Clinicopathologic Features of Gastrointestinal Tract Langerhans Cell Histiocytosis

IF 7.1 1区 医学 Q1 PATHOLOGY Modern Pathology Pub Date : 2024-06-17 DOI:10.1016/j.modpat.2024.100543
Shaomin Hu , Rondell P. Graham , Won-Tak Choi , Kwun Wah Wen , Juan Putra , Wei Chen , Jingmei Lin , Ivan A. Gonzalez , Nicole Panarelli , Qiang Liu , Lei Zhao , Shunyou Gong , Melissa Mejia-Bautista , David J. Escobar , Changqing Ma , Akram Shalaby , Xiaotang Du , Liang-I Kang , Wei Zhang , Xiuxu Chen , Yue Xue
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Abstract

Gastrointestinal (GI) tract involvement by Langerhans cell histiocytosis (LCH) is rare and its clinicopathologic characteristics have only been described in case reports and small series. We reviewed hematoxylin and eosin and CD1a, S100, and Langerin immunohistochemical–stained slides from 47 patients with well-documented demographic and clinical findings. Our cases included 8 children and 39 adults, with a mean follow-up of 63 months. All pediatric patients had concurrent multisystem LCH, presented with GI symptoms, and showed nonpolypoid lesions. Seven (88%) showed multifocal GI disease, including 5 with multiple GI organ involvement. All sampled lesions from children exhibited infiltrative growth. More than half had died of the disease or manifested persistent LCH at last follow-up. Twenty-five of 39 (64%) adults had LCH involving only the GI tract (single system), with the remaining 14 (36%) exhibiting multisystem disease. Adult single-system GI LCH was typically encountered incidentally on screening/surveillance endoscopy (72%). Most exhibited isolated colorectal involvement (88%) as a solitary polyp (92%), with a well-demarcated/noninfiltrative growth pattern (70%), and excellent prognosis (100%). In comparison, adult patients with multisystem LCH more frequently presented with GI symptoms (92%, P < .001), noncolorectal GI site involvement (50%, P = .02), multifocal GI lesions (43%, P = .005), nonpolypoid lesions (71%, P < .001), infiltrative histologic growth pattern (78%, P = .04), and persistent disease (57%, P < .001). Adult patients with multisystem LCH appear to exhibit similar clinicopathologic features to those of pediatric patients. These results demonstrated that adults with single-system LCH involving the GI tract have an excellent prognosis, whereas multisystem LCH occurring at any age carries an unfavorable prognosis. High-risk features of GI LCH include pediatric age, GI symptomatology, noncolorectal GI involvement, multifocal GI disease, nonpolypoid lesions, and infiltrative growth pattern.

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胃肠道朗格汉斯细胞组织细胞增生症的临床病理特征。
朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)累及胃肠道(GI)非常罕见,其临床病理特征仅在病例报告和小型系列病例中有所描述。我们回顾了 47 例患者的苏木精、伊红、CD1a、S100 和 Langerin 免疫组化染色切片,这些患者的人口统计学和临床表现均有详细记录。我们的病例包括 8 名儿童和 39 名成人,平均随访时间为 63 个月。所有儿童患者均并发多系统LCH,出现消化道症状,病变呈非息肉状。七名患者(88%)出现多灶性消化道疾病,其中五名患者累及多个消化道器官。所有儿童患者的病变均呈浸润性生长。半数以上的患儿在最后一次随访时死于疾病或表现为持续性LCH。39名成人中有25人(64%)的LCH仅累及消化道(单系统),其余14人(36%)表现为多系统疾病。成人单系统消化道 LCH 通常是在筛查/监测内镜检查中偶然发现的(72%)。大多数患者表现为孤立的结直肠受累(88%),为单发息肉(92%),分界清楚/无浸润生长模式(70%),预后良好(100%)。相比之下,多系统 LCH 成人患者更常出现消化道症状(92%,P<0.05)。
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来源期刊
Modern Pathology
Modern Pathology 医学-病理学
CiteScore
14.30
自引率
2.70%
发文量
174
审稿时长
18 days
期刊介绍: Modern Pathology, an international journal under the ownership of The United States & Canadian Academy of Pathology (USCAP), serves as an authoritative platform for publishing top-tier clinical and translational research studies in pathology. Original manuscripts are the primary focus of Modern Pathology, complemented by impactful editorials, reviews, and practice guidelines covering all facets of precision diagnostics in human pathology. The journal's scope includes advancements in molecular diagnostics and genomic classifications of diseases, breakthroughs in immune-oncology, computational science, applied bioinformatics, and digital pathology.
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