Purpose/Objective: Undifferentiated sarcoma of the liver (UESL) is the third most commonhepatic tumor in children. In 2002 we reported a long-term disease-free survival rate of 70%.on 17 children with UESL treated with the same multimodal approach. Therefore, adedicated protocol for children with UESL was designed.We describe the results obtained in9 children so far treated.Materials and Methods: From 1996 to 2012 children were treated with the samemultimodal approach: initial surgery/biopsy at diagnosis, chemotherapy (CT) according tothe VAIA (vincristine, actinomycin, ifosfamide and adriamycin) regimen, diseaseevaluation after the initial 3 cycles, second-look surgery. A total of 9 CT cycles wereadministeredResults: Male/female ¼ 6/3. Median age: 8.3 years (range, 3.7-14.3). Clinical presentation:abdominal mass (n ¼ 4), acute abdominal pain (n ¼ 4), abdominal distention (n ¼ 4) withassociated fever in 3, shoulder sore associated with serotine fever (n ¼ 1), infection withfever (n ¼ 2). Tumor involvement: right lobe (n ¼ 7), left lobe (n ¼ 1), both (n ¼ 1). Tumorsize: 5-10 cm (n ¼ 2), > 10 cm (n ¼ 7). Lung metastases were evident in 1 child.Liver tumor resection was attempted at diagnosis in 5 children: 3 complete (1 metastaticpatient), 1 with microscopic and 1 with macroscopic residue. Biopsy was performed in 4.Response to CT was evaluable in 5 patients: partial response (n ¼ 2), minor response(n ¼ 1), tumor stable (n ¼ 2). Second-look surgery resulted complete in 6 children.Radiotherapy was administered in 2 cases. Outcome (follow-up of 3-13 yrs): all patients arealive in 1st CR of their UESL but 2 children developed a second malignancy (Ewing sarcoma,hemangioendothelioma).Conclusions: Our update confirms the good prognosis of children with UESL when aneffective multidisciplinary protocol is utilized. UESL patients seems at high risk of secondmalignancy.
Undifferentiated sarcoma of the liver in childhood: an update. Pediatr Blood Cancer 60S3;109,2014 (XXXXV Meeting SIOP, Hong Kong, China, 25-28 September, 2013.
DI CATALDO, Andrea;
2013-01-01
Abstract
Purpose/Objective: Undifferentiated sarcoma of the liver (UESL) is the third most commonhepatic tumor in children. In 2002 we reported a long-term disease-free survival rate of 70%.on 17 children with UESL treated with the same multimodal approach. Therefore, adedicated protocol for children with UESL was designed.We describe the results obtained in9 children so far treated.Materials and Methods: From 1996 to 2012 children were treated with the samemultimodal approach: initial surgery/biopsy at diagnosis, chemotherapy (CT) according tothe VAIA (vincristine, actinomycin, ifosfamide and adriamycin) regimen, diseaseevaluation after the initial 3 cycles, second-look surgery. A total of 9 CT cycles wereadministeredResults: Male/female ¼ 6/3. Median age: 8.3 years (range, 3.7-14.3). Clinical presentation:abdominal mass (n ¼ 4), acute abdominal pain (n ¼ 4), abdominal distention (n ¼ 4) withassociated fever in 3, shoulder sore associated with serotine fever (n ¼ 1), infection withfever (n ¼ 2). Tumor involvement: right lobe (n ¼ 7), left lobe (n ¼ 1), both (n ¼ 1). Tumorsize: 5-10 cm (n ¼ 2), > 10 cm (n ¼ 7). Lung metastases were evident in 1 child.Liver tumor resection was attempted at diagnosis in 5 children: 3 complete (1 metastaticpatient), 1 with microscopic and 1 with macroscopic residue. Biopsy was performed in 4.Response to CT was evaluable in 5 patients: partial response (n ¼ 2), minor response(n ¼ 1), tumor stable (n ¼ 2). Second-look surgery resulted complete in 6 children.Radiotherapy was administered in 2 cases. Outcome (follow-up of 3-13 yrs): all patients arealive in 1st CR of their UESL but 2 children developed a second malignancy (Ewing sarcoma,hemangioendothelioma).Conclusions: Our update confirms the good prognosis of children with UESL when aneffective multidisciplinary protocol is utilized. UESL patients seems at high risk of secondmalignancy.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
