Volume 223, December 2022, 107498Abstract

Meningiomas are the most common benign intracranial tumors. They are generally asymptomatic, and discovered incidentally during cerebral imaging. The vast majority of meningiomas are solid, highly cellular and well-vascularized neoplasms. However, in several cases, they can be partially or, even rarely, almost completely cystic making their differential diagnosis and management challenging. In this paper, we present the rare case of a 59-year-old female patient, presenting with persistent headaches, who was diagnosed with a left parieto-occipital purely cystic lesion. The patient underwent a complete resection of this cystic lesion because of increasing headaches and volumetric progression. Interestingly, the histological assessment confirmed a cystic WHO grade I meningioma. The evolution was favorable and there was no recurrence after 3 years of follow-up. We also perform a systematic review of the literature concerning purely cystic meningiomas and we discuss the particular histological features of cystic meningiomas as well as the possible pathogenesis. This challenging clinical entity can easily be misdiagnosed as hemangioblastoma or glial/metastatic tumor with cystic component.

Introduction

Meningiomas are the most common benign intracranial tumors, representing 13–18 % of all intracranial tumors, a percentage that reaches even the one third according to some authors [1], [3]. They are more common in women, with a ratio of 2–3/1, and especially those who are middle aged (40–60 years old) [3]. Generally, meningiomas are asymptomatic and discovered incidentally [31], [17]. They are typically solid, highly cellular and well-vascularized lesions. Nevertheless, in some cases, they can be partially or, even rarely, almost completely cystic in adults. Herein we describe an extremely rare case of a purely cystic meningioma, we discuss its histological specificities and possible pathogenesis, and we perform a systematic review of the literature.

Section snippetsCase description

A 59-year-old female patient, with a history of adrenal gland hyperplasia, has been followed for almost three years in a neurology clinic for a left parieto-occipital cystic lesion that was discovered in the context of atypical headaches. The initial neurologic exam was normal, including fundoscopy. A standard annual follow-up was initially decided.

During this follow-up, the cystic lesion progressively grew (36 ×55 mm on the last imaging before surgery, whereas 32 ×40 mm on the first cerebral

Systematic review

A systematic literature review was performed according to the PRISMA guidelines [21]. Articles reporting cases of cystic meningiomas were searched in the Pubmed electronic database, from the existence of the database until December 2021. The keywors used were “meningioma”, “cystic” and “cyst”, with a proper use of truncation [ meningioma* AND (cystic OR cyst*)] [32]. The final step of our systematic review revealed only one article with one case (Fig. 5) [12]. The references of this article

General facts about solid meningiomas

Meningiomas are extra-axial tumors that originate from the meningothelial cells of the arachnoid cap and most of them are WHO grade I slow-growing solid neoplasms [3]. Ionizing radiation is a well-established risk factor, especially in radiotherapy. Radiation-associated meningiomas may also develop in a predisposed genetic background [25]. Mutations in the neurofibromatosis gene (NF2) are associated with an increased risk. The role of hormones is reflected by the higher incidence of meningiomas

Conclusion

The present case is unique because the meningioma presented as a purely cystic lesion without evident solid component, raising the differential diagnosis of an hemangioblastoma or a glial tumor/metastasis with cystic component. There is no definitive method for preoperatively differentiating a completely cystic meningioma from other cystic tumors, and neuropathology evidently remains the key for the definitive diagnosis.

Funding

No funding.

Human and animal ethics

We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this article is consistent with those guidelines.

Patient consent

We confirm that we have obtained written permission from the patient, according to the Journal’s position on ethical publication.

CRediT authorship contribution statement

GG - Project draft, systematic literature review, data analysis, and manuscript writing, AV - Histological analysis, participation in manuscript editing, evaluation of the manuscript, SC - Neuro-oncological analysis, evaluation of the relevant literature and of the manuscript, IPG - Critical analysis and evaluation of the literature and the discussion of the manuscript, JG - Critical analysis and evaluation of the literature and the discussion of the manuscript, CD - Critical review of the

Acknowledgements

None.

Competing interests

The authors declare no conflicts of interest.

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