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Retroperitoneal cystic lymphangioma
Other Resources UpToDate PubMed

Retroperitoneal cystic lymphangioma

Contributors: Michael W. Winter MD, Paritosh Prasad MD
Other Resources UpToDate PubMed


Lymphangiomas are rare benign cystic tumors caused by uncontrolled lymphatic growth. Most lymphangiomas arise in the axilla or neck. In approximately 1% of cases, lymphangiomas develop in the retroperitoneal space, characterized as a retroperitoneal cystic lymphangioma.

These tumors can be asymptomatic, but often, due to their size and location, patients will present with a palpable abdominal mass, bowel obstruction, abdominal pain, anorexia, weight loss, ascites, or obstructive nephropathy from ureter compression. Asymptomatic cases can be found incidentally during surgery or autopsy.

The diagnosis of a cystic lymphangioma typically requires pathology, as the appearance of a complex cyst on cross-sectional imaging is not specific to these types of tumors.


D18.1 – Lymphangioma, any site

40225001 – Cystic lymphangioma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Cystic tumor, retroperitoneum:
  • Cystic mesothelioma
  • Teratoma
  • Sarcoma
  • Metastases (gastric or ovarian primary malignancy)
  • Malignant mesenchymoma
  • Urothelial or foregut complex or simple cysts
  • Microcystic pancreatic adenoma
  • Retroperitoneal hematoma
  • Duplication cyst
  • Ovarian cyst
  • Pancreatic pseudocyst
  • Müllerian cyst
  • Epidermoid cyst
  • Tailgut cyst
  • Paraganglioma
  • Neuroblastoma
  • Retroperitoneal pseudomyxoma
  • Lymphocele
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Last Reviewed:03/06/2018
Last Updated:04/12/2018
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Retroperitoneal cystic lymphangioma
A medical illustration showing key findings of Retroperitoneal cystic lymphangioma : Diarrhea, Fever, Nausea, Abdominal distension, Epigastric pain, Anorexia, LUQ pain, Palpable abdominal mass
Copyright © 2023 VisualDx®. All rights reserved.