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.
|Titolo:||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.|
|Data di pubblicazione:||2013|
|Citazione:||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. / Cavaliere, E; Cecchetto, G; Ferrari, A; Basso, Me; Garaventa, A; Dall’Igna, P; Alaggio, R; DI CATALDO, Andrea; D’Angelo, P; Bisogno, G.. - 60:S3(2013), pp. 109-109. ((Intervento presentato al convegno 45th Congress of The International Society of Paediatric Oncology (SIOP) tenutosi a Hong Kong, China nel 25-28 September 2013.|
|Appare nelle tipologie:||4.2 Abstract in Atti di convegno|