Neuroblastoma is the most common extra-cranial, solid, malignant tumour in children. Advances in radiology have made possible the detection and staging of the disease. Nevertheless, there is no method available at present that can go beyond detection and qualitative analysis, towards quantitative assessment of the tissue composition of the primary tumour mass in neuroblastoma. Such quantitative analysis could provide important information and serve as a decision-support tool to the radiologist and the oncologist, result in better treatment and follow-up and even lead to the avoidance of delayed surgery. The problem investigated was the improvement of the analysis of the primary tumour mass, in patients with neuroblastoma, using X-ray computed tomography (CT) images. A methodology was proposed for the estimation of the tissue content of the mass: it comprised a Gaussian mixture model for estimation, from segmented CT images, of the tissue composition of the primary tumour. To demonstrate the potential of the method, the results are presented of its application to ten CT examinations of four patients. The method provides quantitative information, and it was observed that the tumour in one of the patients reduced from 523 cm3 to 81 cm3 in volume, with an increase in calcification from about 20% to about 88% of the tumour volume, in response to chemotherapy over a period of five months. Results indicate that the proposed technique may be of considerable value in assessing the response to therapy of patients with neuroblastoma.

The MYCN oncogene is amplified in 20% of childhood neuroblastoma and is associated independently with poor prognosis. Alteration of the p53 tumor supressor gene, in contrast, occurs infrequently in these tumors. In this report, we described a 3‐year‐old girl with stage IV neuroblastoma. Molecular analysis revealed both MYCN gene amplification and a point mutation of the p53 tumor supressor gene. To our knowledge, this is the first reported case of neuroblastoma with genetic alterations of both these genes. Med. Pediatr. Oncol. 29:206–207, 1997. © 1997 Wiley‐Liss, Inc.

Background Chemotherapy has dramatically improved the rates of cure and survival of patients with localized and metastatic osteosarcoma. Nonetheless, the number of chemotherapeutic agents active against osteosarcoma is limited to doxorubicin, cisplatin, high‐dose methotrexate, and ifosfamide. Carboplatin, a cisplatin analogue, has been tested as a single agent in patients with recurrent osteosarcoma or as part of multiagent chemotherapy in newly diagnosed patients. Procedure We tested the activity and toxicity of two cycles of intraarterial carboplatin as a “window therapy” (600 mg/m2 per cycle) in 33 consecutive patients with extremity osteosarcoma before the start of multiagent chemotherapy. Response was based on clinical (tumor diameter, local inflammatory signs, and range of motion) and radiological parameters (plain local films and arteriographic studies prior to drug administration). Results Patients' age ranged between 8 and 18 years (median age 13 years). Primarytumor originated from the femur (15 patients), tibia (10 patients), fibula (4 patients), humerus (3 patients), and calcaneus (1 patient). Only 7 patients (21%) had metastatic disease at diagnosis (5 in the lung and 2 in other bones). A favorable clinical and radiological response was documented in 81% and 73% of the patients, respectively. Clinical and radiological progression occurred in 12% and 9% of the patients, respectively. Seventeen of the patients remain alive and disease‐free. Survival and event‐free survival at 3 years for nonmetastatic patients are 71% (SE = 9%) and 65% (SE = 9%), respectively; for metastatic patients, the figures are 17% (SE = 15%) and 14% (SE = 13%), respectively. Conclusions We conclude that carboplatin is an active agent in the treatment of newly diagnosed extremity osteosarcoma. Med. Pediatr. Oncol. 33:71–75, 1999. © 1999 Wiley‐Liss, Inc.

The treatment of cancer in children has several side effects, including ototoxicity. Inner ear structures may be affected and hearing loss may ensue. Aim: To estimate the prevalence of hearing loss in patients with cancer using the American Speech-Language-Hearing Association (ASHA), the Pediatric Oncology Group Toxicity (POGT), and the Bilateral Hearing Loss (PAB) criteria. Study design: a prospective study. Material and Methods: 94 patients admitted between 2003 and 2004 were analyzed. Visual inspection of the external auditory meatus and an audiologic evaluation were done. Descriptive statistics was used to characterize the sample, and Kappa statistics was used to investigate concordance of hearing loss in the three types of classification. Results: The prevalence of hearing loss was 42.5% using ASHA, 40.4% using POGT, and 12.8% using PAB. The concordance of hearing loss was weak for POGT and PAB (k=0.36) and for PAB and ASHA (k=0.33). The concordance between ASHA and POGT was almost perfect (k=0.96). Conclusions: Hearing loss is an important side effect of the treatment of cancer in children. Periodic audiology monitoring is recommended to detect early hearing loss and to revise the treatment if necessary. Adoption of a classification system that detects mild hearing loss (ASHA) is recommended.

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