Vascular Malformations and Tumors: A Review of Classification and Imaging Features for Cardiothoracic Radiologists

Overview

Journal Radiol Cardiothorac Imaging

Specialty Cardiology & Vascular Diseases

Date 2023 Sep 11

PMID 37693195

Authors

Yoshiaki Ota

Elizabeth Lee

Edith Sella

Prachi Agarwal

Affiliations

Soon will be listed here.

Abstract

The International Society for the Study of Vascular Anomalies (ISSVA) classification is a comprehensive histology-based scheme that was updated in 2018. It is important for cardiothoracic imagers to understand this classification to ensure that accurate terminology is used and that archaic terms are avoided when vascular lesions are described. Knowledge of the various malformations (including common conditions, such as venous malformation, arteriovenous fistula, and arteriovenous malformation) and vascular tumors allows for timely diagnosis and appropriate management. This review describes various vascular anomalies, in accordance with ISSVA classification and terminology; highlights key imaging features associated with each; and discusses the role of different imaging modalities. Pulmonary, Soft Tissues/Skin, Vascular, Arteriovenous Malformation © RSNA, 2023.

Citing Articles

The Evaluation, Diagnosis, and Management of Infantile Hemangiomas-A Comprehensive Review.

Resic A, Barcot Z, Habek D, Pogorelic Z, Baskovic M J Clin Med. 2025; 14(2).

PMID: 39860430 PMC: 11765582. DOI: 10.3390/jcm14020425.

Prenatal Identification of Intracardiac Mass Expedites Surgical Management of Capillary-Venous Malformation.

Norton D, Rao M, Zhang J, Hicks M, Parthiban A, Sexson Tejtel S JACC Case Rep. 2025; 29(24):102922.

PMID: 39822627 PMC: 11734063. DOI: 10.1016/j.jaccas.2024.102922.

Imaging of Vascular Anomalies.

Gayou E, Chau A, Josephs S Semin Intervent Radiol. 2024; 41(4):339-349.

PMID: 39524243 PMC: 11543108. DOI: 10.1055/s-0044-1791205.

Vascular Anomalies of the Head and Neck - Case Series.

Soumya M, Sreenivas V, Thomas T Indian J Otolaryngol Head Neck Surg. 2024; 76(5):3865-3870.

PMID: 39376291 PMC: 11455764. DOI: 10.1007/s12070-024-04732-6.

Successful treatment by coil embolization for pediatric brachial arteriovenous fistula: A case report.

Peng H, Liu C Radiol Case Rep. 2024; 19(9):3599-3604.

PMID: 38989450 PMC: 11233829. DOI: 10.1016/j.radcr.2024.05.039.

References

Antonescu C . Malignant vascular tumors--an update. Mod Pathol. 2014; 27 Suppl 1:S30-8. DOI: 10.1038/modpathol.2013.176. View

Green J, Resnick S, Restrepo R, Lee E . Spectrum of Imaging Manifestations of Vascular Malformations and Tumors Beyond Childhood: What General Radiologists Need to Know. Radiol Clin North Am. 2020; 58(3):583-601. DOI: 10.1016/j.rcl.2020.01.004. View

Faughnan M, Palda V, Garcia-Tsao G, Geisthoff U, McDonald J, Proctor D . International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet. 2009; 48(2):73-87. DOI: 10.1136/jmg.2009.069013. View

Chinello M, Di Carlo D, Olivieri F, Balter R, De Bortoli M, Vitale V . Successful Management of Kaposiform Hemangioendothelioma with Long-Term Sirolimus Treatment: a Case Report and Review of the Literature. Mediterr J Hematol Infect Dis. 2018; 10(1):e2018043. PMC: 6039087. DOI: 10.4084/MJHID.2018.043. View

Behravesh S, Yakes W, Gupta N, Naidu S, Chong B, Khademhosseini A . Venous malformations: clinical diagnosis and treatment. Cardiovasc Diagn Ther. 2017; 6(6):557-569. PMC: 5220204. DOI: 10.21037/cdt.2016.11.10. View

