Epithelioid hemangioendothelioma in children: The European Pediatric Soft Tissue Sarcoma Study Group experience

被引:3
作者
Orbach, Daniel [1 ]
Van Noesel, Max M. [2 ,3 ]
Brennan, Bernadette [4 ]
Corradini, Nadege [5 ]
Alaggio, Rita [6 ]
Ben Arush, Myriam [7 ]
Schoot, Reineke A. [2 ,3 ]
Berlanga, Pablo [8 ]
Zanetti, Ilaria [9 ]
Hjalgrim, Lisa Lyngsie [10 ]
Di Corti, Federica [11 ]
Ramirez, Gema [12 ]
Casanova, Michela [13 ]
Ferrari, Andrea [13 ]
机构
[1] PSL Univ, Inst Curie, Care Innovat & Res Children Adolescents & Young A, SIREDO Oncol Ctr Care, Paris, France
[2] Princess Maxima Ctr Pediat Oncol, Utrecht, Netherlands
[3] Univ Med Ctr Utrecht, Div Canc & Imaging, Utrecht, Netherlands
[4] Royal Manchester Childrens Hosp, Paediat Oncol, Manchester, Lancs, England
[5] Leon Berard Ctr, IHOPe, Dept Pediat Hematol & Oncol, Lyon, France
[6] Osped Pediat Bambino Gesu IRCCS, Pathol Dept, Rome, Italy
[7] Ruth Rappaport Childrens Hosp, Haifa, Israel
[8] Univ Paris Saclay, Gustave Roussy Canc Campus, Dept Pediat & Adolescent Oncol, Villejuif, France
[9] Padova Univ Hosp, Dept Womens & Childrens Hlth, Haematol Oncol Div, Padua, Italy
[10] Univ Copenhagen, Rigshosp, Dept Pediat & Adolescent Med, Copenhagen, Denmark
[11] Univ Hosp Padua, Women & Childrens Hlth Dept, Pediat Surg, Padua, Italy
[12] Pediat Hosp Virgen del Rocio, Pediat Oncol & Hematol, Seville, Spain
[13] Fdn IRCCS Ist Nazl Tumori, Pediat Oncol Unit, Milan, Italy
关键词
children; hemangioendothelioma; soft tissue sarcoma; surgery;
D O I
10.1002/pbc.29882
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
The European pediatric Soft tissue sarcoma Study Group analyzed all children with epithelioid hemangioendothelioma prospectively registered in the NRSTS-05 (EUDRACT 2005-001139-31) and in MTS-2008 (NCT00379457) studies: 10 patients with localized and one with metastatic disease. Median age was 14.3 years (range, 9.0-18.8). Local therapy was initial primary surgery in seven cases, and five patients received systemic therapy. No patients received radiotherapy. After a median follow-up of 50 months (range, 6-176) for living patients, nine patients remain alive off therapy and two died. Five-year progression free and overall survivals are, respectively, 77.1% (95% confidence interval [CI]: 34.5-93.9) and 74.1% (95% CI: 28.1-93.0).
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页数:4
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