RESUMO
INTRODUCTION: Meningiomas are the most common primary brain and central nervous system tumors, accounting for approximately 40% of these tumors. The most important exams for the radiological study of meningiomas are computed tomography (CT) and magnetic resonance imaging (MRI). We aimed to analyze the radiological features of patients with meningioma related to the simultaneous presence of bilateral macronodular adrenocortical disease (BMAD), with or without pathogenic variants of ARMC5. METHODS: This study included 10 patients who were diagnosed with BMAD. All of them had a radiological diagnosis of expansive brain lesions suggestive of meningioma. All patients underwent brain MRI and a neuroradiolgist analyzed the following parameters: number, site and size of lesions; presence of calcification, edema and bone involvement. RESULTS AND DISCUSSION: Eight patients presented with germline variants of ARMC5; the other 2, did not. The most significant result was the incidence of multiple meningiomas, which was 50% in BMAD patients, whereas the average incidence described thus far is lower than 10%. Considering location, the 22 tumors in the BMAD patients were 5 convexity tumors (22.7%), and 17 skull base tumors (77.2%), the opposite proportion of patients without BMAD. A total of 40.9% of the tumors had calcification, 9% had cerebral edema and 40.9% had bone invasion due to hyperostosis. The literature describes meningioma calcification in 25% of patients, bone invasion by tumor hyperostosis in 20%, and cerebral edema in approximately 60%. CONCLUSION: Relevant results were found considering the rate of multiple meningiomas and tumor location. This finding reinforces the need for further research into the neurological effects caused by genetic variants of ARMC5 in patients with BMAD.
Assuntos
Imageamento por Ressonância Magnética , Neoplasias Meníngeas , Meningioma , Humanos , Meningioma/genética , Meningioma/diagnóstico por imagem , Meningioma/patologia , Feminino , Masculino , Neoplasias Meníngeas/genética , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/patologia , Pessoa de Meia-Idade , Adulto , Idoso , Tomografia Computadorizada por Raios X , Proteínas do Domínio ArmadilloRESUMO
Paragangliomas of the cauda equina are tumors of rare incidence, with ~ 220 cases described in the world literature. They are benign lesions, grade I by the World Health Organization (WHO), whose definitive diagnosis can only be made by immunohistochemical analysis. Its neuroendocrine nature is evidenced by the presence of chromogranin. The relevance of reporting this case is because paragangliomas of the cauda equina should be included among the differential diagnoses of intradural and extra-medullary tumors, and especially because they can cause perioperative and intraoperative hypertensive crises by adrenergic discharge. The present study presents the case of a 36-year-old male patient diagnosed with a lumbar spine tumor located in the central spinal canal that presented as cauda equina syndrome involving 4 months of bilateral sciatica, paraparesis, urinary and fecal retention. The diagnosis of paraganglioma was confirmed by immunohistochemical positivity for chromogranin after microsurgical resection of the tumor.
Paragangliomas da cauda equina são tumores de incidência rara, com ~ 220 casos descritos na literatura mundial. São lesões benignas, grau 1 pela Organização Mundial da Saúde (OMS), cujo diagnóstico definitivo apenas pode ser feito pela análise imunohistoquímica, onde se evidencia a natureza neuroendócrina dada especialmente pela presença de cromogranina. O presente relato de caso se torna importante porque este deve ser considerado entre os diagnósticos diferenciais de tumores intradurais e extramedulares, e porque há trabalhos na literatura descrevendo crises hipertensivas durante a ressecção da lesão devidas a descarga adrenérgica. O presente estudo apresenta o caso de um paciente masculino de 36 anos diagnosticado com tumor na coluna lombar localizado no canal medular, que cursou com síndrome de cauda equina, com 4 meses de lombociatalgia bilateral, evoluindo à paraparesia, retenção urinária e fecal. Após microcirurgia para ressecção tumoral, o diagnóstico de paraganglioma só foi confirmado através da imunohistoquímica, com positividade para cromogranina.