Skip to main content
SpringerLink
Log in

Primary cardiac and pericardial lymphangiomas: clinical, radiologic, and pathologic characterization derived from an institutional series and review of the literature

  • Original Article
  • Published:
Virchows Archiv Aims and scope Submit manuscript

Abstract

Lymphangiomas are comprised of aggregates of lymphatic vessels, considered to represent either aberrant embryogenic remnants or developing secondary to obstruction. Lymphangiomas primary to the heart and pericardial are exceedingly rare, and to date sparingly reported in individual case reports. In this study, the histopathologic, clinical, and radiologic features of 35 cases of cardiac/pericardial lymphangiomas described in the literature to date together with four cases from our own institution (39 cases in total) are examined to provide clinicopathologic characterization. Cardiac/pericardial lymphangiomas were identified in both children and adults, with two cases initially discovered in utero. If presenting with symptoms, patients most commonly exhibited respiratory distress/dyspnea. By X-ray, a widened cardiac silhouette could be noted, and echocardiogram generally showed an echogenic mass with cystic and septal components. On computed tomography (CT) and magnetic resonance imaging (MRI), cystic and septal components were again observed, with CT showing an absence of calcifications or macroscopic fat. Most lymphangiomas were pericardial (specifically visceral) based, and frequently situated in the right atrioventricular groove. A majority of cases proceeded to surgical resection, with no evidence of recurrence post-operatively. Grossly, lesions had a median size of 6 cm and in almost all cases were multicystic/multilocular. Microscopically, the lymphangiomas were composed of lymphatic spaces lined by endothelial cells that specifically express podoplanin (D2-40) with immunoperoxidase staining. Further investigation with a larger and more uniformly organized cohort is required to better characterize the clinicopathologic features of lymphangiomas of this unusual anatomic location.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Park JG, Aubry MC, Godfrey JA, Midthun DE (2006) Mediastinal lymphangioma: Mayo Clinic experience of 25 cases. Mayo Clin Proc 81:1197–1203

    Article  PubMed  Google Scholar 

  2. Brown LR, Reiman HM, Rosenow EC 3rd, Gloviczki PM, Divertie MB (1986) Intrathoracic lymphangioma. Mayo Clin Proc 61:882–892

    Article  CAS  PubMed  Google Scholar 

  3. Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD et al (2000) Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 161:1037–1046

    Article  CAS  PubMed  Google Scholar 

  4. Shaffer K, Rosado-de-Christenson ML, Patz EF Jr, Young S, Farver CF (1994) Thoracic lymphangioma in adults: CT and MR imaging features. AJR Am J Roentgenol 162:283–289

    Article  CAS  PubMed  Google Scholar 

  5. Almarsafawy H, Matter M, Elgamal MA, Zalata K (2011) Cardiac lymphangioma in an infant. Pediatr Cardiol 32:1253–1255

    Article  PubMed  Google Scholar 

  6. Anbe DT, Fine G (1973) Cardiac lymphangioma and lipoma. Report of a case of simultaneous occurrence in association with lipomatous infiltration of the myocardium and cardiac arrhythmia. Am Heart J 86:227–235

    Article  CAS  PubMed  Google Scholar 

  7. Bansal N, Haidar-El-Atrache S, Walters HL 3rd, Kobayashi D (2017) Cardiac lymphangioma encasing right coronary artery in an infant. Ann Thorac Surg 104:e279–ee81

    Article  PubMed  Google Scholar 

  8. Biskupski A, Waligorski S, Mokrzycki K, Biskupska K, Brykczynski M (2013) Cardiac lymphangioma in the right atrium. Ann Thorac Surg 96:328

    Article  PubMed  Google Scholar 

  9. Cailleba L, Labrousse L, Marty M, Montaudon M, Gerbaud E (2013) Pericardial cystic lymphangioma. Eur Heart J Cardiovasc Imaging 14:246

    Article  PubMed  Google Scholar 

  10. Daubeney PE, Ogilvie BC, Moore IE, Webber SA (1996) Intrapericardial lymphangioma presenting as neonatal cardiac tamponade. Pediatr Cardiol 17:129–131

    Article  CAS  PubMed  Google Scholar 

  11. Eliaschewitsch PA (1929) Ein fall von perikardcyste. Virchows Arch 270:868–872

    Article  Google Scholar 

  12. Florchinger B, Rummele P, Lehane C, Schmid FX, Birnbaum DE (2005) Mitral prolapse caused by lymphangioma. Thorac Cardiovasc Surg 53:180–183