Henzler T, Vogler N, Lange B, Dally F, Meyer M, Schoenberg S . Low dose time-resolved CT-angiography in pediatric patients with venous malformations using 3rd generation dual-source CT: Initial experience. Eur J Radiol Open. 2016; 3:216-22. PMC: 4990662. DOI: 10.1016/j.ejro.2016.08.003. View

Hussein A, Malguria N . Imaging of Vascular Malformations. Radiol Clin North Am. 2020; 58(4):815-830. DOI: 10.1016/j.rcl.2020.02.003. View

Lee E, Biko D, Sherk W, Masch W, Ladino-Torres M, Agarwal P . Understanding Lymphatic Anatomy and Abnormalities at Imaging. Radiographics. 2022; 42(2):487-505. DOI: 10.1148/rg.210104. View

Mamlouk M, Nicholson A, Cooke D, Hess C . Tips and tricks to optimize MRI protocols for cutaneous vascular anomalies. Clin Imaging. 2017; 45:71-80. DOI: 10.1016/j.clinimag.2017.05.019. View

10.

Lee H, Hyun D . Pulmonary Arteriovenous Malformation and Its Vascular Mimickers. Korean J Radiol. 2022; 23(2):202-217. PMC: 8814702. DOI: 10.3348/kjr.2021.0417. View

11.

Chen Y, Li Y, Zhang N, Shang J, Li X, Liu J . Clinical and Imaging Features of Primary Cardiac Angiosarcoma. Diagnostics (Basel). 2020; 10(10). PMC: 7600236. DOI: 10.3390/diagnostics10100776. View

12.

Khalil A, Carette M, CADRANEL J, Mayaud C, Bigot J . Intrathoracic Kaposi's sarcoma. CT findings. Chest. 1995; 108(6):1622-6. DOI: 10.1378/chest.108.6.1622. View

13.

Restrepo C, Martinez S, Lemos J, Carrillo J, Lemos D, Ojeda P . Imaging manifestations of Kaposi sarcoma. Radiographics. 2006; 26(4):1169-85. DOI: 10.1148/rg.264055129. View

14.

Kolarich A, Solomon A, Bailey C, Latif M, Rowan N, Galiatsatos P . Imaging Manifestations and Interventional Treatments for Hereditary Hemorrhagic Telangiectasia. Radiographics. 2021; 41(7):2157-2175. DOI: 10.1148/rg.2021210100. View

15.

Gaballah A, Jensen C, Palmquist S, Pickhardt P, Duran A, Broering G . Angiosarcoma: clinical and imaging features from head to toe. Br J Radiol. 2017; 90(1075):20170039. PMC: 5594986. DOI: 10.1259/bjr.20170039. View

16.

Hu P, Zhou Z . Clinical and imaging features of Kaposiform Hemangioendothelioma. Br J Radiol. 2018; 91(1086):20170798. PMC: 6223283. DOI: 10.1259/bjr.20170798. View

17.

Turner V, Kearns C, Wattamwar K, McKenney A . Klippel-Trenaunay Syndrome. Radiographics. 2022; 42(6):E167-E168. DOI: 10.1148/rg.220150. View

18.

Bertino F, Braithwaite K, Hawkins C, Gill A, Briones M, Swerdlin R . Congenital Limb Overgrowth Syndromes Associated with Vascular Anomalies. Radiographics. 2019; 39(2):491-515. DOI: 10.1148/rg.2019180136. View

19.

Lee S, Ko S, Park W . Macrocystic lymphatic malformation of the chest wall and axilla: A case report in a 45-year-old man. Radiol Case Rep. 2021; 17(1):152-155. PMC: 8601962. DOI: 10.1016/j.radcr.2021.10.001. View

20.

Asano Y, Tada A, Shinya T, Masaoka Y, Iguchi T, Sato S . Utility of second-generation single-energy metal artifact reduction in helical lung computed tomography for patients with pulmonary arteriovenous malformation after coil embolization. Jpn J Radiol. 2018; 36(4):285-294. DOI: 10.1007/s11604-018-0723-6. View