Malignant vascular tumors in children and adolescents

A report from the Italian and German soft tissue sarcoma cooperative group

Andrea Ferrari, Michela Casanova, Gianni Bisogno, Giovanni Cecchetto, Cristina Meazza, Lorenza Gandola, Alberto Garaventa, Adrian Mattke, Jorn Treuner, Modesto Carli

Research output: Contribution to journalArticle

49 Citations (Scopus)

Abstract

Background. Malignant vascular tumors are extremely rare in childhood and few data on their clinical management are available. We report on a series of 18 children who had malignant vascular tumors, treated from 1980 to 2000 by the Italian and German Soft Tissue Sarcoma Cooperative Group. Procedure. Twelve patients had angiosarcoma, four had malignant hemangioendothelioma, and two had Kaposi's sarcoma. Surgical resection was completed in six cases; radiotherapy was administered to 6 children, and chemotherapy to 14. Results. After a median follow-up of 208 months, the 5-year survival and event-free survival rates were 30.9 and 20.8%. Six patients were alive, four in first remission (three had tumor <5 cm, grossly completely resected), and two in second remission. Response to chemotherapy was evaluable in nine cases and was: six no response, two partial remission, one complete remission. Conclusions. Angiosarcoma and related malignant vascular tumors are aggressive neoplasms with a poor prognosis; their behavior in children seems no different from their adult counterparts. Complete surgical resection remains the mainstay of treatment, but is probably sufficient in only a minority of cases. Postoperative radiotherapy may have a role and could be added to improve local control. The role of chemotherapy is uncertain, but the high rate of metastatic spread prompts investigation into new chemotherapeutic approaches.

Original languageEnglish
Pages (from-to)109-114
Number of pages6
JournalMedical and Pediatric Oncology
Volume39
Issue number2
DOIs
Publication statusPublished - 2002

Fingerprint

Sarcoma
Blood Vessels
Hemangiosarcoma
Neoplasms
Drug Therapy
Radiotherapy
Hemangioendothelioma
Kaposi's Sarcoma
Child Behavior
Disease-Free Survival
Survival Rate
Survival
Therapeutics

Keywords

  • Angiosarcoma
  • Children
  • Kaposis sarcoma
  • Malignant hemangioendothelioma
  • Malignant vascular tumor
  • Soft tissue sarcoma

ASJC Scopus subject areas

  • Cancer Research
  • Oncology
  • Pediatrics, Perinatology, and Child Health

Cite this

Malignant vascular tumors in children and adolescents : A report from the Italian and German soft tissue sarcoma cooperative group. / Ferrari, Andrea; Casanova, Michela; Bisogno, Gianni; Cecchetto, Giovanni; Meazza, Cristina; Gandola, Lorenza; Garaventa, Alberto; Mattke, Adrian; Treuner, Jorn; Carli, Modesto.

In: Medical and Pediatric Oncology, Vol. 39, No. 2, 2002, p. 109-114.

Research output: Contribution to journalArticle

@article{d648fccf1af040f4abbd3c3b72522761,
title = "Malignant vascular tumors in children and adolescents: A report from the Italian and German soft tissue sarcoma cooperative group",
abstract = "Background. Malignant vascular tumors are extremely rare in childhood and few data on their clinical management are available. We report on a series of 18 children who had malignant vascular tumors, treated from 1980 to 2000 by the Italian and German Soft Tissue Sarcoma Cooperative Group. Procedure. Twelve patients had angiosarcoma, four had malignant hemangioendothelioma, and two had Kaposi's sarcoma. Surgical resection was completed in six cases; radiotherapy was administered to 6 children, and chemotherapy to 14. Results. After a median follow-up of 208 months, the 5-year survival and event-free survival rates were 30.9 and 20.8{\%}. Six patients were alive, four in first remission (three had tumor <5 cm, grossly completely resected), and two in second remission. Response to chemotherapy was evaluable in nine cases and was: six no response, two partial remission, one complete remission. Conclusions. Angiosarcoma and related malignant vascular tumors are aggressive neoplasms with a poor prognosis; their behavior in children seems no different from their adult counterparts. Complete surgical resection remains the mainstay of treatment, but is probably sufficient in only a minority of cases. Postoperative radiotherapy may have a role and could be added to improve local control. The role of chemotherapy is uncertain, but the high rate of metastatic spread prompts investigation into new chemotherapeutic approaches.",
keywords = "Angiosarcoma, Children, Kaposis sarcoma, Malignant hemangioendothelioma, Malignant vascular tumor, Soft tissue sarcoma",
author = "Andrea Ferrari and Michela Casanova and Gianni Bisogno and Giovanni Cecchetto and Cristina Meazza and Lorenza Gandola and Alberto Garaventa and Adrian Mattke and Jorn Treuner and Modesto Carli",
year = "2002",
doi = "10.1002/mpo.10078",
language = "English",
volume = "39",
pages = "109--114",
journal = "Pediatric Blood and Cancer",
issn = "1545-5009",
publisher = "John Wiley and Sons Inc.",
number = "2",

