Review of diagnosis, differential diagnosis, and management of retroperitoneal lymphangioma

Jpn J Radiol. 2023 Mar;41(3):283-301. doi: 10.1007/s11604-022-01356-0. Epub 2022 Nov 3.

Abstract

Lymphatic malformation (LM) is the currently preferred term for what was previously known as lymphangioma. Retroperitoneal LMs are extremely rare, benign, cystic masses that arise from lymphatic vessels. They can be challenging to diagnose because they resemble other retroperitoneal cystic tumors. The development of treatment strategies for rare diseases, including retroperitoneal LM, requires the acquisition of new knowledge to enhance our understanding of the disease progression. Therefore, we present an update regarding fundamental and advanced issues associated with retroperitoneal LM. This review describes the epidemiology, histopathology, biomedicine, clinical manifestations, radiological features, differential diagnosis, and management of this lesion.

Keywords: Abdominal masses; Cystic lesion; Lymphatic malformation; Retroperitoneal lymphangioma; Vascular malformations.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Humans
  • Lymphangioma* / diagnostic imaging
  • Lymphangioma* / therapy
  • Lymphangioma, Cystic* / diagnostic imaging
  • Lymphangioma, Cystic* / surgery
  • Lymphatic Abnormalities*
  • Retroperitoneal Neoplasms* / diagnostic imaging
  • Retroperitoneal Neoplasms* / therapy
  • Retroperitoneal Space / diagnostic imaging
  • Retroperitoneal Space / pathology