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Locally Advanced Epithelioid Hemangioendothelioma

MedGen UID:
1646364
•Concept ID:
C4687513
•
Neoplastic Process

Definition

An epithelioid hemangioendothelioma that has spread from its original site of growth to nearby tissues or lymph nodes. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLocally Advanced Epithelioid Hemangioendothelioma

Professional guidelines

PubMed

Management Strategies for Patients With Epithelioid Hemangioendothelioma: Charting an Indolent Disease Course.
Yurkiewicz IR, Zhou M, Ganjoo KN, Charville GW, Bolleddu S, Lohman M, Bui N
Am J Clin Oncol 2021 Aug 1;44(8):419-422. doi: 10.1097/COC.0000000000000827. PMID: 34028371
Treatment of hepatic epithelioid hemangioendothelioma: a single-institution experience with 25 cases.
Cardinal J, de Vera ME, Marsh JW, Steel JL, Geller DA, Fontes P, Nalesnik M, Gamblin TC
Arch Surg 2009 Nov;144(11):1035-9. doi: 10.1001/archsurg.2009.121. PMID: 19917940
The multidisciplinary management of gastrointestinal cancer. The diagnostic and therapeutic approach for primary solid liver tumours in adults.
Verslype C, Libbrecht L
Best Pract Res Clin Gastroenterol 2007;21(6):983-96. doi: 10.1016/j.bpg.2007.09.006. PMID: 18070699

Recent clinical studies

Etiology

Vascular Neoplasms With NFATC1/C2 Gene Alterations : Expanding the Clinicopathologic and Molecular Characteristics of a Distinct Entity.
Dashti NK, Perret R, Balzer B, Naous R, Michal M, Dermawan JK, Antonescu CR
Am J Surg Pathol 2024 Apr 1;48(4):487-496. Epub 2024 Jan 8 doi: 10.1097/PAS.0000000000002175. PMID: 38189436
Epithelioid hemangio-endothelioma (EHE) in NETSARC: The nationwide series of 267 patients over 12 years.
Blay JY, Piperno-Neumann S, Watson S, Dufresne A, Valentin T, Duffaud F, Toulmonde M, Italiano A, Bertucci F, Tlemsani C, Firmin N, Bompas E, Perrin C, Ropars M, Saada-Bouzid E, Dubray-Longeras P, Hervieu A, Lebbe C, Gantzer J, Chaigneau L, Fiorenza F, Rios M, Isambert N, Soibinet P, Boudou-Roquette P, Verret B, Ferron G, Ryckewaert T, Lebellec L, Brahmi M, Gouin F, Meeus P, Vaz G, Le Loarer F, Karanian M, De Pinieux G, Ducimetiere F, Chemin C, Morelle M, Le Cesne A, Penel N; NETSARC/REPPS/RESOS and French Sarcoma Group–Groupe d′Etude des Tumeurs Osseuses (GSF-GETO) networks
Eur J Cancer 2023 Oct;192:113262. Epub 2023 Jul 29 doi: 10.1016/j.ejca.2023.113262. PMID: 37625241
Management Strategies for Patients With Epithelioid Hemangioendothelioma: Charting an Indolent Disease Course.
Yurkiewicz IR, Zhou M, Ganjoo KN, Charville GW, Bolleddu S, Lohman M, Bui N
Am J Clin Oncol 2021 Aug 1;44(8):419-422. doi: 10.1097/COC.0000000000000827. PMID: 34028371
Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature.
Frezza AM, Ravi V, Lo Vullo S, Vincenzi B, Tolomeo F, Chen TW, Teterycz P, Baldi GG, Italiano A, Penel N, Brunello A, Duffaud F, Hindi N, Iwata S, Smrke A, Fedenko A, Gelderblom H, Van Der Graaf W, Vozy A, Connolly E, Grassi M, Benjamin RS, Broto JM, Grignani G, Jones RL, Kawai A, Tysarowski A, Mariani L, Casali PG, Stacchiotti S
Cancer Med 2021 Apr;10(8):2645-2659. Epub 2021 Mar 13 doi: 10.1002/cam4.3807. PMID: 33713582Free PMC Article
Sirolimus in Advanced Epithelioid Hemangioendothelioma: A Retrospective Case-Series Analysis from the Italian Rare Cancer Network Database.
Stacchiotti S, Provenzano S, Dagrada G, Negri T, Brich S, Basso U, Brunello A, Grosso F, Galli L, Palassini E, Libertini M, Colia V, Gronchi A, Dei Tos AP, Crippa F, Morosi C, Pilotti S, Casali PG
Ann Surg Oncol 2016 Sep;23(9):2735-44. Epub 2016 Jun 22 doi: 10.1245/s10434-016-5331-z. PMID: 27334221

