Atypical Epstein-Barr-Virus infection in a 5-year-old girl.

Primary Epstein-Barr virus (EBV) infection is often asymptomatic before five years of age, whereas it is more likely to be symptomatic in older children, adolescents and young adults. The classic triad of symptoms including fever, pharyngitis and lymphadenopathy is well known, but the spectrum of clinical manifestations associated with EBV infection is large and continues to expand, including increasing atypical presentations. This article presents the case of a five-year-old female in which only a protracted and severe itch, resistant to antihistamines, caught the attention of the girl's parents, thus allowing the diagnosis of EBV. Furthermore, EBV related splenomegaly and mild hepatitis as well as thrombocytopenia had an atypical prolonged course.

[Nutritional status disorders prevalence rates in a sample of pediatric oncology Day Hospital patients]. / Prevalenza delle alterazioni dello stato nutrizionale in un campione di pazienti afferenti a un day hospital oncologico pediatrico.

BACKGROUND: Nutritional status is more important in children than in adults because it is necessary to support normal growth and development. In industrialized countries the prevalence of nutritional status disorders in the pediatric population are as follows: 35-40% of children are overweight/obese, while the underweight status disappeared in some reports or, when it is present, it is associated with other diseases. The aim of this study was to investigate the prevalence rates of nutritional status disorders among an unselected sample of Pediatric Oncology Day Hospital patients. METHODS: Anthropometric parameters of weight and height have been measured in patients affected with solid tumor either on-therapy or off-therapy (0-24 mo). Then Real Body Weight (% RBW) was estimated referring to National Center for Health Statistic percentiles. The sample was then divided into 4 weight classes (under-weight, normal-weight, over-weight and obese) according to % RBW. RESULTS: Overweight patients (overweight + obese) were 44.4%, and 13.9% underweight. Dividing the patients according to whether they are on-therapy or off-therapy, the prevalence of overweight was 36.9% in the former group and 52.9% in the latter, and the underweight prevalence was 26.3 vs 0%. CONCLUSIONS: Our preliminary data show that in this sample of patients on treatment obesity and overweight are present in a similar percentage of the healthy population, but underweight status prevalence is 26.3%. In the sample of patients off-therapy the underweight status disappeared while the overweight status increased. These data suggest that nutritional assessment in oncologic patients is required in order to provide nutritional strategies.

Ten-day schedule oral etoposide therapy in advanced childhood malignancies.

PURPOSE: The activity of etoposide (VP-16) has been demonstrated to be schedule-dependent. Several studies have been conducted on the efficacy and safety of different schedules of VP-16 both in adults and in children, but the optimal schedule has not been determined. METHODS: In the current study, the feasibility and effectiveness of prolonged oral VP-16 in children with high-risk malignancies were evaluated. Between April 1995 and February 1999, 15 pretreated patients with high-risk tumors received oral VP-16. The schedule of therapy was oral VP-16 50 mg/m2/day for 10 consecutive days and 1-week interval between cycles. Therapy was stopped after 1 year of treatment or at time of progressive disease or possible surgery. All patients had received parenteral VP-16 in their earlier chemotherapy. RESULTS: Twelve patients were evaluable for tumor response. After 2 to 4 months of treatment, one patient had complete remission (CR), two had partial response (PR), two had minor response (MR), two had mixed response (MxR), three had stable disease (SD), and two had progressive disease (PD). A useful palliative effect was noted in patients with stable disease. In three patients, oral VP-16 was administered for maintenance therapy. After an average follow-up of 27.5 months (range, 7-41 months), five patients are alive without disease (in three, total surgery was performed after VP-16 therapy) and three patients are alive with disease. Six patients died of progressive disease, and one died of promyelocytic leukemia. One patient had Grade 34 thrombocytopenia; in the remaining patients, no acute toxicity was observed during treatment. CONCLUSIONS: This schedule of oral VP-16 produced CRs, PRs, and MRs in medulloblastoma, neuroblastoma, teratocarcinoma, and ependymoma. Stable disease was observed in three patients, one with an Askin tumor, one with medulloblastoma, and one with hepatoblastoma. Given the possible leukemogenic risk, this schedule should be used as a palliative form of therapy or in patients with poor prognosis..

Hypersensitivity to carboplatin in children.

BACKGROUND: Hypersensitivity reactions are rare but at times severe complications to cytostatic drugs. PROCEDURE: The percentage of allergic reactions to carboplatin and their clinical features were evaluated in 185 children affected by different solid tumors and treated with etoposide-carboplatin chemotherapy. Allergic reactions that occurred during or immediately following etoposide infusion (5 cases, 2.8%) were excluded from the study. RESULTS: Seventeen out of 185 patients (9.2%) suffered from allergic responses to carboplatin. The first of these occurred after an average of 10.1 courses (range, 1-23; median, 9). The risk calculated according to the number of courses is 2% at 6 courses, 11.3% at 12 courses, and 47% at more than 12 courses. CONCLUSIONS: The high risk of allergic reactions to multiple courses of carboplatin should be kept in mind when developing treatment regimens that include the drug.

Metastatic medulloblastoma in 10-year-old girl treated successfully with chemotherapy without radiotherapy.

