Immunostaining for Hu C/D and CD56 is useful for a definitive histopathological diagnosis of congenital and acquired isolated hypoganglionosis

Koichiro Yoshimaru, Tomoaki Taguchi, Satoshi Obata, Junkichi Takemoto, Yoshiaki Takahashi, Tsuyoshi Iwanaka, Yusuke Yanagi, Masaaki Kuda, Kina Miyoshi, Toshiharu Matsuura, Yoshiaki Kinoshita, Takako Yoshioka, Atsuko Nakazawa, Yoshinao Oda

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)679-685
Number of pages7
JournalVirchows Archiv
Volume470
Issue number6
DOIs
Publication statusPublished - Jun 1 2017

Fingerprint

Hirschsprung Disease
Myenteric Plexus
Ganglia
Staining and Labeling
Hematoxylin
Eosine Yellowish-(YS)
Cell Size
Nerve Fibers
Paraffin
Cell Count

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Molecular Biology
  • Cell Biology

Cite this

Immunostaining for Hu C/D and CD56 is useful for a definitive histopathological diagnosis of congenital and acquired isolated hypoganglionosis. / 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.

In: Virchows Archiv, Vol. 470, No. 6, 01.06.2017, p. 679-685.

Research output: Contribution to journalArticle

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. / Immunostaining for Hu C/D and CD56 is useful for a definitive histopathological diagnosis of congenital and acquired isolated hypoganglionosis. In: Virchows Archiv. 2017 ; Vol. 470, No. 6. pp. 679-685.
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AU - Yoshimaru, Koichiro

AU - Taguchi, Tomoaki

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AU - Takemoto, Junkichi

AU - Takahashi, Yoshiaki

AU - Iwanaka, Tsuyoshi

AU - Yanagi, Yusuke

AU - Kuda, Masaaki

AU - Miyoshi, Kina

AU - Matsuura, Toshiharu

AU - Kinoshita, Yoshiaki

AU - Yoshioka, Takako

AU - Nakazawa, Atsuko

AU - Oda, Yoshinao

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N2 - 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.

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