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

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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.

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References

  1. Morrison SC, Reid JR. Continuing problems with classifications of vascular malformations. Pediatr Radiol. 2007;37:609. https://doi.org/10.1007/s00247-007-0442-0.

    Article  PubMed  Google Scholar 

  2. Ramashankar PC, Shah NK, Giraddi G. Lymphatic malformations: a dilemma in diagnosis and management. Contemp Clin Dent. 2014;5(1):119–22. https://doi.org/10.4103/0976-237X.128689.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Khunger N. Lymphatic malformations: current status. J Cutan Aesthet Surg. 2010;3(3):137–8. https://doi.org/10.4103/0974-2077.74487.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Miceli A, Stewart KM. Lymphangioma. [Updated 2020 Aug 12]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK470333/

  5. ISSVA Classification of Vascular Anomalies © 2018 International Society for the Study of Vascular Anomalies Available at “issva.org/classification” Accessed 12 Nov 2020

  6. Ahlawat S, Fayad LM, Durand DJ, Puttgen K, Tekes A. International society for the study of vascular anomalies classification of soft tissue vascular anomalies: survey-based assessment of musculoskeletal radiologists’ use in clinical practice. Curr Probl Diagn Radiol. 2019;48(1):10–6. https://doi.org/10.1067/j.cpradiol.2017.10.003.

    Article  PubMed  Google Scholar 

  7. Mirza B, Ijaz L, Saleem M, Sharif M, Sheikh A. Cystic hygroma: an overview. J Cutan Aesthet Surg. 2010;3(3):139–44. https://doi.org/10.4103/0974-2077.74488.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Poroes F, Petermann D, Andrejevic-Blant S, Labgaa I, Di Mare L. Pediatric cystic lymphangioma of the retroperitoneum: a case report and review of the literature. Med (Baltimore). 2020;99(28): e20827. https://doi.org/10.1097/MD.0000000000020827.

    Article  Google Scholar 

  9. Minocha PK, Roop L, Persad R. Cases of atypical lymphangiomas in children. Case Rep Pediatr. 2014;2014: 626198. https://doi.org/10.1155/2014/626198.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Kudur MH, Hulmani M. Extensive and invasive lymphangioma circumscriptum in a young female: a rare case report and review of the literature. Indian Dermatol Online J. 2013;4(3):199–201. https://doi.org/10.4103/2229-5178.115516.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Heredea R, Cimpean AM, Cerbu S, Popoiu CM, Jitariu AA, Raica M. New approach to rare pediatric multicystic mesenteric lymphangioma; would it guide the development of targeted therapy? Front Pediatr. 2018;6:223. https://doi.org/10.3389/fped.2018.00223.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Goetsch E. Hygroma colli cysticum and hygroma axillare: pathologic and clinical study and report of twelve cases. Arch Surg. 1938;36:394–479.

    Article  Google Scholar 

  13. Mulliken JB, Burrows PE, Fishman SJ. Mulliken and young’s vascular anomalies: hemangiomas and malformations. USA: OUP; 2013. p. 327–507.

    Book  Google Scholar 

  14. Kolay SK, Parwani R, Wanjari S, Singhal P. Oral lymphangiomas—clinical and histopathological relations: an immunohistochemically analyzed case series of varied clinical presentations. J Oral Maxillofac Pathol. 2018;22(Suppl 1):S108–11. https://doi.org/10.4103/jomfp.JOMFP_157_17.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Motahhary P, Sarrafpour B, Abdirad A. Bilateral symmetrical lymphangiomas of the gingiva: case report. Diagn Pathol. 2006;1:9. https://doi.org/10.1186/1746-1596-1-9.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Usha V, Sivasankari T, Jeelani S, Asokan GS, Parthiban J. Lymphangioma of the tongue—a case report and review of literature. J Clin Diagn Res. 2014;8(9):ZD12–4. https://doi.org/10.7860/JCDR/2014/9890.4792.

