assessment. The tumor was refractive to the conventional first-line chemotherapy. The patient received hepatic chemoperfusion with TACE, which was efficacious; however, the overall curative outcome was unsatisfactory due to synchronous unresectable retroperitoneal MPNST with mesenteric metastases and ultimate progression of the UESL.
This is the first reported case of UESL in a patient with NF1. The results demonstrate the efficacy of hepatic chemoperfusion with TACE in pediatric UESL.
在本报告中，我们首先描述了一名1型神经纤维瘤病（NF1）患者的肝脏未分化胚胎性肉瘤（UESL），该患者因同时发生恶性外周神经鞘瘤（MPNST）且伴有高度侵袭性的转移过程而致命。该病例也是我们首次在儿科患者中进行经导管动脉化疗栓塞术（TACE）化学灌注治疗UESL的经验。
一名13岁女孩被诊断为NF1，并经组织学评估发现患有肝脏肿瘤，确诊为UESL。该肿瘤对传统一线化疗无效。患者接受了TACE肝脏化学灌注治疗，治疗有效；然而，由于同时存在不可切除的腹膜后MPNST伴肠系膜转移以及UESL最终进展，总体治疗效果并不理想。
这是首例报道的NF1患者发生UESL的病例。结果表明TACE肝脏化学灌注治疗儿科UESL有效。
