Adrialdo José Santos

Medulloblastoma in adults: a series from Brazil

We retrospectively reviewed 15 adult patients (11 males, median age 34 years; range 23–48) who had been treated and followed in our Institution since 1991 from the time of diagnosis until death or last follow-up in December 2001. Headache was the most frequent symptom (93%). The tumor was hemispheric in 11 patients. Complete resection was achieved in eight. CSF in 12 patients and craniospinal MRI in 6 did not show metastatic disease. Two patients refused adjuvant treatment and died with progressive disease. Thirteen patients received adjuvant craniospinal radiotherapy and 11 systemic chemotherapy. After initial treatment only 2 of the 13 patients relapsed in the posterior fossa. Recurrence was probably related to sub-optimal radiotherapy planning: inadequate low dose in the posterior fossa (37.5 Gy) and long delay in initiating treatment. Two of the 13 patients that received adjuvant treatment died: one from meningitis, and one from recurrent disease. Eleven patients remained alive, and disease-free with Karnofsky performance status ranging 80–100. The median overall survival was not reached after a median follow-up of 5.6 years (range 0.7–10.8 years). Estimated 1-, 5- and 10-year overall survival rates were 86.7%, 72.7%, and 72.7%, respectively. Adult medulloblastoma was predominant in males and the majority of patients had hemispheric tumors. Long-term survival was not uncommon. Although chemotherapy may be useful and well tolerated, radiotherapy remains the mainstay adjuvant treatment as suggested by our two recurrences associated with a delay or inadequate dose.

Acidente vascular cerebral isquêmico após quimioterapia com cisplatina, etoposide e bleomicina: relato de caso

A 20-year-old man with a germ cell tumor who experienced an ischemic stroke as a complication of cisplatin/etoposide/bleomycin based chemotherapy is reported. The previously reported cases are reviewed as well as the different physiopathologic mechanisms associated with vascular toxicity of this regimen.

Waiting time to radiotherapy as a prognostic factor for glioblastoma patients in a scenario of medical disparities

OBJECTIVE To evaluate the effect of waiting time (WT) to radiotherapy (RT) on overall survival (OS) of glioblastoma (GBM) patients as a reliable prognostic variable in Brazil, a scenario of medical disparities. METHOD Retrospective study of 115 GBM patients from two different health-care institutions (one public and one private) in Brazil who underwent post-operative RT. RESULTS Median WT to RT was 6 weeks (range, 1.3-17.6). The median OS for WT ≤ 6 weeks was 13.5 months (95%CI , 9.1-17.9) and for WT > 6 weeks was 14.2 months (95%CI, 11.2-17.2) (HR 1.165, 95%CI 0.770-1.762; p = 0.470). In the multivariate analysis, the variables associated with survival were KPS (p < 0.001), extent of resection (p = 0.009) and the adjuvant treatment (p = 0.001). The KPS interacted with WT to RT (HR 0.128, 95%CI 0.034-0.476; p = 0.002), showing that the benefit of KPS on OS depends on the WT to RT. CONCLUSION No prognostic impact of WT to RT could be detected on the OS. Although there are no data to ensure that delays to RT are tolerable, we may reassure patients that the time-length to initiate treatment does not seem to influence the control of the disease, particularly in face of other prognostic factors.

Gliomas múltiplos: casos ilustrativos de quatro formas de apresentação

Multiple gliomas are uncommon and may be classified according to: a) the time of presentation in early (at diagnosis) or late (during treatment); b) the characteristics of computed tomography or magnetic resonance imaging (CT/MRI) in multifocal (with evidence of spread) and multicentric (without evidence of spread). From 212 patients with histopathologic diagnosis of glioma evaluated from March/90 to September/99, 15 (7%) had multiple lesions. We describe 4 patients: early multicentric, late multicentric, early multifocal and late multifocal, with emphasis on characteristics of CT/MRI and possible differential diagnosis. The differential diagnosis of multiple lesions in the central nervous system includes mainly infectious/inflammatory diseases and metastasis, however multiple gliomas should always be considered, even in patients with known systemic cancer, as described by others. Considering that CT/MRI features are not definite, the diagnosis should always be confirmed by histopathologic examination.Multiple gliomas are uncommon and may be classified according to: a) the time of presentation in early (at diagnosis) or late (during treatment); b) the characteristics of computed tomography or magnetic resonance imaging (CT/MRI) in multifocal (with evidence of spread) and multicentric (without evidence of spread). From 212 patients with histopathologic diagnosis of glioma evaluated from March/90 to September/99, 15 (7%) had multiple lesions. We describe 4 patients: early multicentric, late multicentric, early multifocal and late multifocal, with emphasis on characteristics of CT/MRI and possible differential diagnosis. The differential diagnosis of multiple lesions in the central nervous system includes mainly infectious/inflammatory diseases and metastasis, however multiple gliomas should always be considered, even in patients with known systemic cancer, as described by others. Considering that CT/MRI features are not definite, the diagnosis should always be confirmed by histopathologic examination.