    Article  Google Scholar 

  17. Cox JA, Bartlett E, Lee EI. Vascular malformations: a review. Semin Plast Surg. 2014;28(2):58–63. https://doi.org/10.1055/s-0034-1376263.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Akaike G, Nozaki T, Makidono A, Saida Y, Hirabayashi T, Suzuki K. A case of lymphatic malformation/lymphangioma of the scrotum. Acta Radiol Short Rep. 2012. https://doi.org/10.1258/arsr.2012.120021.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Al-Shaikh SA, Mubarak AM, Harb ZF. Splenic lymphangioma in an adult. Saudi Med J. 2017;38(11):1148–52. https://doi.org/10.15537/smj.2017.11.20625.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Cheng J, Liu B, Farjat AE, Routh J. National characteristics of lymphatic malformations in children: inpatient estimates and trends in the United States, 2000 to 2009. J Pediatr Hematol Oncol. 2018;40(3):221–3. https://doi.org/10.1097/MPH.0000000000001078.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Grasso DL, Pelizzo G, Zocconi E, Schleef J. Lymphangiomas of the head and neck in children. Acta Otorhinolaryngol Ital. 2008;28(1):17–20 (PMID: 18533550; PMCID: PMC2640069).

    CAS  PubMed  PubMed Central  Google Scholar 

  22. Mota MMDS, Bezerra ROF, Garcia MRT. Practical approach to primary retroperitoneal masses in adults. Radiol Bras. 2018;51(6):391–400. https://doi.org/10.1590/0100-3984.2017.0179.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Alzaraa A, Mousa H, Dickens P, Allen J, Benhamida A. Idiopathic benign retroperitoneal cyst: a case report. J Med Case Rep. 2008;2:43. https://doi.org/10.1186/1752-1947-2-43.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Morotti A, Busso M, ConsiglioBarozzino M, Cinardo P, Angelino V, Familiari U, Veltri A, Guerrasio A. Detection and management of retroperitoneal cystic lesions: a case report and review of the literature. Oncol Lett. 2017;14(2):1602–8. https://doi.org/10.3892/ol.2017.6323.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Gümüştaş OG, Sanal M, Güner O, Tümay V. Retroperitoneal cystic lymphangioma: a diagnostic and surgical challenge. Case Rep Pediatr. 2013. https://doi.org/10.1155/2013/292053 (Epub 2013 Feb 28. PMID: 23533897; PMCID: PMC3600274).

    Article  PubMed  PubMed Central  Google Scholar 

  26. Kopicky L, Humenansky K, Gitzelmann C, Gulati R. Intraabdominal cystic lymphangioma. J Ped Surg Case Rep. 2017;2017(26):32–4. https://doi.org/10.1016/j.epsc.2017.08.010.

    Article  Google Scholar 

  27. GuachilemaRibadeneira A, Monard ÁRT, Endara MC, Garcia CG, Sandoval MO, Cárdenas DA, et al. Intra-abdominal cystic lymphangioma of the mesocolon sigmoids: a rare entity in adult patient woman. J Surg Case Rep. 2020. https://doi.org/10.1093/jscr/rjaa031.

    Article  Google Scholar 

  28. Tripathi M, Parshad S, Karwasra RK, Gupta A, Srivastva S, Sarwal A. Retroperitoneal lymphangioma in an adult: a case report of a rare clinical entity. Case Rep Surg. 2015;2015: 732531. https://doi.org/10.1155/2015/732531.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Suhani AL, Ali S, Thomas S. Giant retroperitoneal lymphangioma: a rare entity. Indian J Surg. 2014;76(5):402–4. https://doi.org/10.1007/s12262-013-0989-y.

    Article  CAS  PubMed  Google Scholar 

  30. Jones IS. Lymphangiomas of the ocular adnexa: an analysis of 62 cases. Trans Am Ophthalmol Soc. 1959;57:602–65 (PMID: 16693588; PMCID: PMC1316348).

