Department of Pathology, Botucatu School of Medicine, São Paulo State University (Unesp), Botucatu, Brazil.
Department of Surgery, Division of Pediatric Surgery, Botucatu School of Medicine, São Paulo State University (Unesp), Botucatu, Brazil.
Mod Pathol. 2017 Jul;30(7):978-985. doi: 10.1038/modpathol.2017.4. Epub 2017 Mar 17.
Intestinal neuronal dysplasia type B is a controversial entity expressed by complex changes in the enteric nervous system. Diagnosis depends on rectal biopsy histopathology and diagnostic criteria, both qualitative and quantitative, have changed over time, hindering the diagnostic practice. We analyzed the morphological criteria for the histological diagnosis of intestinal neuronal dysplasia type B in a series of patients with intestinal neuronal dysplasia type B according to the 1990 Frankfurt Consensus criteria and verified the applicability of the numerical criteria proposed by Meier-Ruge et al in 2004 and 2006. Qualitative criteria adopted for the histological diagnosis of intestinal neuronal dysplasia type B included hyperplasia of the submucous plexus with hyperganglionosis and hypertrophy of the nerve trunks. Quantitative criteria considered more than 20% giant ganglia in the submucosa, with more than eight neurons each on 25 ganglia, and children aged over 1 year. Distal colon surgical specimens from 29 patients, aged 0-16 years, diagnosed with intestinal neuronal dysplasia type B were retrospectively analyzed using sections processed for conventional histology (H&E) and calretinin immunohistochemistry. Hyperplasia of the submucosal nerve plexi with hyperganglionosis and hypertrophy of the nerve trunks was observed in all cases. Ganglia with small, immature neurons were detected in the majority of cases. Quantitative analysis confirmed hyperganglionosis (mean number=10.7 neurons per ganglion) and hypertrophy of the nerve trunks (median=44.6 μm thickness). Neurons showed immunostaining for calretinin, but neuron counts in calretinin-stained sections were lower compared with H&E (P<0.01). No significant differences were verified between children aged under and over 1 year regarding hyperganglionosis (P=0.79), neuron counts (P=0.36), and immature ganglia (P=0.66). Only one patient met the numerical criteria proposed by Meier-Ruge et al in 2004 and 2006. In conclusion, the numerical criteria showed limited applicability when transposed to conventional histopathology. Children aged over 1 year presented very similar histological features of neuronal immaturity to younger children, questioning the need for an age criterion when diagnosing intestinal neuronal dysplasia type B.
肠神经元发育不良 B 型是一种有争议的实体，其肠神经系统表现出复杂的变化。诊断取决于直肠活检的组织病理学，并且随着时间的推移，定性和定量的诊断标准都发生了变化，这阻碍了诊断实践。我们根据 1990 年法兰克福共识标准分析了一系列肠神经元发育不良 B 型患者的肠神经元发育不良 B 型组织学诊断的形态学标准，并验证了 Meier-Ruge 等人在 2004 年和 2006 年提出的数值标准的适用性。用于肠神经元发育不良 B 型组织学诊断的定性标准包括黏膜下神经丛的增生、神经节的过度形成和神经干的肥大。定量标准考虑了黏膜下超过 20%的巨神经节，每个神经节有超过 8 个神经元，儿童年龄超过 1 岁。回顾性分析了 29 例年龄 0-16 岁的肠神经元发育不良 B 型患者的远端结肠手术标本，这些标本均经常规组织学（H&E）和钙视网膜蛋白免疫组织化学处理。所有病例均观察到黏膜下神经丛的增生、神经节的过度形成和神经干的肥大。大多数病例均检测到小而不成熟的神经元神经节。定量分析证实了神经节的过度形成（每个神经节平均 10.7 个神经元）和神经干的肥大（中位数为 44.6 μm 厚）。神经元对钙视网膜蛋白有免疫染色，但钙视网膜蛋白染色切片中的神经元计数低于 H&E（P<0.01）。年龄小于和大于 1 岁的儿童在神经节过度形成（P=0.79）、神经元计数（P=0.36）和不成熟神经节（P=0.66）方面无显著差异。只有 1 名患者符合 Meier-Ruge 等人在 2004 年和 2006 年提出的数值标准。总之，数值标准在转化为常规组织病理学时适用性有限。年龄大于 1 岁的儿童与年龄较小的儿童具有非常相似的神经元不成熟组织学特征，这使得在诊断肠神经元发育不良 B 型时对年龄标准产生了质疑。
