神经纤维瘤病 1 型患者的周围神经鞘瘤：形态学和免疫组织化学研究。
Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Morphological and Immunohistochemical Study.
机构信息
Department of Oral and Craniomaxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany;
Institute of Neuropathology, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany.
出版信息
Anticancer Res. 2022 Mar;42(3):1247-1261. doi: 10.21873/anticanres.15592.
BACKGROUND/AIM: The aim of the present investigation was to characterize the growth pattern and antigen profile of peripheral nerve sheath tumors (PNST) in a large series of tumors obtained from patients with Neurofibromatosis type 1 (NF1) focusing on morphological characteristics of diffuse plexiform neurofibroma (DPNF).
MATERIALS AND METHODS
Tissue micro-array (TMA) analysis was applied to study 520 formalin-fixed, paraffin-embedded human PNST of 385 patients with confirmed NF1 diagnosis. PNST originated from all areas of the body and were classified as cutaneous neurofibroma (CNF, n=114), diffuse neurofibroma (DNF, n=109), DPNF (n=108), plexiform neurofibroma (PNF, n=110), and malignant peripheral nerve sheath tumor (MPNST, n=22). Histomorphology and antigen expression patterns of the tumors were determined [S100, epithelial membrane antigen (EMA), CD90, mast cell tryptase, and neurofilament].
RESULTS
Benign PNST showed significantly more S100-positive tumor cells than MPNST (p<0.001). EMA expression was most pronounced in perineurium of DPNF. The number of mast cells in CNF, DNF and DPNF was significantly higher compared to PNF and MPNST (p<0.001 for both comparisons, Mann-Whitney U-test).
CONCLUSION
DPNF show some distinct cellular characteristics. A high number of EMA positive cells possibly indicates the dissemination of perineural cells to the surrounding tissue. Concerning mast cell density, DPNF resemble DNF and CNS rather than PNF. Close contact of tumor cells in DPNF, DNF and CNF with the immune system is a prerequisite for permanent immunological reactions in contrast to PNF in which tumor cells are partitioned from the immune system by the perineurium and blood-nerve barrier of blood vessels. It is assumed that these morphological distinctions may reflect in part the biological differences between the entities. While PNF is a known precancerous stage in NF1 patients, DPNF are not rated as such. Furthermore, the morphologic differences between benign nerve sheath tumors may be important for the efficacy of drugs to access tumor cells.
背景/目的：本研究的目的是描述神经纤维瘤病 1 型（NF1）患者中大量外周神经鞘瘤（PNST）的生长模式和抗原谱，重点研究弥漫性丛状神经纤维瘤（DPNF）的形态学特征。
材料和方法
应用组织微阵列（TMA）分析研究了 385 例 NF1 确诊患者的 520 例福尔马林固定、石蜡包埋的人 PNST。PNST 起源于身体的所有部位，分为皮肤神经纤维瘤（CNF，n=114）、弥漫性神经纤维瘤（DNF，n=109）、DPNF（n=108）、丛状神经纤维瘤（PNF，n=110）和恶性外周神经鞘瘤（MPNST，n=22）。确定肿瘤的组织形态学和抗原表达模式[S100、上皮膜抗原（EMA）、CD90、肥大细胞胰蛋白酶和神经丝]。
结果
良性 PNST 的 S100 阳性肿瘤细胞明显多于 MPNST（p<0.001）。DPNF 的神经外膜表达最明显。CNF、DNF 和 DPNF 的肥大细胞数量明显高于 PNF 和 MPNST（两种比较均 p<0.001，Mann-Whitney U 检验）。
结论
DPNF 具有一些独特的细胞特征。大量 EMA 阳性细胞可能表明周围神经细胞的扩散到周围组织。关于肥大细胞密度，DPNF 与 DNF 和 CNS 相似，而与 PNF 不同。DPNF、DNF 和 CNF 中肿瘤细胞与免疫系统的密切接触是产生永久性免疫反应的前提，而 PNF 中肿瘤细胞被神经外膜和血管的血神经屏障与免疫系统隔开。可以假设这些形态学差异部分反映了实体之间的生物学差异。虽然 PNF 是 NF1 患者的已知癌前阶段，但 DPNF 并未被归类为癌前阶段。此外，良性神经鞘瘤之间的形态差异对于药物接触肿瘤细胞的疗效可能很重要。
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