    CAS  PubMed  PubMed Central  Google Scholar 

  31. Krüger-Genge A, Blocki A, Franke RP, Jung F. Vascular endothelial cell biology: an Update. Int J Mol Sci. 2019;20(18):4411. https://doi.org/10.3390/ijms20184411.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Ha J, Yu YC, Lannigan F. A review of the management of lymphangiomas. Curr Pediatr Rev. 2014;10(3):238–48. https://doi.org/10.2174/1573396309666131209210751.

    Article  PubMed  Google Scholar 

  33. Shah GH, Deshpande MD. Lymphatic malformation in adult patient: a rare case. J Maxillofac Oral Surg. 2010;9(3):284–8. https://doi.org/10.1007/s12663-010-0082-z.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Ganesh C, Sangeetha GS, Narayanan V, Umamaheswari TN. Lymphangioma circumscriptum in an adult: an unusual oral presentation. J Clin Imaging Sci. 2013;3:44. https://doi.org/10.4103/2156-7514.120779.PMID:24228212;PMCID:PMC3823387.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Flucke U, Karanian M, Broek RWT, Thway K. Soft tissue special issue: perivascular and vascular tumors of the head and neck. Head Neck Pathol. 2020;14(1):21–32. https://doi.org/10.1007/s12105-020-01129-z.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Galambos C, Nodit L. Identification of lymphatic endothelium in pediatric vascular tumors and malformations. Pediatr Dev Pathol. 2005;8(2):181–9. https://doi.org/10.1007/s10024-004-8104-9.

    Article  PubMed  Google Scholar 

  37. Cai X, Zhang W, Chen G, Li RF, Sun YF, Zhao YF. Mesenchymal status of lymphatic endothelial cell: enlightening treatment of lymphatic malformation. Int J Clin Exp Med. 2015;8(8):12239–51 (PMID: 26550134; PMCID: PMC4612819).

    PubMed  PubMed Central  Google Scholar 

  38. Castro EC, Galambos C. Prox-1 and VEGFR3 antibodies are superior to D2–40 in identifying endothelial cells of lymphatic malformations–a proposal of a new immunohistochemical panel to differentiate lymphatic from other vascular malformations. Pediatr Dev Pathol. 2009;12(3):187–94. https://doi.org/10.2350/08-05-0471.1.

    Article  PubMed  Google Scholar 

  39. Abe N, Ohtake T, Saito K, Kumamoto K, Sugino T, Takenoshita S. Clinicopathological significance of lymphangiogenesis detected by immunohistochemistry using D2–40 monoclonal antibody in breast cancer. Fukushima J Med Sci. 2016;62(1):57–63. https://doi.org/10.5387/fms.2015-10.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Adamczyk LA, Gordon K, Kholová I, Meijer-Jorna LB, Telinius N, Gallagher PJ, et al. Lymph vessels: the forgotten second circulation in health and disease. Virchows Arch. 2016;469(1):3–17. https://doi.org/10.1007/s00428-016-1945-6.

    Article  PubMed  PubMed Central  Google Scholar 

  41. Miettinen M, Wang ZF. Prox1 transcription factor as a marker for vascular tumors-evaluation of 314 vascular endothelial and 1086 nonvascular tumors. Am J Surg Pathol. 2012;36(3):351–9. https://doi.org/10.1097/PAS.0b013e318236c312.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Perkins JA. New frontiers in our understanding of lymphatic malformations of the head and neck: natural history and basic research. Otolaryngol Clin North Am. 2018;51(1):147–58. https://doi.org/10.1016/j.otc.2017.09.002.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Padia R, Zenner K, Bly R, Bennett J, Bull C, Perkins J. Clinical application of molecular genetics in lymphatic malformations. Laryngoscope Investig Otolaryngol. 2019;4(1):170–3. https://doi.org/10.1002/lio2.241.

