先天性巨结肠症的组织病理学问题与诊断挑战：一项东欧单中心观察性研究
Histopathologic Concerns and Diagnostic Challenges in Hirschsprung's Disease: An Eastern European Single-Center Observational Study.
作者信息
Horváth Emőke, Derzsi Zoltán, Löckli Eliza, Molnár Gyopár-Beáta, Bara Zsolt, Kovács Evelyn, Gozar Horea
机构信息
Department of Pathology, George Emil Palade University of Medicine, Pharmacy, Science and Technology of Targu Mures, 540142 Targu Mures, Romania.
Pathology Service, Targu Mures, County Emergency Clinical Hospital, 540136 Targu Mures, Romania.
出版信息
Life (Basel). 2025 Feb 20;15(3):329. doi: 10.3390/life15030329.
BACKGROUND
We proposed a comprehensive clinicopathological study involving the characterization of the study cohort and a comparative analysis of biopsies and surgical specimens from patients with Hirschsprung's disease. The study was complemented by the diagnostic value of calretinin, CD56, and S-100 immunohistochemistry.
METHODS
Descriptive statistical analysis of diagnostic variables in the group of biopsy specimens (n = 32) and bowel resection specimens (n = 16) was performed. The pattern of calretinin and CD56 expression in Meissner's plexus elements was analyzed and the maximum thicknesses of the nerve fibers were measured using morphometry with S100-immunostained sections.
CONCLUSIONS
Coupled calretinin-CD56 immunohistochemistry is useful in diagnosing ganglion cell paucity biopsies or specimens with incomplete submucosa. In cases where there are no ganglion cells but there are calretinin-positive nerve fibrils in the lamina propria without nerve trunk (NT) hypertrophy, re-biopsy is the best solution. The significant differences in NT size between biopsies and surgical specimens highlight the importance of assessing NT diameter in all tissue samples examined.
背景
我们开展了一项全面的临床病理研究，包括对研究队列的特征描述以及对先天性巨结肠病患者的活检组织和手术标本进行对比分析。本研究通过钙视网膜蛋白、CD56和S-100免疫组织化学的诊断价值得以完善。
方法
对活检标本组（n = 32）和肠切除标本组（n = 16）的诊断变量进行描述性统计分析。分析了迈斯纳神经丛中钙视网膜蛋白和CD56的表达模式，并使用S100免疫染色切片的形态计量学方法测量神经纤维的最大厚度。
结论
联合钙视网膜蛋白-CD56免疫组织化学有助于诊断神经节细胞稀少的活检组织或黏膜下层不完整的标本。在无神经节细胞但固有层有钙视网膜蛋白阳性神经纤维且无神经干（NT）肥大的情况下，再次活检是最佳解决方案。活检标本和手术标本之间NT大小的显著差异凸显了在所有检查的组织样本中评估NT直径的重要性。
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