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边缘切除术作为婴儿纤维肉瘤潜在的治愈性治疗选择，化疗后反应良好：一例 ETv6-NTRK3 阳性的胫骨远端婴儿纤维肉瘤的病例报告。

Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia.

机构信息

Faculty of Medicine, University of Regensburg, Regensburg, Germany.

Department of Orthopedic and Trauma Surgery, Barmherzige Brueder Regensburg Medical Center, Regensburg, Germany.

出版信息

Medicine (Baltimore). 2023 Jul 7;102(27):e34194. doi: 10.1097/MD.0000000000034194.

DOI:10.1097/MD.0000000000034194
PMID:37417635
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10328687/
Abstract

RATIONALE

The infantile fibrosarcoma (IFS) is a non-rhabdomyosarcoma soft tissue sarcoma with locally aggressive properties. State of the art therapy consists of neoadjuvant chemotherapy followed by wide resection according to the criteria of the musculoskeletal tumor society.

DIAGNOSES

An ETV6-NTRK3 positive IFS of the distal tibia in a 21-months old child showed good response to chemotherapy.

INTERVENTIONS

Due to refusal of amputation marginal resection completing the margins with a high speed drill and filling the space with bone cement was performed.

OUTCOMES

At latest follow-up 10 years after surgery, no recurrence was observed.

LESSONS

An individual therapy for surgical treatment of IIFS is recommended. This comprises marginal resection in instead of the golden standard "wide resection" in selected cases.

摘要

背景

婴儿型纤维肉瘤（IFS）是一种非横纹肌肉瘤的软组织肉瘤，具有局部侵袭性。目前的治疗方法是新辅助化疗，然后根据肌肉骨骼肿瘤协会的标准进行广泛切除。

诊断

21 个月大的儿童的胫骨远端 ETV6-NTRK3 阳性 IFS 对化疗反应良好。

干预措施

由于拒绝截肢，进行了边缘切除术，用高速钻头完成边缘，并填充骨水泥。

结果

手术治疗后 10 年的最新随访中，未观察到复发。

经验教训

建议对 IIFS 的手术治疗进行个体化治疗。在某些情况下，这包括边缘切除术，而不是标准的“广泛切除术”。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d015/10328687/d822c07598b8/medi-102-e34194-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d015/10328687/b7b79064acf5/medi-102-e34194-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d015/10328687/073ce9e142f1/medi-102-e34194-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d015/10328687/072a9dc8df20/medi-102-e34194-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d015/10328687/d822c07598b8/medi-102-e34194-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d015/10328687/b7b79064acf5/medi-102-e34194-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d015/10328687/073ce9e142f1/medi-102-e34194-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d015/10328687/072a9dc8df20/medi-102-e34194-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d015/10328687/d822c07598b8/medi-102-e34194-g004.jpg

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