    Article  PubMed  PubMed Central  Google Scholar 

  44. Wood DE, White JR, Georgiadis A, Van Emburgh B, Parpart-Li S, Mitchell J, et al. A machine learning approach for somatic mutation discovery. Sci Transl Med. 2018;10(457):eaar7939. https://doi.org/10.1126/scitranslmed.aar7939.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Bessi L, Viailly PJ, Bohers E, Ruminy P, Maingonnat C, Bertrand P, et al. Somatic mutations of cell-free circulating DNA detected by targeted next-generation sequencing and digital droplet PCR in classical hodgkin lymphoma. Leuk Lymphoma. 2019;60(2):498–502. https://doi.org/10.1080/10428194.2018.1492123.

    Article  CAS  PubMed  Google Scholar 

  46. Gao J, Chang MT, Johnsen HC, Gao SP, Sylvester BE, Sumer SO, et al. 3D clusters of somatic mutations in cancer reveal numerous rare mutations as functional targets. Genome Med. 2017;9(1):4. https://doi.org/10.1186/s13073-016-0393-x.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  47. North PE, Sander T. Vascular tumors and developmental malformations: pathogenic mechanisms and molecular diagnosis. New York: Springer-Verlag; 2016. p. 1–149.

    Book  Google Scholar 

  48. Freed D, Stevens EL, Pevsner J. Somatic mosaicism in the human genome. Genes (Basel). 2014;5(4):1064–94. https://doi.org/10.3390/genes5041064.

    Article  CAS  PubMed  Google Scholar 

  49. Blesinger H, Kaulfuß S, Aung T, Schwoch S, Prantl L, Rößler J, et al. PIK3CA mutations are specifically localized to lymphatic endothelial cells of lymphatic malformations. PLoS ONE. 2018;13(7): e0200343. https://doi.org/10.1371/journal.pone.0200343.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  50. Castillo SD, Tzouanacou E, Zaw-Thin M, Berenjeno IM, Parker VE, Chivite I, et al. Somatic activating mutations in Pik3ca cause sporadic venous malformations in mice and humans. Sci Transl Med. 2016;8(332):332ra43. https://doi.org/10.1126/scitranslmed.aad9982.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Le Cras TD, Goines J, Lakes N, Pastura P, Hammill AM, Adams DM, et al. Constitutively active PIK3CA mutations are expressed by lymphatic and vascular endothelial cells in capillary lymphatic venous malformation. Angiogenesis. 2020;23(3):425–42. https://doi.org/10.1007/s10456-020-09722-0.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  52. Keppler-Noreuil KM, Parker VE, Darling TN, Martinez-Agosto JA. Somatic overgrowth disorders of the PI3K/AKT/mTOR pathway and therapeutic strategies. Am J Med Genet C Semin Med Genet. 2016;172(4):402–21. https://doi.org/10.1002/ajmg.c.31531.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  53. Melikhan-Revzin S, Kurolap A, Dagan E, Mory A, Gershoni-Baruch R. A novel missense mutation in FLT4 causes autosomal recessive hereditary lymphedema. Lymphat Res Biol. 2015;13(2):107–11. https://doi.org/10.1089/lrb.2014.0044.

    Article  CAS  PubMed  Google Scholar 

  54. Irrthum A, Karkkainen MJ, Devriendt K, Alitalo K, Vikkula M. Congenital hereditary lymphedema caused by a mutation that inactivates VEGFR3 tyrosine kinase. Am J Hum Genet. 2000;67(2):295–301. https://doi.org/10.1086/303019.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Kaser-Eichberger A, Schroedl F, Bieler L, Trost A, Bogner B, Runge C, et al. Expression of lymphatic markers in the adult rat spinal cord. Front Cell Neurosci. 2016;10:23. https://doi.org/10.3389/fncel.2016.00023.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  56. Ghalamkarpour A, Morlot S, Raas-Rothschild A, Utkus A, Mulliken JB, Boon LM, et al. Hereditary lymphedema type I associated with VEGFR3 mutation: the first de novo case and atypical presentations. Clin Genet. 2006;70(4):330–5. https://doi.org/10.1111/j.1399-0004.2006.00687.x.

