ORIGINAL RESEARCH article
Front. Surg.
Sec. Visceral Surgery
This article is part of the Research TopicAdvancing Surgical Outcomes for Retroperitoneal TumorsView all 14 articles
Clinical Features and Outcomes of Adult Primary Retroperitoneal Lymphangioma: Insights from a High-Volume Sarcoma Center
Provisionally accepted
Wenjie Li1
Mengmeng Xiao2Lei Liu1Haining Zheng1Haicheng Gao1Boyuan Zou1Dehu Lu1Mei Huang1Chengli Miao1*- 1Peking University International Hospital, Peking, China
- 2Peking University People's Hospital, Beijing, China
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Background: Adult primary retroperitoneal lymphangioma (RPL) is an exceptionally rare benign vascular malformation. This study aims to elucidate the clinical presentation, imaging characteristics, management strategies, and long-term outcomes of this condition. Methods: We conducted a retrospective analysis of our prospectively maintained retroperitoneal tumor database at Peking University International Hospital (2014-2024). Inclusion criteria comprised adults (≥18 years) undergoing initial surgical resection with pathological RPL confirmation. Patients with other malignancies or recurrent disease were excluded. Comprehensive follow-up was performed to assess outcomes. Results: Among 28 enrolled patients (13 males, 15 females; median age 31 years), clinical presentation included incidental discovery (42.9%), abdominal discomfort (42.9%), lumbago (10.7%), and lower limb pain (3.6%). Preoperative imaging assessment was performed using Ultrasonography (US) (82.1%), computed tomography (CT, 100.0%), and magnetic resonance imaging (MRI, 14.3%). Characteristic CT findings typically included thin-walled multiloculated cystic masses with septal enhancement.All patients achieved R0/R1 resection, with 18 open and 10 laparoscopic procedures (2 conversions). Major complications occurred in 10.7% of cases (lymphatic leakage: 2; pancreatic fistula: 1), all resolving with appropriate management. Histopathological and immunohistochemical analysis confirmed lymphatic differentiation (D2-40: 100%; CD31: 89.3%; CD34: 84.6%). During median follow-up of 76 months, no recurrences or disease-specific mortality were observed. Conclusions: RPL represents a rare benign tumor frequently presenting with nonspecific symptoms. Complete surgical resection demonstrates excellent safety and long-term efficacy, with individualized approach selection based on tumor characteristics. Our findings from this substantial Chinese adult cohort provide valuable insights for managing this uncommon condition.
Keywords: Adult primary retroperitoneal lymphangioma, Imaging Manifestation, laparoscopic surgery, prognosis, surgicaltreatment
Received: 03 Dec 2025; Accepted: 26 Jan 2026.
Copyright: © 2026 Li, Xiao, Liu, Zheng, Gao, Zou, Lu, Huang and Miao. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Chengli Miao
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