Retroperitoneal Lymphangioma

  • Hai Liu
  • Cheng-Hua Luo


Lymphangioma is developed from original lymphatic sac and vessel in mesenchymal tissue. It is tumor-like malformation initiated after the original sac in some parts is isolated from lymphatic system during embryonic development. As a congenital benign hamartoma, it is divided into three subtypes: simple lymphangioma, cavernous lymphangioma, and cystic lymphangioma. 75% of these lesions occur in neck, 20% in auxiliary region, and only 5% in abdominal cavity. Retroperitoneal lymphangioma accounts for less than 1% of all lymphangiomas (Bhavsar et al. 2010). More than 90% of cases occur in people aged ≤2 years. The prevalence is similar between men and women. Localized lymphangioma has a good prognosis after surgery, with a low relapse rate. Diffuse lymphangioma is usually ill-defined, cannot be completely removed, and relapses repeatedly. Elephantiasis of limbs poorly responds to the treatment. Notably, lymphangioma will not become malignant.


  1. Bhavsar T, Saeed-Vafa D, Harbison S. Retroperitoneal cystic lymphangioma in an adult: a case report and review of the literature. World J Gastrointest Pathophysiol. 2010;1(5):171–6.CrossRefPubMedPubMedCentralGoogle Scholar
  2. Bullard Dunn K. Retrorectal tumors. Surg Clin North Am. 2010;90(1):163–71.CrossRefPubMedPubMedCentralGoogle Scholar
  3. Chalian H, Töre HG, Miller FH. CT attenuation of unilocular pancreatic cystic lesions to differentiate pseudocysts from mucincontaining cysts. JOP. 2011;12(4):384–8.PubMedPubMedCentralGoogle Scholar
  4. Kasza J, Brody FJ, Khambaty F. Laparoscopic resection of a retroperitoneal cystic lymphangioma in an adult. Surg Laparosc Endosc Percutan Tech. 2010;20(3):e114–6.CrossRefPubMedPubMedCentralGoogle Scholar
  5. Lee HS, Joo DJ, Huh KH. Laparoscopic fenestration versus percutaneous catheter drainage for lymphocele treatment after kidney transplantation. Transplant Proc. 2013;5(4):1667–70.CrossRefGoogle Scholar
  6. Mattei J, Kim FJ, Phillips J. Male primary retroperitoneal mucinous cystadenoma. Urology. 2013;82(1):e1–2.CrossRefGoogle Scholar
  7. Shebel HM, Farg HM, Kolokythas O. Cysts of the lower male genitourinary tract: embryologic and anatomic considerations and differential diagnosis. Radiographics. 2013;33(4):1125–43.CrossRefPubMedGoogle Scholar
  8. Siderits R, Ouattara O, Abud A. Retroperitoneal cystic abdominal lymphangiomatosis diagnosed by fine needle aspiration: a case report. Acta Cytol. 2009;53(2):191–4.CrossRefPubMedGoogle Scholar
  9. Sugarbaker P, Yan T, Zappa L. Thin-walled cysts as a pathognomonic CT finding in cystic mesothelioma. Tumori. 2008;94(1):14–8.CrossRefPubMedGoogle Scholar
  10. Wiegand S, Eivazi B, Zimmermann AP. Sclerotherapy of lymphangiomas of the head and neck. Head Neck. 2011;33(11):1649–55.CrossRefPubMedPubMedCentralGoogle Scholar
  11. Yang DM, Jung DH, Kim H. Retroperitoneal cystic masses: CT, clinical, and pathologic findings and literature review. Radiographics. 2004;24(5):1353–65.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2018

Authors and Affiliations

  1. 1.The Third Xiangya Hospital of Central South UniversityChangshaChina
  2. 2.Peking University International HospitalBeijingChina

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