    Article  CAS  PubMed  Google Scholar 

  57. Miettinen M, Wang ZF, Paetau A, Tan SH, Dobi A, Srivastava S, et al. ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma. Am J Surg Pathol. 2011;35(3):432–41. https://doi.org/10.1097/PAS.0b013e318206b67b.

    Article  PubMed  PubMed Central  Google Scholar 

  58. Wetzel-Strong SE, Detter MR, Marchuk DA. The pathobiology of vascular malformations: insights from human and model organism genetics. J Pathol. 2017;241(2):281–93. https://doi.org/10.1002/path.4844.

    Article  PubMed  Google Scholar 

  59. Wang QK. Update on the molecular genetics of vascular anomalies. Lymphat Res Biol. 2005;3(4):226–33. https://doi.org/10.1089/lrb.2005.3.226.

    Article  CAS  PubMed  Google Scholar 

  60. Lal A, Gupta P, Singhal M, Sinha SK, Lal S, Rana S, et al. Abdominal lymphatic malformation: Spectrum of imaging findings. Indian J Radiol Imaging. 2016;26(4):423–8. https://doi.org/10.4103/0971-3026.195777.

    Article  PubMed  PubMed Central  Google Scholar 

  61. Li Q, Ji D, Tu KS, Dou CW, Yao YM. Clinical analysis of intraperitoneal lymphangioma. Chin Med J (Engl). 2015;128(22):3043–9. https://doi.org/10.4103/0366-6999.169061.

    Article  PubMed  Google Scholar 

  62. Perez A, Perez MEC, Yuga AC, Viray BAG. Splenic lymphangioma in adulthood: a case report. Int J Surg Case Rep. 2020;67:250–3. https://doi.org/10.1016/j.ijscr.2020.01.061.

    Article  PubMed  PubMed Central  Google Scholar 

  63. Méndez-Gallart R, Solar-Boga A, Gómez-Tellado M, Somoza-Argibay I. Giant mesenteric cystic lymphangioma in an infant presenting with acute bowel obstruction. Can J Surg. 2009;52(3):E42–3 (PMID: 19503641; PMCID: PMC2689734).

    PubMed  PubMed Central  Google Scholar 

  64. Hitzerd E, van Hamont D, Pijnenborg JM. Mesenteric lymphangioma mimicking a cystic ovarian mass on imaging. BMJ Case Rep. 2016. https://doi.org/10.1136/bcr-2015-213727.

    Article  PubMed  PubMed Central  Google Scholar 

  65. Carqueja IM, Sousa J, Mansilha A. Vascular malformations: classification, diagnosis and treatment. Int Angiol. 2018;37(2):127–42. https://doi.org/10.23736/S0392-9590.18.03961-5.

    Article  PubMed  Google Scholar 

  66. Aslan A, Büyükkaya R, Tan S, Erdoğan C, Hakyemez B. Efficacy of ultrasonography in lymphatic malformations: diagnosis, treatment and follow-up: a case report. Med Ultrason. 2013;15(3):244–6. https://doi.org/10.11152/mu.2013.2066.153.aa1rb2.

    Article  PubMed  Google Scholar 

  67. Li J, Zhong W, Geng X, Liu X, Zhang X, Wang Y, et al. Ultrasonographic diagnosis, classification, and treatment of cervical lymphatic malformation in paediatric patients: a retrospective study. BMC Pediatr. 2020;20(1):441. https://doi.org/10.1186/s12887-020-02337-w.

