Optic pathway gliomas (OPG) can cause elevated cerebrospinal fluid (CSF) protein concentrations. We report on two patients with suprasellar low-grade gliomas and high CSF protein levels (590 and 551 mg/dl) that precluded shunt implantation. After two and three doses of bevacizumab, respectively, the levels dropped dramatically to 191 and 178 mg/dl, respectively. Bevacizumab treatment was associated with a decrease in CSF protein level, allowing successful shunt placement. Our results are consistent with the pharmacological mechanism of bevacizumab, which decreases protein leakage from blood vessels to the ventricles.
Standard of care and protocols for the treatment of pediatric cancer lead to a clear improvement in survival rates and quality of life. Little is known about how these treatments are implemented in Brazil. Our study aimed to evaluate children treated for Hodgkin disease (HD) in south Brazil between 2002 and 2013 through the analysis of medical records in 6 different centers.Results: Fifty-nine children and adolescents were included. The median age was 12 years (range 3-18 years). Male:female ratio was 1.95:1. Localized disease (stage I/II) was observed in 30 patients (50.8%) while the remaining 29 (49.2%) had advanced disease (Stage III/IV).The chemotherapeutic treatment schema was different among services and comprised three different based protocols. ABVD schema was the most frequently used (52 children (88.1%)).The number of cycles was highly variable (4-16 cycles) even at the same clinical stage and with similar clinical response.Conclusion: These data highlight the importance of turning the "best practice policies" readily available to all pediatric oncologists. Local protocols allow integrative studies among centers that would certainly maintain or improve cure rates, reduce long-term toxicity and evaluate specific biological characteristics of these diseases in our population. For these reasons, we reinforce the idea that standardization of treatment in pediatric oncology is a child health priority and also a viable low-cost strategy to improve care in middle-income countries such as Brazil .
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. It is located mainly in the head and neck, extremities and trunk, rarely achieving the biliary tract (1%). The clinical presentation is characterized by obstructive jaundice, abdominal pain and palpable mass. The prognosis is favourable when being early diagnosed. The main differential diagnosis is the choledochal cyst. We bring a case report about a patient with biliary tract rhabdomyosarcoma, diagnosed and treated for 1 year as choledochal cyst, presenting obstructive jaundice for one year. A hepatic biopsy was done, showing hepatic fibrosis. He presented clinical worsening and was referred for liver transplant. An exploratory laparotomy was done before planning the transplant, which showed a biliary tract mass, followed by a biopsy. The anatomopathological result was RMS. Biliary tract RMS is a rare entity that should be included in the differential diagnosis of obstructive jaundice in children. Because of its similarity with choledochal cyst, pediatricians and surgeons should consider this hypothesis, for adequate investigation and treatment.
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