    Article  CAS  PubMed  Google Scholar 

  13. Gross RE, Hurwitt ES (1948) Cervicomediastinal and mediastinal cystic hygromas. Surg Gynecol Obstet 87:599–610

    CAS  PubMed  Google Scholar 

  14. Halonen PI, Nylander PE, Viikari SJ (1955) On pericardial lymphangiomas. Cardiologia. 27:59–64

    Article  CAS  PubMed  Google Scholar 

  15. Harley HR, Drew CE (1950) Cystic hygroma of the mediastinum. Thorax. 5:105–115

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Heffron SP, Alviar CL, Towe C, Geisler BP, Axel L, Galloway AC et al (2016) Dyspnea and chest pain in a young woman caused by a giant pericardial lymphohemangioma: diagnosis and treatment. Can J Cardiol 32(1260):e23–e25

    Google Scholar 

  17. Huang Z (2013) Lymphangioma of the left ventricle. J Card Surg 28:24–26

    Article  PubMed  Google Scholar 

  18. Jougon J, Laborde MN, Parrens M, MacBride T (2002) Cystic lymphangioma of the heart mimicking a mediastinal tumor. Eur J Cardiothorac Surg 22:476–478

    Article  PubMed  Google Scholar 

  19. Kaji T, Takamatsu H, Noguchi H, Tahara H, Matsuda H, Nomura Y et al (2002) Cardiac lymphangioma: case report and review of the literature. J Pediatr Surg 37:E32

    Article  PubMed  Google Scholar 

  20. Kim SJ, Shin ES, Kim SW, Shin JK, Cheong JP, Kim YM et al (2007) A case of cardiac lymphangioma presenting as a cystic mass in the right atrium. Yonsei Med J 48:1043–1047

    Article  PubMed  PubMed Central  Google Scholar 

  21. Lenkeit W (1928) Zysten des epi- und perikards. Zentralbl Allg Pathol 44:97–100

    Google Scholar 

  22. Lone NA, Naikoo BA, Khan NA (2016) Cardiac lymphangioma presenting as intrapericardial cystic mass. Saudi Med J 37:90–92

    Article  PubMed  PubMed Central  Google Scholar 

  23. Lymburner RM (1934) Tumours of the heart: histopathological and clinical study. Can Med Assoc J 30:368–373

    CAS  PubMed  PubMed Central  Google Scholar 

  24. Mishra P, Vaideeswar P, Marathe SP, Aironi B, Agrawal NB (2015) Lymphangioma presenting as cardiac tamponade in a child. J Card Surg 30:613–616

    Article  PubMed  Google Scholar 

  25. Moore TC, Cobo JC (1985) Massive symptomatic cystic hygroma confined to the thorax in early childhood. J Thorac Cardiovasc Surg 89:459–462

    Article  CAS  PubMed  Google Scholar 

  26. Nataf P, Mestiri T, de Lasalle Martin E, Benomar M, Gandjbakhch I, Cabrol C (1988) Pericardial hemolymphangioma. Apropos of a case. Arch Mal Coeur Vaiss 81:1137–1140

    CAS  PubMed  Google Scholar 

  27. Pasaoglu I, Dogan R, Ozme S, Kale G, Bozer AY (1991) Cardiac lymphangioma. Am Heart J 121:1821–1824

    Article  CAS  PubMed  Google Scholar 

  28. Robillard J, Pena E, Veinot JP, Fulop J, Dennie C (2014) Pericardial lymphangiohemangioma: multimodality imaging features and pathologic correlation. Circulation. 129:e657–e659

    Article  PubMed  Google Scholar 

  29. Shroff GS, Lata AL, Parks GE, Entrikin DW (2011) Giant pericardial lymphangioma-imaging, surgical, and pathologic correlations. J Comput Assist Tomogr 35:642–644

    Article  PubMed  Google Scholar 

  30. Sinzelle E, Duong Van Huyen JP, Breiteneder-Geleff S, Braunberger E, Deloche A, Kerjaschki D et al (2000) Intrapericardial lymphangioma with podoplanin immunohistochemical characterization of lymphatic endothelial cells. Histopathology. 37:93–94

    Article  CAS  PubMed  Google Scholar 

  31. Skinner GF, Hobbs ME (1936) Intrathoracic cystic lymphangioma. J Thorac Surg 6:98–107

    Article  Google Scholar 

  32. Soler R, Rodriguez E, Remuinan C, Castro JM (2002) Mediastinal lymphangioma involving the heart: CT and MR findings. Clin Radiol 57:415–418

    Article  PubMed  Google Scholar 

  33. Uehlinger E (1927) Die lymphgefassgeschwulste des herzens. Beiträge zur pathologischen Anatomie und zur allgemeinen:Pathologie 78:434–440

