Epidemiological characteristics and survival outcomes of children with medulloblastoma treated at the National Cancer Institute (INCA) in Rio de Janeiro, Brazil.

BACKGROUND: Medulloblastoma (MB),the most common malignant brain tumor of childhood has survival outcomes exceeding 80% for standard-risk and 60% for high-risk patients in high-income countries (HICs). These results have not been replicated in low- and middle-income countries (LMICs), where 80% of children with cancer live. METHODS: This is a retrospective review of 114 children aged 3-18 years diagnosed with MB from 1997 to 2016 at National Cancer Institute (INCA). Sociodemographic, clinical, and treatment data were extracted from the medical records and summarized descriptively. Overall survival (OS) and progression-free survival (PFS) were calculated using the Kaplan-Meier method. RESULTS: The male-to-female ratio was 1.32 and the median age at diagnosis was 8.2 years. Headache (83%) and nausea/vomiting (78%) were the most common presenting symptoms. Five-year OS was 59.1% and PFS was 58.4%. The OS for standard-risk and high-risk patients was 69% and 53%, respectively. The median time to diagnosis interval was 50.5 days and the median time from surgery to radiation therapy initiation was 50.4 days. Patients who lived >40 km from INCA fared better (OS = 68.2% vs. 51.1%, p = .032). Almost 20% of families lived below the Brazilian minimum wage. Forty-five patients (35%) had metastatic disease at admission. Gross total resection was achieved in 57% of the patitents. CONCLUSIONS: Although there are considerable barriers to deliver effective MB treatment in countries like Brazil, the OS seen in the present study demonstrates that good outcomes are not only feasible but can and should be increased with appropriate interventions.

Optic pathways tuberculoma mimicking glioma: case report.

BACKGROUND: Optochiasmatic tuberculomas are very rare lesions. They can occur with concomitant tuberculous meningitis, and pulmonary tuberculosis or as the only manifestation of the disease. The authors present a case of optic pathways tuberculoma with radiologic appearance simulating an optic pathways glioma. CASE DESCRIPTION: We report a case of a 20-year-old man with mental retardation due to anoxic encephalopathy who developed a sudden bilateral amaurosis. He also presented with diabetes insipidus, panhypopituitarism, right proptosis, and chemosis. Computed tomography (CT) and magnetic resonance imaging (MRI) showed an enhancing lesion in the optochiasmatic region extending to both optic nerves, with a mass in the right orbit, mimicking an optic pathways glioma. There was no other evidence of systemic involvement of the tuberculosis. The lesion was explored through a right pterional transylvian approach with opening of the optic canal and orbital roof, and a biopsy and an internal decompression were performed. Histopathological studies demonstrated a granulomatous lesion with central caseous necrosis with acid-fast bacilli. The patient improved after treatment with tuberculostatic drugs, but vision recovery could not be achieved. CONCLUSIONS: Visual compromise in tuberculosis is associated with hydrocephalus, optical neuritis or tuberculomas involving the optic pathways. Reviewing the literature on tuberculomas of the optochiasmatic area, we could not find any other case with such extensive involvement of the optic pathways that was radiologically suggestive of an infiltrating glioma. Histopathological studies remain crucial in the diagnosis of intrinsic expansive processes of the optochiasmatic region.

Sclerosing Cavernous Hemangioma in the Cavernous Sinus: Case Report.

Cavernous hemangiomas of the cavernous sinus belong to a well-distinguished entity of extra-axial cavernous hemangiomas located in the cavernous sinus and have a typical appearance on magnetic resonance imaging (MRI). Severe intraoperative bleeding has been described during the excision of these lesions that is probably associated with their pathological features. An atypical case of a sclerosing lesion with distinct MRI features is described. In these particular cases, especially with giant hemangiomas where en bloc excision would be difficult, safe internal decompression and resection can be achieved.