TY - JOUR
T1 - Clinicopathologic Features of Gastrointestinal Tract Langerhans Cell Histiocytosis
AU - Hu, Shaomin
AU - Graham, Rondell P.
AU - Choi, Won Tak
AU - Wen, Kwun Wah
AU - Putra, Juan
AU - Chen, Wei
AU - Lin, Jingmei
AU - Gonzalez, Ivan A.
AU - Panarelli, Nicole
AU - Liu, Qiang
AU - Zhao, Lei
AU - Gong, Shunyou
AU - Mejia-Bautista, Melissa
AU - Escobar, David J.
AU - Ma, Changqing
AU - Shalaby, Akram
AU - Du, Xiaotang
AU - Kang, Liang I.
AU - Zhang, Wei
AU - Chen, Xiuxu
AU - Ding, Xianzhong
AU - Chen, Hannah H.
AU - Ye, Zhan
AU - Pezhouh, Maryam K.
AU - Liao, Xiaoyan
AU - Liu, Yongjun
AU - Yang, Zhaohai
AU - Alpert, Lindsay
AU - Hart, John
AU - Goldblum, John R.
AU - Allende, Daniela
AU - Zheng, Wei
AU - Gonzalez, Raul S.
AU - Wang, Hanlin L.
AU - Zhang, Xuchen
AU - Liu, Xiuli
AU - Longacre, Teri
AU - Westerhoff, Maria
AU - Xue, Yue
N1 - Publisher Copyright:
© 2024 United States & Canadian Academy of Pathology
PY - 2024/9
Y1 - 2024/9
N2 - 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.
AB - 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.
KW - Langerhans cell histiocytosis
KW - adult
KW - gastrointestinal
KW - pediatric
UR - http://www.scopus.com/inward/record.url?scp=85198114762&partnerID=8YFLogxK
U2 - 10.1016/j.modpat.2024.100543
DO - 10.1016/j.modpat.2024.100543
M3 - Article
C2 - 38897453
AN - SCOPUS:85198114762
SN - 0893-3952
VL - 37
JO - Modern Pathology
JF - Modern Pathology
IS - 9
M1 - 100543
ER -