Brain Metastases in Soft Tissue Sarcomas: Case Report and Literature Review

Background and purpose: Brain metastasis is a relatively uncommon event in the natural history of soft tissue sarcomas. The increasing use of chemotherapy may have caused a reduction in local relapses as well as distant failures leading to an improvement in survival, thereby allowing metachronous seeding of the brain, a sanctuary site. The purpose of this report is to increase awareness amongst clinicians regarding such a possibility

Increased p53 immunopositivity in anaplastic medulloblastoma and supratentorial PNET is not caused by JC virus

BACKGROUND: p53 mutations are relatively uncommon in medulloblastoma, but abnormalities in this cell cycle pathway have been associated with anaplasia and worse clinical outcomes. We correlated p53 protein expression with pathological subtype and clinical outcome in 75 embryonal brain tumors. The presence of JC virus, which results in p53 protein accumulation, was also examined. METHODS: p53 protein levels were evaluated semi-quantitatively in 64 medulloblastomas, 3 atypical teratoid rhabdoid tumors (ATRT), and 8 supratentorial primitive neuroectodermal tumors (sPNET) using immunohistochemistry. JC viral sequences were analyzed in DNA extracted from 33 frozen medulloblastoma and PNET samples using quantitative polymerase chain reaction. RESULTS: p53 expression was detected in 18% of non-anaplastic medulloblastomas, 45% of anaplastic medulloblastomas, 67% of ATRT, and 88% of sPNET. The increased p53 immunoreactivity in anaplastic medulloblastoma, ATRT, and sPNET was statistically significant. Log rank analysis of clinical outcome revealed significantly shorter survival in patients with p53 immunopositive embryonal tumors. No JC virus was identified in the embryonal brain tumor samples, while an endogenous human retrovirus (ERV-3) was readily detected. CONCLUSION: Immunoreactivity for p53 protein is more common in anaplastic medulloblastomas, ATRT and sPNET than in non-anaplastic tumors, and is associated with worse clinical outcomes. However, JC virus infection is not responsible for increased levels of p53 protein

Late recurrence of combined hepatocellular carcinoma and hepatoblastoma in a child: Case report and review of the literature

We report on a case of late relapse of hepatocellular carcinoma in a child suffering from combined hepatoblastoma and hepatocellular carcinoma, stage IV. This is a rare event, as it has been accepted that a 5-year period free of any signs of disease in children suffering from malignant hepatic tumors is sufficient to classify such patients as survivors. In our patient, recurrence of the hepatocellular carcinoma component was diagnosed more than five years after the initial diagnosis. This case illustrates the need for more prolonged follow-ups for such children

Plasma heparanase as a significant marker of treatment response in pediatric malignancies: Pilot study

9540 Introduction: The aim of this pilot study was to determine heparanase plasma levels (HP) at diagnosis and at restaging in children diagnosed with cancer, and to investigate whether this parameter provides prognostic information for remission after induction therapy and response to treatment. Patients and Methods: Heparanase plasma levels of 45 consecutive pediatric patients (pts) with various types of cancer were assayed at diagnosis and at restaging. Mean age: 10.3 years (y) (0.5y-21y), 20 females, 25 males. Levels of heparanase were determined using a commercially available ELISA anti-human heparanase immunoassay kit. According to diagnosis, bone marrow aspiration, CAT scan, MRI, technetium bone scan, PET-CT fusion were performed to assess response to treatment after the first month of induction for leukemia patients, 2 to 3 courses of chemotherapy for other malignancies. Results: Changes in HP levels were found to correlate with response to treatment for most of the children. At diagnosis, average HP level was 1,539 pg/mL (range, 55 pg/mL-6,567 pg/mL) and decreased at restaging to 943 pg/mL (range, 0 pg/mL -3,682 pg/mL) (p=0.0133). At diagnosis, the average HP of the 37 patients in CR or VGPR was 1,692 pg/mL and at restaging decreased to 928 pg/mL (p=0.0071). At diagnosis, the average HP level for the 5 pts with TP or PR was 1,873 pg/mL and increased to 2,633 pg/mL at restaging (p=0.4223). Due to the small number of patients we did not observe any correlation with diagnosis or any other clinical prognostic factor. Conclusions: Changes in plasma heparanase levels correlated with response to treatment for all of the children diagnosed with cancer. This provides a rationale for exploring clinical interest in plasma heparanase measurements of a larger group of children with malignancies, and using the test for clinical trials of antiangiogenic therapies. No significant financial relationships to disclose. </jats:p