    Google Scholar 

  34. Vinayakumar D, Arunkumar G, Sajeev CG, Rajesh G, Muneer K, Haridasan V et al (2014) Cystic lymphangioma of pericardium presenting as isolated chylopericardium - a case report. Indian Heart J 66:119–121

    Article  CAS  PubMed  Google Scholar 

  35. Walker WP, Landas SK (1990) Nuchal cystic hygroma associated with massive lymphangiomatosis of the upper extremity and myocardium in a stillborn fetus: a case report. Am J Cardiovasc Pathol 3:341–346

    CAS  PubMed  Google Scholar 

  36. Zakaria RH, Barsoum NR, El-Basmy AA, El-Kaffas SH (2011) Imaging of pericardial lymphangioma. Ann Pediatr Cardiol 4:65–67

    Article  PubMed  PubMed Central  Google Scholar 

  37. Sekulic SP, Kalfa DM, Fenlon E, Shah A, LaCorte J, Sekulic M (2021) Pericardial lymphangioma: a rare benign albeit variably symptomatic tumefactive lesion. Cardiovasc Pathol 2021:107402

    Google Scholar 

  38. Diao WJ, Shi C, Liu G, Liu XG, Li HH, Meng JJ et al (2019) The diagnosis and treatment of cardiac lymphangioma: a case report and literature review. Medicine. 98:e14000

    Article  PubMed  PubMed Central  Google Scholar 

  39. Pennec PY, Blanc JJ (2006) Cardiac lymphangioma: a benign cardiac tumour. Eur Heart J 27:2913

    Article  PubMed  Google Scholar 

  40. Okazaki T, Iwatani S, Yanai T, Kobayashi H, Kato Y, Marusasa T et al (2007) Treatment of lymphangioma in children: our experience of 128 cases. J Pediatr Surg 42:386–389

    Article  PubMed  Google Scholar 

  41. Krywanczyk AR, Tan CD, Rodriguez ER (2021) A clinico-pathologic approach to the differential diagnosis of pericardial tumors. Curr Cardiol Rep 23:119

    Article  PubMed  Google Scholar 

  42. Pichler Sekulic S, Glasgow A, Wasman J, Sabik JF, Fitzsimons B, Sekulic M (2020) Cardiac varix: an example via a case report of a radiological mimicker of cardiac myxoma. Cardiovasc Pathol 45:107183

    Article  PubMed  Google Scholar 

  43. Hidron A, Vogenthaler N, Santos-Preciado JI, Rodriguez-Morales AJ, Franco-Paredes C, Rassi A Jr (2010) Cardiac involvement with parasitic infections. Clin Microbiol Rev 23:324–349

    Article  PubMed  PubMed Central  Google Scholar 

  44. Burke AP, Anderson PG, Virmani R, James TN, Herrera GA, Ceballos R (1990) Tumor of the atrioventricular nodal region. A clinical and immunohistochemical study. Arch Pathol Lab Med 114:1057–1062

    CAS  PubMed  Google Scholar 

  45. Zimmerman KG, Paplanus SH, Dong S, Nagle RB (1983) Congenital blood cysts of the heart valves. Hum Pathol 14:699–703

    Article  CAS  PubMed  Google Scholar 

  46. Armstrong H, Monkeberg JG (1911) Herzblock, bedingt durch primaren herztumor, bei einem 5 jahrigen kinde. Dtsch Arch Klin Med 102:144–166

    Google Scholar 

  47. Fontaliran F, Guillois B, Colin A, Blanc JJ, Chabaud JJ, Boog G et al (1989) Congenital atrioventricular block and maternal lupus erythematosus. Histologic discovery of tumor of the atrioventricular node. Arch Mal Coeur Vaiss 82:609–613

    CAS  PubMed  Google Scholar 

  48. Hatipoglu A (1998) Intrapericardial and cardiac lymphangiomas. Pediatr Cardiol 19:192

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology and Cell Biology, Columbia University Irving Medical Center, 630 West 168th Street, VC14-238C, New York, NY, 10032, USA

    Simona Pichler Sekulic & Miroslav Sekulic

Authors
  1. Simona Pichler Sekulic
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Miroslav Sekulic
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

SPS and MS provided patient care and contributed to the preparation of the manuscript.

Corresponding author

Correspondence to Miroslav Sekulic.

Ethics declarations

Ethical approval

This was performed in accordance with research policies approved by the Institution Review Boards of the authors’ institution.

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Pichler Sekulic, S., Sekulic, M. Primary cardiac and pericardial lymphangiomas: clinical, radiologic, and pathologic characterization derived from an institutional series and review of the literature. Virchows Arch 480, 1211–1221 (2022). https://doi.org/10.1007/s00428-022-03269-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00428-022-03269-9

Keywords

Search

Navigation