}

TY - JOUR

T1 - Malignant vascular tumors in children and adolescents

T2 - A report from the Italian and German soft tissue sarcoma cooperative group

AU - Ferrari, Andrea

AU - Casanova, Michela

AU - Bisogno, Gianni

AU - Cecchetto, Giovanni

AU - Meazza, Cristina

AU - Gandola, Lorenza

AU - Garaventa, Alberto

AU - Mattke, Adrian

AU - Treuner, Jorn

AU - Carli, Modesto

PY - 2002

Y1 - 2002

N2 - Background. Malignant vascular tumors are extremely rare in childhood and few data on their clinical management are available. We report on a series of 18 children who had malignant vascular tumors, treated from 1980 to 2000 by the Italian and German Soft Tissue Sarcoma Cooperative Group. Procedure. Twelve patients had angiosarcoma, four had malignant hemangioendothelioma, and two had Kaposi's sarcoma. Surgical resection was completed in six cases; radiotherapy was administered to 6 children, and chemotherapy to 14. Results. After a median follow-up of 208 months, the 5-year survival and event-free survival rates were 30.9 and 20.8%. Six patients were alive, four in first remission (three had tumor <5 cm, grossly completely resected), and two in second remission. Response to chemotherapy was evaluable in nine cases and was: six no response, two partial remission, one complete remission. Conclusions. Angiosarcoma and related malignant vascular tumors are aggressive neoplasms with a poor prognosis; their behavior in children seems no different from their adult counterparts. Complete surgical resection remains the mainstay of treatment, but is probably sufficient in only a minority of cases. Postoperative radiotherapy may have a role and could be added to improve local control. The role of chemotherapy is uncertain, but the high rate of metastatic spread prompts investigation into new chemotherapeutic approaches.

AB - Background. Malignant vascular tumors are extremely rare in childhood and few data on their clinical management are available. We report on a series of 18 children who had malignant vascular tumors, treated from 1980 to 2000 by the Italian and German Soft Tissue Sarcoma Cooperative Group. Procedure. Twelve patients had angiosarcoma, four had malignant hemangioendothelioma, and two had Kaposi's sarcoma. Surgical resection was completed in six cases; radiotherapy was administered to 6 children, and chemotherapy to 14. Results. After a median follow-up of 208 months, the 5-year survival and event-free survival rates were 30.9 and 20.8%. Six patients were alive, four in first remission (three had tumor <5 cm, grossly completely resected), and two in second remission. Response to chemotherapy was evaluable in nine cases and was: six no response, two partial remission, one complete remission. Conclusions. Angiosarcoma and related malignant vascular tumors are aggressive neoplasms with a poor prognosis; their behavior in children seems no different from their adult counterparts. Complete surgical resection remains the mainstay of treatment, but is probably sufficient in only a minority of cases. Postoperative radiotherapy may have a role and could be added to improve local control. The role of chemotherapy is uncertain, but the high rate of metastatic spread prompts investigation into new chemotherapeutic approaches.

KW - Angiosarcoma

KW - Children

KW - Kaposis sarcoma

KW - Malignant hemangioendothelioma

KW - Malignant vascular tumor

KW - Soft tissue sarcoma

UR - http://www.scopus.com/inward/record.url?scp=0036288661&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0036288661&partnerID=8YFLogxK

U2 - 10.1002/mpo.10078

DO - 10.1002/mpo.10078

M3 - Article

VL - 39

SP - 109

EP - 114

JO - Pediatric Blood and Cancer

JF - Pediatric Blood and Cancer

SN - 1545-5009

IS - 2

ER -