    Article  PubMed  PubMed Central  Google Scholar 

  68. Loberant N, Chernihovski A, Goldfeld M, Sweed Y, Vais M, Tzilman B, Cohen I. Role of doppler sonography in the diagnosis of cystic lymphangioma of the scrotum. J Clin Ultrasound. 2002;30(6):384–7. https://doi.org/10.1002/jcu.10080.

    Article  PubMed  Google Scholar 

  69. Dubois J, Garel L, Grignon A, David M, Laberge L, Filiatrault D, et al. Imaging of hemangiomas and vascular malformations in children. Acad Radiol. 1998;5(5):390–400. https://doi.org/10.1016/S1076-6332(98)80158-X.

    Article  CAS  PubMed  Google Scholar 

  70. Paltiel HJ, Burrows PE, Kozakewich HPW, Zurakowski D, Mulliken JB. Soft-tissue vascular anomalies: utility of US for diagnosis. Radiology. 2000;214(3):747–54. https://doi.org/10.1148/radiology.214.3.r00mr21747.

    Article  CAS  PubMed  Google Scholar 

  71. Hyodoh H, Hori M, Akiba H, Tamakawa M, Hyodoh K, Hareyama M. Peripheral vascular malformations: imaging, treatment approaches, and therapeutic issues. Radiographics. 2005;25(Suppl 1):S159–71. https://doi.org/10.1148/rg.25si055509.

    Article  PubMed  Google Scholar 

  72. Madani H, Farrant J, Chhaya N, Anwar I, Marmery H, Platts A, et al. Peripheral limb vascular malformations: an update of appropriate imaging and treatment options of a challenging condition. Br J Radiol. 2015;88(1047):20140406. https://doi.org/10.1259/bjr.20140406.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  73. Chaker K, Sellami A, Ouanes Y, Zehani A, Jallouli W, Ben Chehida MA, et al. Retroperitoneal cystic lymphangioma in an adult: a case report. Urol Case Rep. 2018;18:33–4. https://doi.org/10.1016/j.eucr.2018.02.019.

    Article  PubMed  PubMed Central  Google Scholar 

  74. Flors L, Leiva-Salinas C, Maged IM, Norton PT, Matsumoto AH, Angle JF, et al. MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up. Radiographics. 2011;31(5):1321–40. https://doi.org/10.1148/rg.315105213 (discussion 1340-1).

    Article  PubMed  Google Scholar 

  75. Moukaddam H, Pollak J, Haims AH. MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol. 2009;38(6):535–47. https://doi.org/10.1007/s00256-008-0609-2.

    Article  PubMed  Google Scholar 

  76. Nosher JL, Murillo PG, Liszewski M, Gendel V, Gribbin CE. Vascular anomalies: a pictorial review of nomenclature, diagnosis and treatment. World J Radiol. 2014;6(9):677–92. https://doi.org/10.4329/wjr.v6.i9.677.

    Article  PubMed  PubMed Central  Google Scholar 

  77. Ohgiya Y, Hashimoto T, Gokan T, Watanabe S, Kuroda M, Hirose M, et al. Dynamic MRI for distinguishing high-flow from low-flow peripheral vascular malformations. AJR Am J Roentgenol. 2005;185(5):1131–7. https://doi.org/10.2214/AJR.04.1508.

    Article  PubMed  Google Scholar 

  78. Bashir U, Shah S, Jeph S, O’Keeffe M, Khosa F. Magnetic resonance (MR) imaging of vascular malformations. Pol J Radiol. 2017;82:731–41. https://doi.org/10.12659/PJR.903491.

    Article  PubMed  PubMed Central  Google Scholar 

  79. Coffin A, Boulay-Coletta I, Sebbag-Sfez D, Zins M. Radioanatomy of the retroperitoneal space. Diagn Interv Imaging. 2015;96(2):171–86. https://doi.org/10.1016/j.diii.2014.06.015.

