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巨大转移性恶性周围神经鞘瘤的外科治疗与靶向治疗：一例报告及文献综述

Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review.

作者信息

Skórka Patryk, Kordykiewicz Dawid, Ilków Andrzej, Ptaszyński Konrad, Wójcik Janusz, Skórka Wiktoria, Wojtyś Małgorzata Edyta

机构信息

Department of Thoracic Surgery and Transplantation, Pomeranian Medical University in Szczecin, Alfreda Sokołowskiego 11, 70-891 Szczecin, Poland.

Department of General, Vascular and Oncological Surgery, Provincial Hospital, Mikołaja Kopernika, Tytusa Chałubińskiego 7, 75-581 Koszalin, Poland.

出版信息

Life (Basel). 2024 Dec 12;14(12):1648. doi: 10.3390/life14121648.

DOI:10.3390/life14121648
PMID:39768355
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11680011/
Abstract

Neurofibromatosis type 1 (NF1) significantly increases the risk of malignant peripheral nerve sheath tumors (MPNST), a rare and aggressive malignancy for which treatment is clinically challenging. This paper presents the case of a 24-year-old male with an NF1 who developed MPNST with lung metastases. Due to the limited effectiveness of systemic therapy in the treatment of MPNST, the patient underwent radical surgical resection and radiotherapy. Pathological evaluation confirmed high-grade MPNST, and PET-CT imaging revealed further metastatic progression. The treatment results for our patient are compared with those of other patients with NF1 who also developed MPNST with lung metastases in the literature. The findings suggest the need for further research into personalized treatment strategies that may improve prognosis and overall survival in patients with NF1 and MPNST, with immunotherapy being a promising therapeutic option.

摘要

1型神经纤维瘤病（NF1）显著增加了恶性外周神经鞘瘤（MPNST）的发病风险，MPNST是一种罕见且侵袭性强的恶性肿瘤，其治疗在临床上具有挑战性。本文介绍了一名24岁患有NF1的男性患者，他患上了伴有肺转移的MPNST。由于全身治疗对MPNST的疗效有限，该患者接受了根治性手术切除和放疗。病理评估证实为高级别MPNST，PET-CT成像显示有进一步的转移进展。将我们患者的治疗结果与文献中其他同样患有伴有肺转移的MPNST的NF1患者的结果进行了比较。研究结果表明，需要进一步研究个性化治疗策略，这可能会改善NF1和MPNST患者的预后和总生存期，免疫疗法是一种有前景的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/08cb3c0769de/life-14-01648-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/595538656e33/life-14-01648-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/ee3578d77f08/life-14-01648-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/9f606ea6ab09/life-14-01648-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/46a319ab2c52/life-14-01648-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/2bc1b56e865d/life-14-01648-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/fbc4b080551a/life-14-01648-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/045b4baa5b0a/life-14-01648-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/08cb3c0769de/life-14-01648-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/595538656e33/life-14-01648-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/ee3578d77f08/life-14-01648-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/9f606ea6ab09/life-14-01648-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/46a319ab2c52/life-14-01648-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/2bc1b56e865d/life-14-01648-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/fbc4b080551a/life-14-01648-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/045b4baa5b0a/life-14-01648-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83f7/11680011/08cb3c0769de/life-14-01648-g008.jpg

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