Yoshimaru Koichiro, Taguchi Tomoaki, Obata Satoshi, Takemoto Junkichi, Takahashi Yoshiaki, Iwanaka Tsuyoshi, Yanagi Yusuke, Kuda Masaaki, Miyoshi Kina, Matsuura Toshiharu, Kinoshita Yoshiaki, Yoshioka Takako, Nakazawa Atsuko, Oda Yoshinao
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Department of Pathology, National Center for Child Health and Development, Setagaya, Japan.
Virchows Arch. 2017 Jun;470(6):679-685. doi: 10.1007/s00428-017-2128-9. Epub 2017 Apr 19.
Isolated hypoganglionosis (IHG) has been proposed as a distinct entity with two subtypes: congenital IHG (CIHG) and acquired IHG (AIHG). However, due to the rarity of the disease and the lack of defining histological criteria, the concept of IHG is not widely accepted. We studied paraffin-embedded intestinal specimens from 79 patients diagnosed with Hirschsprung's disease (HD) (n = 49), CIHG (n = 25), and AIHG (n = 5) collected between January 1996 and December 2015. Histopathological diagnosis of HD, CIHG, and AIHG was confirmed by hematoxylin and eosin staining and immunohistochemical staining using Hu C/D and CD56. We evaluated (immuno)histopathological findings, counted the number of ganglion cells, and measured the size of Auerbach's plexus. Hu C/D labeled neuronal cell bodies, whereas CD56 was detected in all neuronal components. In HD, all ganglion cells in Auerbach's plexus in the normoganglionic segment (NGS) were immunoreactive for Hu C/D, whereas in the aganglionic segment (AGS), these were replaced by CD56-positive extrinsic nerve fibers and bundles. The number of ganglion cells in AIHG and CIHG was significantly lower than in the NGS of HD (p < 0.05). Auerbach's plexus was significantly smaller in CIHG (p < 0.05) but in AIHG equivalent to the NGS in HD. In summary, immunostaining for Hu C/D and CD56 is useful for definitive histopathological diagnosis of IHG.
孤立性神经节减少症（IHG）已被提出作为一种独特的疾病实体，有两种亚型：先天性IHG（CIHG）和后天性IHG（AIHG）。然而，由于该疾病罕见且缺乏明确的组织学标准，IHG的概念尚未被广泛接受。我们研究了1996年1月至2015年12月期间收集的79例诊断为先天性巨结肠（HD）（n = 49）、CIHG（n = 25）和AIHG（n = 5）患者的石蜡包埋肠标本。HD、CIHG和AIHG的组织病理学诊断通过苏木精和伊红染色以及使用Hu C/D和CD56的免疫组织化学染色得以证实。我们评估了（免疫）组织病理学发现，计数了神经节细胞数量，并测量了奥尔巴赫神经丛的大小。Hu C/D标记神经元细胞体，而CD56在所有神经元成分中均有检测到。在HD中，正常神经节段（NGS）的奥尔巴赫神经丛中的所有神经节细胞对Hu C/D呈免疫反应性，而在无神经节段（AGS）中，这些细胞被CD56阳性的外在神经纤维和束所取代。AIHG和CIHG中的神经节细胞数量显著低于HD的NGS中的神经节细胞数量（p < 0.05）。CIHG中的奥尔巴赫神经丛明显较小（p < 0.05），但AIHG中的与HD的NGS相当。总之，Hu C/D和CD56的免疫染色有助于IHG的明确组织病理学诊断。