    Article  CAS  PubMed  Google Scholar 

  80. Brennan C, Kajal D, Khalili K, Ghai S. Solid malignant retroperitoneal masses-a pictorial review. Insights Imaging. 2014;5(1):53–65. https://doi.org/10.1007/s13244-013-0294-0.

    Article  PubMed  Google Scholar 

  81. Scali EP, Chandler TM, Heffernan EJ, Coyle J, Harris AC, Chang SD. Primary retroperitoneal masses: what is the differential diagnosis? Abdom Imaging. 2015;40(6):1887–903. https://doi.org/10.1007/s00261-014-0311-x.

    Article  PubMed  Google Scholar 

  82. Zhao M, Li C, Zheng J, Yan M, Sun K, Wang Z. Cystic lymphangioma-like adenomatoid tumor of the adrenal gland: report of a rare case and review of the literature. Int J Clin Exp Pathol. 2013;6(5):943–50 (PMID: 23638228; PMCID: PMC3638107).

    PubMed  PubMed Central  Google Scholar 

  83. Rajiah P, Sinha R, Cuevas C, Dubinsky TJ, Bush WH Jr, Kolokythas O. Imaging of uncommon retroperitoneal masses. Radiographics. 2011;31(4):949–76. https://doi.org/10.1148/rg.314095132.

    Article  PubMed  Google Scholar 

  84. Yang DM, Jung DH, Kim H, Kang JH, Kim SH, Kim JH, Hwang HY. Retroperitoneal cystic masses: CT, clinical, and pathologic findings and literature review. Radiographics. 2004;24(5):1353–65. https://doi.org/10.1148/rg.245045017.

    Article  PubMed  Google Scholar 

  85. Hoang VT, Trinh CT, Le TB, Le TK. Recurrence of retroperitoneal mature cystic teratoma in an adult: a case report. Radiol Case Rep. 2019;14(6):692–6. https://doi.org/10.1016/j.radcr.2019.03.008.

    Article  PubMed  PubMed Central  Google Scholar 

  86. Xiao J, Shao Y, Zhu S, He X. Characteristics of adult abdominal cystic Lymphangioma: a single-center Chinese cohort of 12 cases. BMC Gastroenterol. 2020;20(1):244. https://doi.org/10.1186/s12876-020-01388-8.

    Article  PubMed  PubMed Central  Google Scholar 

  87. Surabhi VR, Menias C, Prasad SR, Patel AH, Nagar A, Dalrymple NC. Neoplastic and non-neoplastic proliferative disorders of the perirenal space: cross-sectional imaging findings. Radiographics. 2008;28(4):1005–17. https://doi.org/10.1148/rg.284075157.

    Article  PubMed  Google Scholar 

  88. Funaki K, Fukunishi H, Tsuji Y, Maeda T, Takahashi T. Giant cystic leiomyoma of the uterus occupying the retroperitoneal space. J Radiol Case Rep. 2013;7(12):35–40. https://doi.org/10.3941/jrcr.v7i12.1447.

    Article  PubMed  PubMed Central  Google Scholar 

  89. Gemici K, Buldu İ, Acar T, Alptekin H, Kaynar M, Tekinarslan E, Karatağ T, Efe D, Çolak H, Küçükkartallar T, İstanbulluoğlu MO. Management of patients with retroperitoneal tumors and a review of the literature. World J Surg Oncol. 2015;13:143. https://doi.org/10.1186/s12957-015-0548-z.

    Article  PubMed  PubMed Central  Google Scholar 

  90. Kumar N, Yadav P, Ansari MS, Lal H. Surgical management of giant retroperitoneal lymphangioma in a child. BMJ Case Reports CP. 2020;13: e234447. https://doi.org/10.1136/bcr-2020-234447.

    Article  Google Scholar 

  91. Borman P. Lymphedema diagnosis, treatment, and follow-up from the view point of physical medicine and rehabilitation specialists. Turk J Phys Med Rehabil. 2018;64(3):179–97. https://doi.org/10.5606/tftrd.2018.3539.

