Unusual leptomeningeal dissemination in a child with extracranial metastatic alveolar rhabdomyosarcoma.

The authors describe a 6-year-old boy diagnosed with alveolar rhabdomyosarcoma located in the thigh, with distal metastases to lungs, bones, and bone marrow. A very good partial response to first-line chemotherapy was obtained, but the child developed fatal leptomeningeal dissemination immediately after complete resection of the primary tumor. This case demonstrates the rapidity with which leptomeningeal spread of extracranial metastatic alveolar rhabdomyosarcoma can occur and underscores the importance of diagnostic lumbar puncture and brain radiological investigations at diagnosis, even when the tumors are not in the parameningeal location.

Plasma heparanase as a significant marker of treatment response in children with Hodgkin lymphoma: pilot study.

INTRODUCTION: The aim of this pilot study was to determine heparanase plasma levels (HP) at diagnosis and at restaging in children diagnosed with Hodgkin lymphoma and to investigate whether this parameter provides prognostic information for response to treatment after induction therapy. PATIENTS AND METHODS: HP levels of 19 pediatric patients (mean age: 10.3 years (y) (range, 4-18 y), 9 girls, 10 boys) with Hodgkin lymphoma were assayed at diagnosis and at restaging. HP levels were determined using an ELISA anti-human heparanase immunoassay kit. According to diagnosis, CAT scan and/or FDG/ PET-CT fusion were performed to assess response to treatment after 2-3 courses of chemotherapy. Two patients received VAMP protocol (1 stage IA, 1 stage IIA), 1 received AV-PC (nonbulky stage IIA), 4 received COPP/ABV (3 stage IIA bulky, 1 stage IIIA nonbulky), 4 received ABVE-PC (2 stage IIB, 1 stage IIA bulky, 1 stage IIIA bulky), 2 received ABVD (1 stage IIA bulky, 1 stage IIIA), and 6 received escalated BEACOPP (1 stage IIIB, 3 stage IVA, 2 stage IVB). RESULTS: Changes in HP levels were found to correlate with response to treatment for most of the children. At diagnosis, average HP level was 1019 pg/mL (range, 141-5733 pg/mL), decreasing at restaging to 588 pg/mL (range, 62-3267 pg/mL) (p = .034). At diagnosis, the average HP of the 16 patients in CR or VGPR was 1104 pg/mL; it had decreased at restaging to 586 pg/mL (p = .032). At diagnosis, the average HP level for the 3 patients with TP or PR was 1704 pg/mL; it had increased to 1938 pg/mL at restaging (p = .166). Due to the small number of patients, no correlation was observed between HP levels at diagnosis, staging, or any other clinical prognostic factor. CONCLUSIONS: Changes in plasma HP levels correlated with response to treatment for children diagnosed with Hodgkin lymphoma. This provides a rationale for exploring clinical interest in plasma heparanase measurements of a larger group, using the test for clinical trials of antiangiogenic therapies.

Utility of sonographically guided biopsy in metabolically suspected recurrent lymphoma.

OBJECTIVE: The purpose of this study was to determine the diagnostic accuracy of sonographically guided biopsy of [(18)F]fluorodeoxyglucose (FDG)-avid foci on positron emission tomography (PET)/computed tomography (CT) in patients with lymphoma. METHODS: We retrospectively reviewed the medical records of 56 patients with lymphoma (25 male and 31 female; mean age, 48.5 years; range, 22-80 years) who underwent sonographically guided biopsy of hypermetabolic FDG-avid foci precisely localized by PET/CT. Biopsies were performed up to 3 months after PET/CT. The accuracy of core biopsy was calculated and compared with clinical follow-up and histopathologic results of open biopsy. RESULTS: Sixty-six sonographically guided biopsies were performed in the 56 patients. Histopathologic results were conclusive in 53 (80%) of 66. No complications occurred during or after the procedure. The overall sensitivity, specificity, positive predictive value, and accuracy for diagnosis of lymphoma were 100%, 95%, 97%, and 98%, respectively. CONCLUSIONS: Sonographically guided biopsy is a safe and effective means for investigating metabolically active lesions on FDG-PET/CT in patients with known lymphoma.