Pediatric Malignant Liver Tumors: Results of a Single Center

Primary malignant tumors of the liver are rare in childhood and comprise 1-2% of all childhood malignancies. In this retrospective study the characteristics, treatment and outcome of childhood malignant hepatic tumors diagnosed and treated in a single center between 1996 and 2010 were evaluated. The pretreatment extent of disease (PRETEXT) system was used for staging and the SI-OPEL-3 or modified protocol for treatment. 20 children (13 male, 7 female), diagnosed and treated with primary malignant tumors of the liver (11 hepatocellular carcinoma [HCC], 9 hepatoblastoma [HBS]) were evaluated. In our series the cases with HCC were more than the cases with HBS. In cases with HCC, 8 had chronic hepatitis B infection. Serum alpha-fetoprotein levels were elevated in all cases with HBS and in six with HCC. According to the PRETEXT staging, 12 cases were stage (S) IV, 2 S III, 6 S II. Four had lung metastasis. Four cases with HBS, one patient with HCC are alive with no evidence of disease (19, 20, 21, 26, 102 months). The high incidence of hepatocellular carcinoma in our series is thought to be due to the high incidence of hepatitis B infection in Turkey. Children with chronic hepatitis B virus infection should be followed up keeping in mind the risk of HCC. Children with hemihypertrophia and other predisposing conditions should be followed up for the risk of liver tumors so that they can be diagnosed at early stages

Testis Involvement in Neuroblastoma: Report of 3 Cases in the Turkish Pediatric Oncology Group-Neuroblastoma Study and Review of the Literature

Background: Neuroblastoma (NB) is the most common extracranial solid tumor of childhood. Primary and secondary testicular involvement is extremely uncommon in neuroblastoma. Procedure: All children with neuroblastoma treated with the Turkish Pediatric Oncology Group (TPOG)-Neuroblastoma (NB) Study and who had testis involvement either at diagnosis or at relapse were retrospectively evaluated. A review of all cases with neuroblastoma and testis involvement in the literature was done. Results: There were 3 children with NB documented to have involvement of the testis, 2 at diagnosis, 1 at recurrence, within the 559 cases (0.5%) treated with the Turkish Pediatric Oncology Group (TPOG)-Neuroblastoma Protocol. All had advanced stage. Two were infants. A total of 57 cases of testicular or paratesticular neuroblastoma have been reported in children, and most cases represent metastases as in the 3 cases in our series. Conclusions: Neuroblastoma should be considered in the differential diagnosis of testicular mass and work-up for neuroblastoma should be done before orchiectomy. Scrotal ultrasonography should be used as the first diagnostic tool and abdominal ultrasonography shall be done additionally. Testis examination should be performed at diagnosis and regularly during follow-up for boys diagnosed with neuroblastoma. Testes may be sanctuary sites when neuroblastoma is metastatic, as is the case in leukemia

TREATMENT OF WILMS TUMOR: A Report from the Turkish Pediatric Oncology Group (TPOG)

WOS: 000278808800001PubMed ID: 20367260Aim: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. Methods and patients: Between 1998 and 2006, WT patients were registered from 19 centers. Patients 2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. Conclusions: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects

Testis Involvement in Neuroblastoma: Report of 3 Cases in the Turkish Pediatric Oncology Group-Neuroblastoma Study and Review of the Literature

Background: Neuroblastoma (NB) is the most common extracranial solid tumor of childhood. Primary and secondary testicular involvement is extremely uncommon in neuroblastoma. Procedure: All children with neuroblastoma treated with the Turkish Pediatric Oncology Group (TPOG)-Neuroblastoma (NB) Study and who had testis involvement either at diagnosis or at relapse were retrospectively evaluated. A review of all cases with neuroblastoma and testis involvement in the literature was done. Results: There were 3 children with NB documented to have involvement of the testis, 2 at diagnosis, 1 at recurrence, within the 559 cases (0.5%) treated with the Turkish Pediatric Oncology Group (TPOG)-Neuroblastoma Protocol. All had advanced stage. Two were infants. A total of 57 cases of testicular or paratesticular neuroblastoma have been reported in children, and most cases represent metastases as in the 3 cases in our series. Conclusions: Neuroblastoma should be considered in the differential diagnosis of testicular mass and work-up for neuroblastoma should be done before orchiectomy. Scrotal ultrasonography should be used as the first diagnostic tool and abdominal ultrasonography shall be done additionally. Testis examination should be performed at diagnosis and regularly during follow-up for boys diagnosed with neuroblastoma. Testes may be sanctuary sites when neuroblastoma is metastatic, as is the case in leukemia