    Article  PubMed  PubMed Central  Google Scholar 

  92. Rodriguez-Laguna L, Agra N, Ibañez K, Oliva-Molina G, Gordo G, Khurana N, et al. Somatic activating mutations in PIK3CA cause generalized lymphatic anomaly. J Exp Med. 2019;216(2):407–18. https://doi.org/10.1084/jem.20181353.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  93. Geissler EK. The influence of mTOR inhibitors on immunity and the relationship to post-transplant malignancy. Transplant Res. 2013;2(Suppl 1):S2. https://doi.org/10.1186/2047-1440-2-S1-S2.

    Article  PubMed  PubMed Central  Google Scholar 

  94. Ricci KW. Advances in the medical management of vascular anomalies. Semin Intervent Radiol. 2017;34(3):239–49. https://doi.org/10.1055/s-0037-1604297.

    Article  PubMed  PubMed Central  Google Scholar 

  95. Teachey DT, Greiner R, Seif A, Attiyeh E, Bleesing J, Choi J, et al. Treatment with sirolimus results in complete responses in patients with autoimmune lymphoproliferative syndrome. Br J Haematol. 2009;145(1):101–6. https://doi.org/10.1111/j.1365-2141.2009.07595.x.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  96. Mofenson LM, Brady MT, Danner SP, Dominguez KL, Hazra R, Handelsman E, et al. Guidelines for the prevention and treatment of opportunistic infections among HIV-exposed and HIV-infected children: recommendations from CDC, the National Institutes of Health, the HIV Medicine Association of the Infectious Diseases Society of America, the Pediatric Infectious Diseases Society, and the American Academy of Pediatrics. MMWR Recomm Rep. 2009;58(RR-11):1–166.

    PubMed  Google Scholar 

  97. Margolin JF, Soni HM, Pimpalwar S. Medical therapy for pediatric vascular anomalies. Semin Plast Surg. 2014;28(2):79–86. https://doi.org/10.1055/s-0034-1376264.

    Article  PubMed  PubMed Central  Google Scholar 

  98. Padia R, Bly R, Bull C, Geddis AE, Perkins J. Medical management of vascular anomalies. Curr Treat Options Pediatr. 2018;4(2):221–36. https://doi.org/10.1007/s40746-018-0130-3.

    Article  PubMed  PubMed Central  Google Scholar 

  99. Institute of Medicine (US) Committee on Accelerating Rare Diseases Research and Orphan Product Development. Rare Diseases and Orphan Products: Accelerating Research and Development. In: Field MJ, Boat TF, (eds). National Academies Press, Washington (DC), USA, 2010. PMID: 21796826

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  1. Van Trung Hoang and Minh Duc Nguyen share the first authorship.

Authors and Affiliations

  1. Department of Radiology, Thien Hanh Hospital, Buon Ma Thuot, 630000, Vietnam

    Van Trung Hoang & Hoang Anh Thi Van

  2. Department of Radiology, Pham Ngoc Thach University of Medicine, Ho Chi Minh City, 700000, Vietnam

    Minh Duc Nguyen

  3. Division of Endocrinology, Department of Medicine, Walter Reed National Military Medical Center, Bethesda, MD, 20889, USA

    Duc Thanh Hoang

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  1. Van Trung Hoang
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  3. Hoang Anh Thi Van
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VTH contributed to the conception and design of the manuscript and data acquisition and wrote the initial manuscript. HATV critically revised the manuscript. MDN and DTH supported the revision and critically reviewed the paper. All authors participated in the approval of the final version.

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Hoang, V.T., Nguyen, M.D., Van, H.A.T. et al. Review of diagnosis, differential diagnosis, and management of retroperitoneal lymphangioma. Jpn J Radiol 41, 283–301 (2023). https://doi.org/10.1007/s11604-022-01356-0

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