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Rapid Malignant Transformation of a Biopsy Proven Sporadic Soft Tissue Schwannoma to Spindle Cell MPNST With Mutation as an Early Event.

作者信息

Ramineni Madhurya, Ulici Veronica, Hicks David G, Huber Aaron R, Dieudonne Gregory, Wang Xi

机构信息

Department of Pathology, University of Rochester Medical Center, Rochester, NY, USA.

出版信息

Int J Surg Pathol. 2025 Dec;33(8):1807-1815. doi: 10.1177/10668969251340430. Epub 2025 May 23.

DOI:10.1177/10668969251340430
PMID:40405809
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12446714/
Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) primarily originate from the neurofibromatosis 1 (NF1)- associated and/or sporadic neurofibromas. Reports of malignant transformation from a sporadic soft tissue schwannoma are rare. In most tumors documented in this context so far, the malignant component is an epithelioid MPNST with a strong and diffuse S100 positivity. We present a patient diagnosed with a sporadic soft tissue schwannoma on core biopsy, which underwent rapid malignant transformation to a spindle cell type MPNST. Somatic tumor profiling of the excision specimen revealed loss, , , and subclonal gene mutations. A retrospective immunohistochemical stain for p53 on the core biopsy demonstrated an aberrant staining pattern, indicating an early effect of in the process of malignant transformation.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a25c/12446714/f5b8f4f707ef/10.1177_10668969251340430-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a25c/12446714/c3284604f644/10.1177_10668969251340430-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a25c/12446714/11c4e840897e/10.1177_10668969251340430-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a25c/12446714/278edf802239/10.1177_10668969251340430-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a25c/12446714/f5b8f4f707ef/10.1177_10668969251340430-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a25c/12446714/c3284604f644/10.1177_10668969251340430-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a25c/12446714/11c4e840897e/10.1177_10668969251340430-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a25c/12446714/278edf802239/10.1177_10668969251340430-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a25c/12446714/f5b8f4f707ef/10.1177_10668969251340430-fig4.jpg

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本文引用的文献

1
Malignant transformation of vestibular schwannoma following radiosurgery-a case report and review of the literature.听神经鞘瘤放射手术后恶变：病例报告及文献复习。
Acta Neurochir (Wien). 2024 Jan 30;166(1):52. doi: 10.1007/s00701-024-05921-6.
2
Functional interactions between neurofibromatosis tumor suppressors underlie Schwann cell tumor de-differentiation and treatment resistance.神经纤维瘤病肿瘤抑制因子之间的功能相互作用是施万细胞瘤去分化和治疗耐药的基础。
Nat Commun. 2024 Jan 12;15(1):477. doi: 10.1038/s41467-024-44755-9.
3
Prevalence and detection of actionable BRAF V600 and NRAS Q61 mutations in malignant peripheral nerve sheath tumor by droplet digital PCR.
应用液滴数字 PCR 技术检测恶性外周神经鞘瘤中 BRAF V600 和 NRAS Q61 突变及其临床意义
Hum Pathol. 2022 Nov;129:90-97. doi: 10.1016/j.humpath.2022.08.005. Epub 2022 Sep 5.
4
Practical Approach to Histological Diagnosis of Peripheral Nerve Sheath Tumors: An Update.外周神经鞘瘤组织学诊断的实用方法：最新进展
Diagnostics (Basel). 2022 Jun 14;12(6):1463. doi: 10.3390/diagnostics12061463.
5
Clinical sequencing of soft tissue and bone sarcomas delineates diverse genomic landscapes and potential therapeutic targets.软组织和骨肉瘤的临床测序描绘了不同的基因组图谱和潜在的治疗靶点。
Nat Commun. 2022 Jun 15;13(1):3405. doi: 10.1038/s41467-022-30453-x.
6
Transition of a vestibular schwannoma to a malignant peripheral nerve sheath tumor with loss of H3K27 trimethylation after radiosurgery-a case report and review of the literature.听神经瘤经放射外科手术后转变为H3K27三甲基化缺失的恶性外周神经鞘瘤——病例报告及文献复习
Neurosurg Rev. 2022 Feb;45(1):915-922. doi: 10.1007/s10143-021-01620-3. Epub 2021 Aug 15.
7
Epithelioid malignant peripheral nerve sheath tumor arising in schwannoma.起源于神经鞘瘤的上皮样恶性外周神经鞘膜瘤。
Rare Tumors. 2020 Aug 21;12:2036361320950862. doi: 10.1177/2036361320950862. eCollection 2020.
8
Recurrent SMARCB1 Inactivation in Epithelioid Malignant Peripheral Nerve Sheath Tumors.上皮样恶性外周神经鞘瘤中 SMARCB1 反复失活。
Am J Surg Pathol. 2019 Jun;43(6):835-843. doi: 10.1097/PAS.0000000000001242.
9
Inferior survival for patients with malignant peripheral nerve sheath tumors defined by aberrant TP53.异常 TP53 定义的恶性外周神经鞘瘤患者的生存预后较差。
Mod Pathol. 2018 Nov;31(11):1694-1707. doi: 10.1038/s41379-018-0074-y. Epub 2018 Jun 26.
10
Loss of H3K27 trimethylation is not suitable for distinguishing malignant peripheral nerve sheath tumor from melanoma: a study of 387 cases including mimicking lesions.H3K27 三甲基化缺失并不适用于鉴别恶性外周神经鞘瘤和黑色素瘤：一项包括模拟病变的 387 例研究。
Mod Pathol. 2017 Dec;30(12):1677-1687. doi: 10.1038/modpathol.2017.91. Epub 2017 Jul 28.