A review of 20 years of experience in the Italian and German Soft Tissue Sarcoma Cooperative Group identified 12 children with angiosarcoma. One objective response to chemotherapy was observed, and the overall behavior of this tumor was identical to angiosarcoma in adults. A subsequent retrospective study of 14 children with angiosarcoma performed by the Polish and German Cooperative Paediatric Soft Tissue Sarcoma Study Groups identified four chemotherapy responses in ten children. Another review of 15 patients demonstrated a 33% survival rate.
Anti-angiogenesis therapy may prove useful in the treatment of this group of neoplasms.
Hemangioendotheliomas are tumors found in infants that arise within the liver or elsewhere and usually remain benign.[153,154]
The tumors are sometimes associated with consumptive coagulopathy, also known as the Kasabach-Merritt syndrome (or phenomenon).[155,156,157]; [Level of evidence: 3iiiDiv] Chemotherapy and interferon have had some benefit in isolated cases.[156,157] In older children and adults, hemangioendotheliomas may occur elsewhere in the body and can metastasize to lungs, lymph nodes, bones, and within the pleural or peritoneal cavities. The preferred pathologic designation for these lesions in older persons is epithelioid hemangioendothelioma, which connotes the possibility of distant spread. These latter lesions are considered to be of intermediate malignant potential, between benign hemangioma and angiosarcoma.[159,160] In more extensive hemangioendothelioma, inhibition of mTOR pathway may be helpful. However, this should be investigated as part of a clinical trial before use in the clinical setting.
Treatment of asymptomatic liver hemangioendothelioma in a child younger than 1 year may include close observation, because some tumors will regress. Symptomatic lesions require urgent medical or surgical management, especially if coagulopathy is present.[153,155,156,157] Epithelioid hemangioendothelioma of the liver should be managed surgically. Some patients may need orthotopic liver transplantation because this disease does not respond to radiation therapy or chemotherapy.
Liver hemangioendotheliomas may regress and then enlarge; these tumors may become malignant. Of five girls with liver angiosarcoma, three were infants younger than 4 months with cutaneous hemangiomas; two also had multinodular liver hemangiomas. Liver size initially decreased; however, at age 2.5 to 5 years, their livers enlarged, and all three girls died of angiosarcoma. Two other girls presented with vascular liver tumors at age 2 and 3.5 years, without previous histories. The younger girl had a benign unifocal hemangioendothelioma on biopsy; 3 months later, another biopsy showed both benign and malignant histology, and she died. The older girl had multinodular angiosarcomas without metastases, underwent liver transplantation, and was recurrence free 2 years later. Chemotherapy and radiation therapy are ineffective for angiosarcoma. The authors recommend liver ultrasound surveillance every 6 months for infants with multinodular liver hemangiomas; and liver transplantation, if feasible, for angiosarcoma if no metastases are found.