Associaçäo de malformaçäo vascular e gliomas: estudo de quatro casos

We reviewed the clinical presentation, imaging and histopathologic findings in 4 patients with the diagnosis of arteriovenous malformation associated with glioma that were operated on from 1991 to 2000 in our institution. Four patients (2 males; age between 15 and 52 years) presented with progressive headache with clinical evidence of intracranial hypertension (in 3) and partial seizures (in 1). CT scan showed a brain tumor without any detectable pathologic vessels. Histologic examination revealed astrocytic tumors associated with arteriovenous malformation. No patient presented the vascular component intermixed with the tumor. The arteriovenous-glioma association is rare and must be identified by a clear demarcation between the malformation and the tumor.Entre os pacientes operados no Hospital Sao Paulo e acompanhados pelo setor de neuro-oncologia no periodo de 1991 a 2000, avaliamos a apresentacao clinica, aspectos de imagem e caracteristicas histopatologicas de 4 pacientes (2 homens; idade entre 15 e 52 anos) cujo diagnostico histologico foi malformacao vascular associada a glioma. O quadro inicial foi cefaleia progressiva com caracteristicas de hipertensao intracraniana (em 3) e crises parciais motoras (em 1). O diagnostico tomografico inicial foi processo expansivo, sem que houvesse suspeita de malformacao vascular pelo aspecto da imagem em nenhum caso. O exame histologico mostrou neoplasias de linhagem astrocitica associadas a malformacoes vasculares. Em nenhum paciente o componente vascular esteve localizado na intimidade da neoplasia. A associacao de malformacao vascular e gliomas e rara e deve ser caracterizada por nitida separacao entre a malformacao e a neoplasia, independente da vascularizacao propria do tumor.

Postictal thoracocervicofacial purpura

A 28-year-old Brazilian man presented with a 2-hour history of headache and skin lesions in the shoulder girdle, face and upper trunk. Two hours before, he had a generalised tonic-clonic seizure, his third since being diagnosed with genetic generalised epilepsy 1 year before. Each previous episode had been accompanied by the same pattern of skin lesions, which improved after 48 hours. On examination, …

Metastatic breast cancer in a man with nonprogressive ataxia and epilepsy

A 44-year-old man presented with subacute-onset paraparesis and urinary incontinence. Medical history disclosed macrocephaly, neurodevelopmental delay, epilepsy, and nonprogressive ataxia. Examination disclosed a complete spinal cord syndrome without clear sensory level. Neuroimaging disclosed marked brain hemiatrophy (figure 1) and a compressive mass at T10-T11 level (figure 2). Lesion biopsy revealed a breast adenocarcinoma. Genetic analysis confirmed mutations in the PTEN gene, diagnosing Bannayan-Riley-Ruvalcaba syndrome (BRRS).

Ossifying fibroma of the maxilla and tuberous sclerosis complex

A 19-year-old man with refractory generalized epilepsy related to tuberous sclerosis complex (TSC) (figure 1) complained of chronic left facial swelling. Physical examination disclosed typical mucocutaneous findings of TSC (hypomelanotic macules, shagreen patch, ungual fibromas, facial angiofibromas)1 and a hardened well-limited mass in his left maxilla, evidenced as an odontogenic extensive left maxillary mass (figure 2). Histopathologic study was compatible with an ossifying fibroma. TSC is a major neurocutaneous syndrome frequently associated with gingival hyperplasia or fibromas.1 Ossifying maxillary fibroma is an extremely rare finding associated with TSC.2

Non-aneurysmal non-traumatic subarachnoid hemorrhage: think also in acute promyelocytic leukemia

A 45-year-old woman presented with sudden severe excruciating headache. Medical history disclosed intermit-tent fever and weight loss in the past 3 months. No focal neurological deficits were seen. CT scan disclosed diffuse subarachnoid hemorrhage. Cranial CT angiography showed no intracranial aneurysms (Figure). Laboratory evaluation disclosed thrombocytopenia, anemia and abnormalities of white blood cell. Bone marrow biopsy confirmed acute promyelocytic leukemia (APML).Cerebrovascular disease is a frequent neurological compli-cation in oncological patients, generally secondary to coagulop -athies related to the neoplasm or drug-induced bleeding

Brain MRI features in late-onset nonketotic hyperglycinemia.

A 22-year-old woman presented with learning disability and gait unsteadiness since adolescence. Medical history revealed episodes of encephalopathy and myoclonic jerks associated with intercurrent infections. Examination showed spastic paraparesis, ataxia and optic atrophy. Neuroimaging revealed agenesis of corpus callosum (Figure). Plasma amino acid analysis disclosed elevated glycine levels with an increased cerebrospinal fluid:plasma glycine ratio. Nonketotic hyperglycinemia (MIM #605899) is an autosomal recessive disorder of glycine metabolism caused by a defect in the glycine cleavage system with three different clinical forms: neonatal, infantile and late-onset with heterogeneous brain malformations, such as abnormal corpus callosum, gyral malformations and enlarged ventricles1,2.