Diagnosis

Epithelioid hemangio-endothelioma (EHE) in NETSARC: The nationwide series of 267 patients over 12 years.
Blay JY, Piperno-Neumann S, Watson S, Dufresne A, Valentin T, Duffaud F, Toulmonde M, Italiano A, Bertucci F, Tlemsani C, Firmin N, Bompas E, Perrin C, Ropars M, Saada-Bouzid E, Dubray-Longeras P, Hervieu A, Lebbe C, Gantzer J, Chaigneau L, Fiorenza F, Rios M, Isambert N, Soibinet P, Boudou-Roquette P, Verret B, Ferron G, Ryckewaert T, Lebellec L, Brahmi M, Gouin F, Meeus P, Vaz G, Le Loarer F, Karanian M, De Pinieux G, Ducimetiere F, Chemin C, Morelle M, Le Cesne A, Penel N; NETSARC/REPPS/RESOS and French Sarcoma Group–Groupe d′Etude des Tumeurs Osseuses (GSF-GETO) networks
Eur J Cancer 2023 Oct;192:113262. Epub 2023 Jul 29 doi: 10.1016/j.ejca.2023.113262. PMID: 37625241
Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature.
Frezza AM, Ravi V, Lo Vullo S, Vincenzi B, Tolomeo F, Chen TW, Teterycz P, Baldi GG, Italiano A, Penel N, Brunello A, Duffaud F, Hindi N, Iwata S, Smrke A, Fedenko A, Gelderblom H, Van Der Graaf W, Vozy A, Connolly E, Grassi M, Benjamin RS, Broto JM, Grignani G, Jones RL, Kawai A, Tysarowski A, Mariani L, Casali PG, Stacchiotti S
Cancer Med 2021 Apr;10(8):2645-2659. Epub 2021 Mar 13 doi: 10.1002/cam4.3807. PMID: 33713582Free PMC Article
The 2015 World Health Organization Classification of Tumors of the Pleura: Advances since the 2004 Classification.
Galateau-Salle F, Churg A, Roggli V, Travis WD; World Health Organization Committee for Tumors of the Pleura
J Thorac Oncol 2016 Feb;11(2):142-54. doi: 10.1016/j.jtho.2015.11.005. PMID: 26811225
Diagnostic and therapeutic challenges in hepatic epithelioid hemangioendothelioma.
Mistry AM, Gorden DL, Busler JF, Coogan AC, Kelly BS
J Gastrointest Cancer 2012 Dec;43(4):521-5. doi: 10.1007/s12029-012-9389-y. PMID: 22544493
Focal liver lesions: MR imaging-pathologic correlation.
Bartolozzi C, Cioni D, Donati F, Lencioni R
Eur Radiol 2001;11(8):1374-88. doi: 10.1007/s003300100845. PMID: 11519546

Therapy

A Single-Arm Phase 2 Trial of Trametinib in Patients with Locally Advanced or Metastatic Epithelioid Hemangioendothelioma.
Schuetze SM, Ballman KV, Heise R, Ganjoo KN, Davis EJ, George S, Burgess MA, Choy E, Shepard DR, Tinoco G, Hirbe A, Kelly CM, Attia S, Deshpande HA, Schwartz GK, Siontis BL, Riedel RF, von Mehren M, Kozlowski E, Chen HX, Astbury C, Rubin BP
Clin Cancer Res 2024 Oct 15;30(20):4584-4592. doi: 10.1158/1078-0432.CCR-23-3817. PMID: 38446990Free PMC Article
Management Strategies for Patients With Epithelioid Hemangioendothelioma: Charting an Indolent Disease Course.
Yurkiewicz IR, Zhou M, Ganjoo KN, Charville GW, Bolleddu S, Lohman M, Bui N
Am J Clin Oncol 2021 Aug 1;44(8):419-422. doi: 10.1097/COC.0000000000000827. PMID: 34028371
Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature.
Frezza AM, Ravi V, Lo Vullo S, Vincenzi B, Tolomeo F, Chen TW, Teterycz P, Baldi GG, Italiano A, Penel N, Brunello A, Duffaud F, Hindi N, Iwata S, Smrke A, Fedenko A, Gelderblom H, Van Der Graaf W, Vozy A, Connolly E, Grassi M, Benjamin RS, Broto JM, Grignani G, Jones RL, Kawai A, Tysarowski A, Mariani L, Casali PG, Stacchiotti S
Cancer Med 2021 Apr;10(8):2645-2659. Epub 2021 Mar 13 doi: 10.1002/cam4.3807. PMID: 33713582Free PMC Article
Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma.
van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG
Clin Cancer Res 2018 Jun 1;24(11):2678-2687. Epub 2018 Mar 6 doi: 10.1158/1078-0432.CCR-17-3512. PMID: 29511030
Sirolimus in Advanced Epithelioid Hemangioendothelioma: A Retrospective Case-Series Analysis from the Italian Rare Cancer Network Database.
Stacchiotti S, Provenzano S, Dagrada G, Negri T, Brich S, Basso U, Brunello A, Grosso F, Galli L, Palassini E, Libertini M, Colia V, Gronchi A, Dei Tos AP, Crippa F, Morosi C, Pilotti S, Casali PG
Ann Surg Oncol 2016 Sep;23(9):2735-44. Epub 2016 Jun 22 doi: 10.1245/s10434-016-5331-z. PMID: 27334221

