Endothelial cell malignancies in infants, children and adolescents: Treatment results of three Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry

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Endothelial cell malignancies in infants, children and adolescents : Treatment results of three Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry

Sparber-Sauer, Monika (author): Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany

Koscielniak, Ewa (author): Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany;Childrens Hosp, Dept Pediat Hematol & Oncol, Tubingen, Germany

Vokuhl, Christian (author): Kiel Pediat Tumor Registry, Sect Pediat Pathol, Dept Pathol, Kiel, Germany

Schmid, Irene (author): Ludwig Maximilians Univ Munchen, Dr Hauner Childrens Hosp, Div Pediat Hematol & Oncol, Munich, Germany

Bien, Ewa (author): Med Univ Gdansk, Dept Pediat Hematol Oncol & Endocrinol, Gdansk, Poland

Seitz, Guido (author): Univ Childrens Hosp Marburg, Dept Pediat Surg, Marburg, Germany

Hallmen, Erika (author): Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany

von Kalle, Thekla (author): Klinikum Stuttgart, Inst Radiol, Stuttgart Canc Ctr,Zentrum Kinder Jugend & Frauen, Olgahosp, Stuttgart, Germany

Scheer, Monika (author): Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany

Muenter, Marc (author): Klinikum Stuttgart, Inst Radiotherapy, Stuttgart, Germany

Bielack, Stefan S. (author): Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany;Univ Munster, Dept Pediat Hematol & Oncol, Munster, Germany

Niggli, Felix (author): Univ Zurich, Dept Pediat Oncol, Zurich, Switzerland

Ljungman, Gustaf, 1958- (author): Uppsala universitet,Barnneurologi/Barnonkologi,Childrens Univ Hosp, Uppsala, Sweden

Fuchs, Joerg (author): Univ Childrens Hosp, Dept Pediat Surg & Urol, Tubingen, Germany

Hettmer, Simone (author): Univ Freiburg, Div Pediat Hematol & Oncol, Dept Pediat & Adolescent Med, Fac Med, Freiburg, Germany

Roessler, Jochen (author): Univ Bern, Univ Klin Kinderheilkunde Hamatol & Onkol, Inselspital, Bern, Switzerland

Klingebiel, Thomas (author): Goethe Univ Frankfurt, Dept Children & Adolescents, Frankfurt, Germany

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(creator_code:org_t)

2019-12-08
2020
English.
In: Pediatric Blood & Cancer. - : WILEY. - 1545-5009 .- 1545-5017. ; 67:3

Related links:: https://urn.kb.se/re...; show more...; https://doi.org/10.1...; show less...

Journal article (peer-reviewed)

Abstract Subject headings

Background Endothelial cell malignancies are extremely rare in childhood. New identification of genetic abnormalities (WWTR1:CAMTA1 translocation) helps to recognize potential therapeutic targets. Little is known about treatment and outcome of these patients. Methods Clinical course, treatment, and outcome in patients with endothelial cell malignancies treated within the Cooperative Weichteilsarkom Studiengruppe (CWS) trials CWS-91, -96, -2002P, and the Soft-Tissue Sarcoma Registry (SoTiSaR) were analyzed (1991-2019). Results Patients had angiosarcoma (AS) (n = 12), malignant epithelioid hemangioendothelioma (EHE) (n = 16), and kaposiform hemangioendothelioma (KHE) (n = 13). The median age was 5.39 years (range, 0.8-17.34); 33 patients had localized disease (LD), and 8 patients had metastatic disease. Therapy consisted of chemotherapy (CHT) (AS n = 8, EHE n = 9, KHE n = 5), interferon or new agent therapy (EHE n = 5, 2 KHE n = 2), microscopically or macroscopically complete resection (AS n = 3, EHE n = 6, KHE n = 3), and radiotherapy (AS n = 6, EHE n = 2, KHE n = 1). Two patients (KHE) had watch-and-wait strategy resulting in stable disease. Complete remission (CR) was achieved in AS (10/12; 83%), EHE (10/16; 63%), and KHE (5/13; 38%). The five-year EFS and OS for patients with AS was 64% (+/- 29 CI 95%) and 80% (+/- 25, CI 95%), with EHE 62% (+/- 24, CI 95%) and 78% (+/- 23, CI 95%), with KHE 33% (+/- 34, CI 95%) and 92% (+/- 15, CI 95%), respectively. Complete resection was a significant prognostic factor for AS, LD for EHE. Conclusions Endothelial cell malignancies in childhood have a fair outcome with multimodal treatment. New treatment options are needed for metastic disease.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)

Keyword

angiosarcoma
children
CWS Group
endothelial cell malignancies
epithelioid hemangioendothelioma
kaposiform hemangioendothelioma

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art (subject category)

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By the author/editor: Sparber-Sauer, M ...; Koscielniak, Ewa; Vokuhl, Christia ...; Schmid, Irene; Bien, Ewa; Seitz, Guido; show more...; Hallmen, Erika; von Kalle, Thekl ...; Scheer, Monika; Muenter, Marc; Bielack, Stefan ...; Niggli, Felix; Ljungman, Gustaf ...; Fuchs, Joerg; Hettmer, Simone; Roessler, Jochen; Klingebiel, Thom ...; show less...

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MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Hematology

Articles in the publication: Pediatric Blood ...

By the university: Uppsala University

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