Medical Abstracts

The best way to understand the current state of the art in cause, natural history and treatment of a disease is through the medical literature. A useful, standard search engine can be found at PubMed:

A recent search of ‘epithelioid hemangioendothelioma’ pulled 945 papers dating from 1982. I’ve listed a few helpful papers as a starting point for the uninitiated. I have not included case reports for they are essentially not useful in understanding patterns beyong the specific individual reported and are therefore not generalizable; n or reports of medical treatment since there are no randomized controlled trials at this point. Therefore, the listed papers emphasize epidemiologic (outcome) and surgical studies which are long enough or sizable enough to be meaningful and/or have general significance. This is not to say medical treatment isn’t potentially helpful; just that there are not sufficient studies at this point to say they are. That will  hopefully change some day. I’ve also included two papers each on the underlying genetic alteration in EHE and the theoretical association with Bartonella infection. The link after the citation can be copied into your browser to take you to the Abstract in PubMed.



Some classic papers:


Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982 Sep 1;50(5):970-81.

Classic description of EHE.


Ishak KG, Sesterhenn IA, Goodman ZD, Rabin L, Stromeyer FW. Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases.Hum Pathol. 1984 Sep;15(9):839-52.

Early study of clinical outcomes


Mehrabi A, Kashfi A, Schemmer P, Sauer P, Encke J, Fonouni H, Friess H, Weitz J, Schmidt J, Büchler MW, Kraus TW. Surgical treatment of primary hepatic epithelioid hemangioendothelioma.Transplantation. 2005 Sep 27;80(1 Suppl):S109-12.




Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP. Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma.Sci Transl Med. 2011 Aug 31;3(98):98ra82.

Errani C, Zhang L, Sung YS, Hajdu M, Singer S, Maki RG, Healey JH, Antonescu CR.A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes Chromosomes Cancer. 2011 Aug;50(8):644-53. doi: 10.1002/gcc.20886. Epub 2011 May 16.



Some transplant papers

Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases.Cancer. 1999 Feb 1;85(3):562-82.

Early study of clinical outcomes.


Madariaga JR, Marino IR, Karavias DD, Nalesnik MA, Doyle HR, Iwatsuki S, Fung JJ, Starzl TE. Long-term results after liver transplantation for primary hepatic epithelioid hemangioendothelioma.Ann Surg Oncol. 1995 Nov;2(6):483-7.

Early study of outcomes from liver transplant indicating good survival even in the setting of metastatic disease.


Guiteau JJ, Cotton RT, Karpen SJ, O'Mahony CA, Goss JA. Pediatric liver transplantation for primary malignant liver tumors with a focus on hepatic epithelioid hemangioendothelioma: the UNOS experience.Pediatr Transplant. 2010 May;14(3):326-31. Epub 2009 Dec 30.


Lerut JP, Orlando G, Sempoux C, Ciccarelli O, Van Beers BE, Danse E, Horsmans Y, Rahier J, Roggen F. Hepatic haemangioendothelioma in adults: excellent outcome following liver transplantation. Transpl Int. 2004 May;17(4):202-7. Epub 2004 Apr 28.


Agrawal N, Parajuli S, Zhao P, Satoskar R, Laurin J, Azumi N, Matsumoto C, Shetty K. Liver transplantation in the management of hepaticepithelioid hemangioendothelioma: a single-center experience and review of the literature. Transplant Proc. 2011 Sep;43(7):2647-50


Lerut JP, Orlando G, Adam R, Schiavo M, Klempnauer J, Mirza D, Boleslawski E, Burroughs A, Sellés CF, Jaeck D, Pfitzmann R, Salizzoni M, Söderdahl G, Steininger R, Wettergren A, Mazzaferro V, Le Treut YP, Karam V, The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: report of the European liver transplant registry.Ann Surg. 2007 Dec;246(6):949-57.


Nudo CG, Yoshida EM, Bain VG, Marleau D, Wong P, Marotta PJ, Renner E, Watt KD, Deschênes M. Liver transplantation for hepatic epithelioid hemangioendothelioma: the Canadian multicentre experience.Can J Gastroenterol. 2008 Oct;22(10):821-4.


Rodriguez JA, Becker NS, O'Mahony CA, Goss JA, Aloia TA. Long-term outcomes following liver transplantation for hepatic hemangioendothelioma: the UNOS experience from 1987 to 2005.J Gastrointest Surg. 2008 Jan;12(1):110-6. Epub 2007 Aug 21.



Some general clinical experience papers


Lau K, Massad M, Pollak C, Rubin C, Yeh J, Wang J, Edelman G, Yeh J, Prasad S, Weinberg G. Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer. Chest. 2011 Nov;140(5):1312-8. Epub 2011 May 5.


Wang LR, Zhou JM, Zhao YM, He HW, Chai ZT, Wang M, Ji Y, Chen Y, Liu C, Sun HC, Wu WZ, Ye QH, Zhou J, Fan J, Tang ZY, Wang L. Clinical experience with primary hepaticepithelioid hemangioendothelioma: retrospective study of 33 patients. World J Surg. 2012 Nov;36(11):2677-83. doi: 10.1007/s00268-012-1714-x.


Cardinal J, de Vera ME, Marsh JW, Steel JL, Geller DA, Fontes P, Nalesnik M, Gamblin TC. Treatment of hepatic epithelioid hemangioendothelioma: a single-institution experience with 25 cases.Arch Surg. 2009 Nov;144(11):1035-9.


Mosoia L, Mabrut JY, Adham M, Boillot O, Ducerf C, Partensky C, Baulieux J. Hepatic epithelioid hemangioendothelioma: long-term results of surgical management.J Surg Oncol. 2008 Nov 1;98(6):432-7.


Mascarelli PE, Iredell JR, Maggi RG, Weinberg G, Breitschwerdt EB. Bartonella species bacteremia in two patients with epithelioid hemangioendothelioma.J Clin Microbiol. 2011 Nov;49(11):4006-12. Epub 2011 Sep 14.



Hypothetical Bartonella association


Breitschwerdt EB, Maggi RG, Varanat M, Linder KE, Weinberg G. Isolation of Bartonella vinsonii subsp. berkhoffii genotype II from a boy with epithelioid hemangioendothelioma and a dog with hemangiopericytoma.J Clin Microbiol. 2009 Jun;47(6):1957-60. Epub 2009 Apr 15.


Mascarelli PE, Iredell JR, Maggi RG, Weinberg G, Breitschwerdt EB.Bartonella species bacteremia in two patients with epithelioid hemangioendothelioma.J Clin Microbiol. 2011 Nov;49(11):4006-12. Epub 2011 Sep 14.