Prognosis

Epithelioid hemangioendothelioma-its history, clinical features, molecular biology and current therapy.
Tsuchihashi K, Baba E
Jpn J Clin Oncol 2024 Jul 7;54(7):739-747. doi: 10.1093/jjco/hyae037. PMID: 38555494
Management Strategies for Patients With Epithelioid Hemangioendothelioma: Charting an Indolent Disease Course.
Yurkiewicz IR, Zhou M, Ganjoo KN, Charville GW, Bolleddu S, Lohman M, Bui N
Am J Clin Oncol 2021 Aug 1;44(8):419-422. doi: 10.1097/COC.0000000000000827. PMID: 34028371
Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature.
Frezza AM, Ravi V, Lo Vullo S, Vincenzi B, Tolomeo F, Chen TW, Teterycz P, Baldi GG, Italiano A, Penel N, Brunello A, Duffaud F, Hindi N, Iwata S, Smrke A, Fedenko A, Gelderblom H, Van Der Graaf W, Vozy A, Connolly E, Grassi M, Benjamin RS, Broto JM, Grignani G, Jones RL, Kawai A, Tysarowski A, Mariani L, Casali PG, Stacchiotti S
Cancer Med 2021 Apr;10(8):2645-2659. Epub 2021 Mar 13 doi: 10.1002/cam4.3807. PMID: 33713582Free PMC Article
The 2015 World Health Organization Classification of Tumors of the Pleura: Advances since the 2004 Classification.
Galateau-Salle F, Churg A, Roggli V, Travis WD; World Health Organization Committee for Tumors of the Pleura
J Thorac Oncol 2016 Feb;11(2):142-54. doi: 10.1016/j.jtho.2015.11.005. PMID: 26811225
Focal liver lesions: MR imaging-pathologic correlation.
Bartolozzi C, Cioni D, Donati F, Lencioni R
Eur Radiol 2001;11(8):1374-88. doi: 10.1007/s003300100845. PMID: 11519546

Clinical prediction guides

Epithelioid hemangioendothelioma-its history, clinical features, molecular biology and current therapy.
Tsuchihashi K, Baba E
Jpn J Clin Oncol 2024 Jul 7;54(7):739-747. doi: 10.1093/jjco/hyae037. PMID: 38555494
A Single-Arm Phase 2 Trial of Trametinib in Patients with Locally Advanced or Metastatic Epithelioid Hemangioendothelioma.
Schuetze SM, Ballman KV, Heise R, Ganjoo KN, Davis EJ, George S, Burgess MA, Choy E, Shepard DR, Tinoco G, Hirbe A, Kelly CM, Attia S, Deshpande HA, Schwartz GK, Siontis BL, Riedel RF, von Mehren M, Kozlowski E, Chen HX, Astbury C, Rubin BP
Clin Cancer Res 2024 Oct 15;30(20):4584-4592. doi: 10.1158/1078-0432.CCR-23-3817. PMID: 38446990Free PMC Article
Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature.
Frezza AM, Ravi V, Lo Vullo S, Vincenzi B, Tolomeo F, Chen TW, Teterycz P, Baldi GG, Italiano A, Penel N, Brunello A, Duffaud F, Hindi N, Iwata S, Smrke A, Fedenko A, Gelderblom H, Van Der Graaf W, Vozy A, Connolly E, Grassi M, Benjamin RS, Broto JM, Grignani G, Jones RL, Kawai A, Tysarowski A, Mariani L, Casali PG, Stacchiotti S
Cancer Med 2021 Apr;10(8):2645-2659. Epub 2021 Mar 13 doi: 10.1002/cam4.3807. PMID: 33713582Free PMC Article
Sirolimus in Advanced Epithelioid Hemangioendothelioma: A Retrospective Case-Series Analysis from the Italian Rare Cancer Network Database.
Stacchiotti S, Provenzano S, Dagrada G, Negri T, Brich S, Basso U, Brunello A, Grosso F, Galli L, Palassini E, Libertini M, Colia V, Gronchi A, Dei Tos AP, Crippa F, Morosi C, Pilotti S, Casali PG
Ann Surg Oncol 2016 Sep;23(9):2735-44. Epub 2016 Jun 22 doi: 10.1245/s10434-016-5331-z. PMID: 27334221
Focal liver lesions: MR imaging-pathologic correlation.
Bartolozzi C, Cioni D, Donati F, Lencioni R
Eur Radiol 2001;11(8):1374-88. doi: 10.1007/s003300100845. PMID: 11519546

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