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 language | English |
|---|---|
| Pages (from-to) | 109-114 |
| Number of pages | 6 |
| Journal | Medical and Pediatric Oncology |
| Volume | 39 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - 2002 |
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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 journal › Article
}
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
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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 -