Malignant vascular tumors in children and adolescents: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group.
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.


METHODS
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.
Istituto Nazionale Tumori, Via G. Venezian 1, Milano MI, Italy. ferrari@istitutotumori.mi.it
Andrea
A
Ferrari
Michela
M
Casanova
Gianni
G
Bisogno
Giovanni
G
Cecchetto
Cristina
C
Meazza
Lorenza
L
Gandola
Alberto
A
Garaventa
Adrian
A
Mattke
Jorn
J
Treuner
Modesto
M
Carli
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D005858:Germany; D006390:Hemangioendothelioma / Q000473:pathology; D006394:Hemangiosarcoma / Q000473:pathology; D006801:Humans; D007223:Infant; D007558:Italy; D009383:Neoplasms, Vascular Tissue / Q000473:pathology* / Q000628:therapy; D011379:Prognosis; D012509:Sarcoma / Q000473:pathology* / Q000628:therapy; D012514:Sarcoma, Kaposi / Q000473:pathology; D016896:Treatment Outcome