We report a case of high risk medulloblastoma with leptomeningeal intracranial and spinal metastasis in a 10-year-old girl treated successfully with conventional prolonged chemotherapy without radiotherapy. This is a particular case of medulloblastoma that at onset did not receive standard therapy for medulloblastoma i.e. neither surgery nor craniospinal irradiation. This 10-year-old Chinese girl affected with localized medulloblastoma was previously treated at a medical department in China only with radiotherapy on the posterior fossa. When the child arrived in Italy with progressed metastatic medulloblastoma, she was treated with carboplatin/etoposide association i.v. followed by oral etoposide and partial surgery of the primitive mass. The schedule of chemotherapy was etoposide 300 mg/sqm followed by carboplatin 1000 mg/sqm in one day every 21-28 days for the first six courses, then etoposide 200 mg/sqm and carboplatin 600 mg/sqm in one day every 28-35 days for further 11 courses and oral etoposide 50 mg/sqm/day for ten consecutive days and one week interval between two cycles for one year. At present the girl is alive and disease-free, and has been off-therapy for 31 months. Interestingly, in this case a long-lasting complete remission was obtained without radiotherapy and without myeloablative chemotherapy. Oral etoposide played an important role in achieving a complete remission.

Chemotherapy in low-grade astrocytoma management.

The role of chemotherapy (CHT) in the management of low-grade astrocytoma (LGA) is still unclear. Nineteen children with nonresectable symptomatic LGA were treated with carboplatin (CBDCA) and etoposide (E). There were 15 newly diagnosed cases and 4 were relapses; 6 of the children were under 5 years old. In all children radiological evaluation by CT scan and/or MRI was performed after four courses of CHT. We observed complete response (CR)+ minor response (MR) in 37% of these cases and an improvement in neurological symptoms in 63%. Radiological evaluation performed in 6 patients who received CHT for longer periods (8-12 courses) showed major responses (CR+PR) in 67%. Local radiotherapy (40 Gy) was administered after CHT in 14 cases, but in 3 of these radiotherapy was delayed for 2 years. Five patients did not receive radiotherapy. The overall survival was 58% after an average follow-up of 60 months. All patients with brain stem tumors died of progressive disease even though 3 of these had shown clinical improvement after chemotherapy. In conclusion, in the treatment of nonresectable symptomatic LGA, CHT with CBDCA associated with E can be used to postpone radiotherapy in young children and even to avoid radiotherapy in some cases.

Use of ICRF-187 for prevention of anthracycline cardiotoxicity in children: preliminary results.

The objective of this study is to assess the efficacy of ICRF-187 as a protective agent against anthracycline cardiotoxicity. Cardiac function was evaluated by echocardiography before and after each cycle of anthracycline chemotherapy associated with ICRF-187 and compared with that of a second group receiving anthracycline chemotherapy without ICRF-187. The patients were a group of 15 consecutive children affected with various types of solid tumors who were treated with either doxorubicin-daunomycin or epirubicin (average doses 340 and 280 mg/m2, respectively), and treatment was associated with ICRF-187. A second group of 15 consecutive children affected with different malignancies were simultaneously treated with either doxorubicin-daunomycin or epirubicin (average doses 309 and 270 mg/m2, respectively), but without ICRF-187 association. None of the patients treated with anthracyclines and ICRF-187 association showed abnormalities on echocardiographic examination. In the second group of patients treated with anthracyclines but without ICRF-187 association, we observed a decrease in the left ventricular ejection fraction to < 55% and a decrease in the left ventricular fractional shortening to < 28% in two patients (13.3%). One of these (6.6%) showed a dilatative cardiomyopathy. Both groups of patients were treated with low doses of anthracyclines. Although this study was not randomized, in patients without ICRF-87 cardioprotection, there was a trend for a worse evolution with one case of clinical cardiomyopathy as well as subclinical cardiac abnormalities.

Ultrasound in the management of hepatic veno-occlusive disease in three children treated with dactinomycin and vincristine.

Three children, treated with dactinomycin and vincristine without radiotherapy showed hepatic toxicity consistent with diagnostic criteria for hepatic veno-occlusive disease (VOD). Two patients were affected with Wilms' tumor and the third with malignant fibromatosis. The clinical manifestations of VOD were mild and regressed in all patients after supportive therapy. Serial ultrasonography (US) was performed in all cases and was useful in confirming the diagnosis and in evaluating the severity of the disease. US features of VOD were hepatomegaly, gallbladder wall thickening, ill-defined borders of the hepatic vessels, and ascites. Parenchymal heterogeneity was still present after several months as a result of the hepatic injury. The hazy appearance of the portal vessels and the gallbladder wall thickening seemed directly correlated with the degree of hepatic involvement in the early phase of VOD.

Does chemotherapy have a role in low-grade astrocytoma management? A report of 13 cases.

Surgery is the treatment of choice for low-grade astrocytoma while radiotherapy is carried out only when total resection is not possible. This study assessed the effectiveness of chemotherapy in nonresectable cases. Thirteen children with nonresectable astrocytoma were treated with carboplatin and etoposide and after four cycles the response to treatment was evaluated according to radiologic criteria. The results were: one with complete response (CR), three with minor response (MR), six with stable disease (SD), and three with progressive disease (PD). Moreover, in 77% there was an improvement in the neurologic picture. In particular, two cases with hypothalamic astrocytoma showed a regression of the diencephalic syndrome following chemotherapy. In six cases chemotherapy was carried out, at reduced dosage, after the first four cycles either because there was clinical improvement or because it was necessary to postpone radiotherapy in very young patients. After a follow-up period ranging between 11 and 63 months (average: 30 months), nine of the 13 patients are alive (69%) while four died of disease progression. Further studies would be useful to evaluate the role of chemotherapy in the management of low-grade astrocytoma.

[The usefulness of the skeleton in the diagnosis of malformation]. / Utilità dello scheletro nella diagnosi di malformazione.

Most malformation syndromes (70%) show evidence of skeletal anomalies, mainly in the vertebral bodies and in the bony segments of the limbs. This explains the importance of studying the skeleton when faced with any multi-malformation syndrome, using techniques like CT, MRI, echography and densitometry as well as standard radiology. To increase the quantity of information available for diagnostic purposes it would be profitable to create a data bank containing all the possible radiological findings encountered in the numerous multi-malformation syndromes.