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Neuroradiological features of patients with bilateral macronodular adrenocortical disease and meningiomas associated or not with genetic variants of ARMC5– a case series

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Abstract

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.

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Data availability

No datasets were generated or analysed during the current study.

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Authors and Affiliations

  1. Adrenal Unit, Laboratory of Hormones and Molecular Genetics LIM/42, Division of Endocrinology and Metabolism, Clinics Hospital, University of Sao Paulo Medical School, São Paulo, Brazil

    Arthur Araújo Massoud Salame, Helaine Laiz Silva Charchar, Berenice Mendonca & Maria Candida Barisson Villares Fragoso

  2. Adrenal Unit, Endocrinology Service of the University Hospital of the Federal University of Santa Catarina, Florianópolis, Brazil

    Guilherme Asmar Alencar

  3. Laboratório de Patologia Quantitativa, Centro de Patologia do Instituto Adolfo Lutz, São Paulo, Brazil

    Leonardo José Tadeu de Araújo

  4. Departamento de Radiologia, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, LIM 44, Sao Paulo, SP, Brazil

    Paula Ricci Arantes

  5. Division of Neurosurgery, Clinics Hospital, University of São Paulo Medical School, São Paulo, Brazil

    Nicollas Nunes Rabelo & Eberval Gadelha Figueiredo

  6. Neurosurgery Department, Instituto Tocantinense Presidente Antônio Carlos Porto S/A– ITPAC Porto, Porto Nacional, Brazil

    José Paulo de Oliveira Dourado

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  1. Arthur Araújo Massoud Salame
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Contributions

AAMS, JPOD, BM, GAA, LJTDA with the search of databases, construction of the manuscript and its formatting.HLSC and PRA contributed to the process of checking the database, constructing tables and correcting the manuscript. NNR, EGF and MCBVF contributed to the orientation of the theme, selected cases and correction of this manuscript.All the authors revised the manuscript on occasion.

Corresponding author

Correspondence to José Paulo de Oliveira Dourado.

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Salame, A.A.M., Charchar, H.L.S., de Oliveira Dourado, J.P. et al. Neuroradiological features of patients with bilateral macronodular adrenocortical disease and meningiomas associated or not with genetic variants of ARMC5– a case series. J Neurooncol (2024). https://doi.org/10.1007/s11060-024-04680-9

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