Martin Enrico, Acem Ibtissam, Grünhagen Dirk J, Bovée Judith V M G, Verhoef Cornelis
Department of Surgical Oncology, Erasmus Medical Center, Rotterdam, Netherlands.
Department of Plastic and Reconstructive Surgery, University Medical Center Utrecht, Utrecht, Netherlands.
Front Oncol. 2020 Dec 22;10:594069. doi: 10.3389/fonc.2020.594069. eCollection 2020.
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with dismal prognosis. Pathological and genetic markers may predict more aggressive behavior in MPNSTs but have uncommonly been investigated, and few are used in daily practice. This study reviews the prognostic value of immunohistochemical markers and genetic alterations in MPNST.
A systematic search was performed in PubMed and Embase databases according to the PRISMA guidelines. Search terms related to 'MPNST' and 'prognostic' were used. Studies investigating the association of immunohistochemical markers or genetic alterations with prognosis were included. Qualitative synthesis was performed on all studies. A distinction was made between univariable and multivariable associations.
Forty-six studies were included after full-text screening. Sixty-seven different immunohistochemical markers were investigated. Absence of S100 and H3K27me3 and high Ki67 and p53 staining was most commonly independently associated with worse survival and disease-free survival. Several genetic alterations were investigated as well with varying association to survival. , alterations were independently associated with worse survival, as well as changes in chromosomal length in Xp, 10q, and 16p.
MPNSTs harbor complex and heterogeneous biology. Immunohistochemical markers and genetic alterations have variable prognostic value. Absence of S100 and H3K27me3 and increased Ki67 can be of prognostic value. Alterations in or increase in p53 staining may distinguish MPNSTs with worse outcomes. Genetic alterations and staining of other cell cycle regulatory and Ras pathway proteins may also help stratifying patients with worse outcomes. A combination of markers can increase the prognostic value.
恶性外周神经鞘瘤（MPNSTs）是侵袭性软组织肉瘤，预后不佳。病理和基因标志物可能预测MPNSTs更具侵袭性的行为，但很少被研究，且在日常实践中很少使用。本研究回顾了免疫组化标志物和基因改变在MPNST中的预后价值。
根据PRISMA指南在PubMed和Embase数据库中进行系统检索。使用与“MPNST”和“预后”相关的检索词。纳入研究免疫组化标志物或基因改变与预后关联的研究。对所有研究进行定性综合分析。区分单变量和多变量关联。
全文筛选后纳入46项研究。研究了67种不同的免疫组化标志物。S100和H3K27me3缺失以及Ki67和p53高染色最常独立与较差的生存率和无病生存率相关。还研究了几种基因改变及其与生存的不同关联。 改变独立与较差的生存率相关，以及Xp、10q和16p染色体长度的变化。
MPNSTs具有复杂和异质性生物学特性。免疫组化标志物和基因改变具有可变的预后价值。S100和H3K27me3缺失以及Ki67增加可能具有预后价值。 改变或p53染色增加可能区分预后较差的MPNSTs。其他细胞周期调节和Ras通路蛋白的基因改变和染色也可能有助于对预后较差的患者进行分层。标志物组合可增加预后价值。
