Chordoid Meningioma: Literature Review

Abstract

Chordoid meningiomas (CMs) are a rare subgroup of tumors, accounting for ∼ 0.5% of all meningiomas. Chordoid meningioma tumors correspond to World Health Organization (WHO) Grade II lesions and behave aggressively, with an increased likelihood of recurrence. There are few genetic studies about CMs, but we understand that there is deletion at many chromosomal loci. Histologically, CMs are characterized by strands and cords of meningothelial cells arranged in a mucinous stroma. Morphologically, it can mimic other chondroid and myxoid tumors within the brain and its vicinity, thus posing a diagnostic challenge. Chordoid meningiomas have an aggressive clinical course and a propensity to recur compared with classical meningiomas. The goal of the treatment is surgery, with total resection of the tumor; however, due to its high degree of recurrence, radiotherapy is often necessary as an adjuvant treatment.

Resumo

Meningiomas cordoides (MCs) são um tipo raro de subgrupo de tumores, representando ∼ 0,5% de todos os meningiomas. Os MCs correspondem ao grau II, pela classificação da Organização Mundial da Saúde (OMS), possuindo agressividade e alto grau de recorrência. Existem poucos estudos publicados sobre a genética desses tumores; porém, entendemos que há diversas deleções em alguns lócus cromossômicos. Histologicamente, os MCs são caracterizados por fios e cordões de células meningoteliais dispostos em um estroma mucinoso. Morfologicamente, podem mimetizar outros tumores condroides e mixoides, o que representa um desafio diagnóstico. Os MCs têm um curso mais agressivo que o meningioma clássico. O objetivo do tratamento é a cirurgia, com ressecção total do tumor, porém, devido à sua alta recidiva, se faz necessário o tratamento adjuvante com radioterapia.

Keywords

meningioma - chordoid - brain neoplasms - pathology

Palavras-chave

meningioma - cordóide - tumor cerebral - patologia

• Reference

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