Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 26, 2021; 9(15): 3758-3764
Published online May 26, 2021. doi: 10.12998/wjcc.v9.i15.3758
Lymphangiomatosis associated with protein losing enteropathy: A case report
Xue-Li Ding, Xiao-Yan Yin, Ya-Nan Yu, Yun-Qing Chen, Wei-Wei Fu, Hua Liu
Xue-Li Ding, Xiao-Yan Yin, Ya-Nan Yu, Hua Liu, Department of Gastroenterology, The Affiliated Hospital of Qingdao University, Qingdao 266003, Shandong Province, China
Yun-Qing Chen, Wei-Wei Fu, Department of Pathology, The Affiliated Hospital of Qingdao University, Qingdao 266003, Shandong Province, China
Author contributions: Ding XL reviewed the literature and contributed to manuscript drafting; Yin XY was the gastroenterologist; Yu YN revised the manuscript to improve its academic merits; Chen YQ and Fu WW contributed histopathology and immunohistochemistry expertise; Liu H revised the article and conceived the study; all authors issued final approval for the version to be submitted.
Supported by The National Natural Science Foundation of China, No. 81502025; China Postdoctoral Science Foundation, No. 2018M632631.
Informed consent statement: Written informed consent was obtained from the patient and his son (delegator) for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest to report.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Hua Liu, MD, Associate Chief Physician, Department of Gastroenterology, The Affiliated Hospital of Qingdao University, No. 16 Jiangsu Road, Qingdao 266003, Shandong Province, China. kaixing1407@sina.com
Received: January 9, 2021
Peer-review started: January 10, 2021
First decision: February 12, 2021
Revised: February 13, 2021
Accepted: March 8, 2021
Article in press: March 8, 2021
Published online: May 26, 2021
Processing time: 122 Days and 7.4 Hours
Abstract
BACKGROUND

Lymphangiomatosis is a multisystem disorder that is rarely localized to the gastrointestinal tract. Lymphangiomatosis usually has no specific clinical presentation and is easily misdiagnosed. A case report and review of the literature on lymphangiomatosis associated with protein-losing enteropathy will help to improve the overall understanding of this disease.

CASE SUMMARY

We report a case of lymphangiomatosis of the bowel and other solid organs. A 78-year-old man presented with recurrent bowel bleeding and protein-losing enteropathy, as well as cystic lesions in the spleen, liver, and kidney. Imaging examinations revealed many cystic lesions on the spleen, liver, kidney, and thickened wall of the ascending colon, as well as pleural effusion and ascites. Colonoscopy revealed a strawberry mucosa, variable spontaneous bleeding, and surface erosion located in the terminal ileum. Several cystic masses with a translucent and smooth surface as well as diffuse white spots were located in the colon. A laterally spreading tumor (LST) was located in the ascending colon. Pathology indicated highly differentiated adenocarcinoma (LST) and lymphangiomatoid dilation, and D2-40 was positive. The final diagnosis was lymphangiomatosis. The patient underwent surgery for LST and then was administered thalidomide 75-150 mg/d. His condition, however, did not improve. He eventually died 6 mo after the initial diagnosis.

CONCLUSION

Lymphangiomatosis usually occurs diffusely and can involve many organs, such as the spleen, kidney, liver, lung, mesentery, and bowel. Recurrent bowel bleeding or protein-losing enteropathy is an important indicator that should alert clinicians about the possibility of this disease when it afflicts the bowel. Doctors should improve the medical understanding of lymphangiomatosis.

Keywords: Lymphangioma; Gastrointestinal hemorrhage; Protein-losing enteropathies; Colonic neoplasms; Small intestine; Case report

Core Tip: Lymphangiomatosis is a multisystem disorder that is rarely localized to the gastrointestinal tract. We report a case of lymphangiomatosis of the bowel and other solid organs (spleen, liver, and kidney). The condition was misdiagnosed because it did not present specific clinical indicators, and the treating doctors were not aware of this condition. After a series of imaging and pathologic examinations, the patient was correctly diagnosed and treated with thalidomide. Follow-up indicated that he died 6 mo after the initial diagnosis. This case emphasizes the importance of improving the understanding of lymphangiomatosis.