Maciej Murawski1, Monika Scheer2, Ivo Leuschner2,3, Joanna Stefanowicz4, Jolanta Bonar5, Bożenna Dembowska-Bagińska6, Piotr Kaliciński7, Ewa Koscielniak2, Piotr Czauderna1, Jörg Fuchs8. 1. Deptartment of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Gdansk, Poland. 2. Department of Pediatric Hematology, Oncology and Immunology, Olgahospital, Klinikum Stuttgart, Germany. 3. Department of Pediatric Pathology, University Hospital of Schleswig-Holstein, Kiel, Germany. 4. Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland. 5. Department of Bone Marrow Transplantation, Children Oncology and Haematology, Medical University of Wroclaw, Wroclaw, Poland. 6. Department of Pediatric Oncology, Children's Memorial Health Institute, Warsaw, Poland. 7. Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, Warsaw, Poland. 8. Department of Pediatric Surgery and Pediatric Urology, University Children's Hospital, Tuebingen, Germany.
Abstract
BACKGROUND: Undifferentiated embryonal sarcomas of the liver (UESL) are extremely rare and continue to pose a diagnostic and therapeutic challenge. The aim of the study was to present a multicenter experience of the German CWS and Polish PPSTG groups in the treatment of UESL in children. PROCEDURE: Twenty-five patients were treated according to the CWS-96, CWS-2002, and CYVADIC protocols. Distant metastases were observed in four cases (16%). In four cases, an initial disease presentation mimicked other entities. A pure cystic appearance of liver mass led to misdiagnosis of hydatid cyst in three cases. In one case, laparotomy was performed due to the signs of appendicitis, and bleeding from ruptured liver tumor was found. All these patients were finally diagnosed as UESL. RESULTS: Thirteen patients received preoperative chemotherapy. Partial response was observed in 10 cases. Tumor resection was performed in 20 patients (primary resections, 12; delayed resections-, 8). In five patients, the primary tumor never became operable. The macroscopically complete resection rate was 95% (19/20). Postoperative chemotherapy was given to 20 children. Local radiotherapy was used in three children. After a median follow-up time of 136 months, 17 patients (68%) were alive with no evidence of disease. All children with unresectable tumor and three out of four patients with distant metastases died. The five-year overall survival (OS) rate was 72%. CONCLUSIONS: In summary, a complete tumor excision plays the central role in the treatment of UESL. A cystic presentation of the liver lesion on imaging does not exclude the diagnosis of malignant tumor.
BACKGROUND:Undifferentiated embryonal sarcomas of the liver (UESL) are extremely rare and continue to pose a diagnostic and therapeutic challenge. The aim of the study was to present a multicenter experience of the German CWS and Polish PPSTG groups in the treatment of UESL in children. PROCEDURE: Twenty-five patients were treated according to the CWS-96, CWS-2002, and CYVADIC protocols. Distant metastases were observed in four cases (16%). In four cases, an initial disease presentation mimicked other entities. A pure cystic appearance of liver mass led to misdiagnosis of hydatid cyst in three cases. In one case, laparotomy was performed due to the signs of appendicitis, and bleeding from ruptured liver tumor was found. All these patients were finally diagnosed as UESL. RESULTS: Thirteen patients received preoperative chemotherapy. Partial response was observed in 10 cases. Tumor resection was performed in 20 patients (primary resections, 12; delayed resections-, 8). In five patients, the primary tumor never became operable. The macroscopically complete resection rate was 95% (19/20). Postoperative chemotherapy was given to 20 children. Local radiotherapy was used in three children. After a median follow-up time of 136 months, 17 patients (68%) were alive with no evidence of disease. All children with unresectable tumor and three out of four patients with distant metastases died. The five-year overall survival (OS) rate was 72%. CONCLUSIONS: In summary, a complete tumor excision plays the central role in the treatment of UESL. A cystic presentation of the liver lesion on imaging does not exclude the diagnosis of malignant tumor.