重新评估神经纤维瘤/分裂瘤混合瘤:尽管 CD34 阳性且最初诊断为神经纤维瘤,但分裂瘤特征仍占主导地位

IF 2.9 3区 医学 Q2 DERMATOLOGY Journal of Dermatology Pub Date : 2024-06-22 DOI:10.1111/1346-8138.17343
Tatsuya Katsumi, Ryota Hayashi, Shingo Takei, Osamu Ansai, Sumiko Takatsuka, Tatsuya Takenouchi, Kyota Saito, Kazuaki Suda, Kosuke Yoshihara, Takahiro Nagai, Shujiro Okuda, Takaya Fukumoto, Shin-ichi Ansai, Anna Nakamura, Koji Katsuumi, Takashi Ariizumi, Akira Ogose, Hiroyuki Kawashima, Riichiro Abe
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引用次数: 0

摘要

组织病理学结果显示,许旺瘤由高细胞区(安东尼 A 区)和低细胞区(安东尼 B 区)组成。神经纤维瘤的特征是由 CD34 阳性的纺锤形细胞组成,细胞核薄,呈波浪状,并有波浪状胶原带。以前的报告将具有神经纤维瘤特征的片段分裂瘤描述为混合瘤,尽管在以前的许多报告中,混合瘤是根据部分 CD34 阳性来诊断的。另一方面,有报道称一些精神分裂瘤的 Antoni B 区 CD34 呈阳性。因此,混合瘤的定义一直不明确。本研究的目的是确定是否仅能用分裂瘤的 CD34 阳性结果来定义混合瘤。在对我们这位由新型 LZTR1 基因突变引起的分裂瘤患者的分析中,部分肿瘤有 CD34 阳性的低细胞区。这些区域没有细长的波浪状核,表明是安东尼 B 区。研究人员利用激光显微切割技术研究了 CD34 阳性区域和 CD34 阴性区域的遗传背景和分子机制差异。在 CD34 阳性区域发现的所有突变在 CD34 阴性区域也同样存在。我们的数据无法明确 CD34 阳性区域的 Antoni B 的遗传背景。随后,我们对 66 例散发性裂头瘤的病理进行了回顾。所有裂隙瘤的组织病理学检查均显示没有细长的波浪状细胞核和波浪状胶原带,也没有发现混合瘤。我们随机抽取了 66 例患者中的 10 例进行 CD34 免疫染色,结果发现所有病例均呈阳性。我们的数据表明,仅靠 CD34 染色很难区分裂隙瘤,因为安东妮 B 区的 CD34 也可能呈阳性。因此,不应仅用 CD34 染色来诊断分裂瘤患者的混合瘤。
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Reevaluating hybrid neurofibroma/schwannoma: Predominance of schwannoma features despite CD34 positivity and initial neurofibroma diagnosis

Schwannomas consist of both high-cellularity regions (Antoni A area) and hypocellular regions (Antoni B area) in histopathological findings. Neurofibromas characteristically consist of CD34 positive spindle cells with thin, wavy, nuclei and wavy collagen bands. Previous reports have described segments of schwannomas with neurofibroma features as hybrid tumors, although hybrid tumors were diagnosed based on partial CD34 positivity in many previous reports. On the other hand, the Antoni B area of some schwannomas was reported to be positive for CD34. Therefore, the definition of a hybrid tumor has not been clear. The objective of this study was to determine whether only CD34 positive findings in schwannomas could be used to define a hybrid tumor. In the analysis of our patient with schwannomatosis caused by a novel LZTR1 germline mutation, part of the tumor had CD34 positive hypocellular regions. These regions contained no thin, wavy, nuclei, indicating an Antoni B area. Laser microdissection was used to investigate the genetic background and differences in molecular mechanisms between CD34 positive and CD34 negative regions. All mutations identified in CD34 positive regions were also found in CD34 negative regions. Our data could not clear the genetic background of Antoni B which was CD34 positive area. We then reviewed the pathologies of 66 sporadic schwannomas. Histopathological examinations of all schwannomas revealed the absence of thin, wavy, nuclei and wavy collagen bands, and no hybrid tumors were found in any of the cases. Ten of 66 patients were randomly selected for CD34 immunostaining and positivity was found in all cases. Our data suggest that it is difficult to distinguish schwannomas by staining for CD34 alone, as Antoni B areas can also be positive for CD34. Therefore, CD34 staining alone should not be used to diagnose hybrid tumors in patients with schwannomas.

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来源期刊
Journal of Dermatology
Journal of Dermatology 医学-皮肤病学
CiteScore
4.60
自引率
9.70%
发文量
368
审稿时长
4-8 weeks
期刊介绍: The Journal of Dermatology is the official peer-reviewed publication of the Japanese Dermatological Association and the Asian Dermatological Association. The journal aims to provide a forum for the exchange of information about new and significant research in dermatology and to promote the discipline of dermatology in Japan and throughout the world. Research articles are supplemented by reviews, theoretical articles, special features, commentaries, book reviews and proceedings of workshops and conferences. Preliminary or short reports and letters to the editor of two printed pages or less will be published as soon as possible. Papers in all fields of dermatology will be considered.
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