Fronto-orbito-ethmoidal intradiploic meningiomas: A case study with systematic review

Abstract

Background: Primary intradiploic meningiomas, extra-axial tumors arising primarily in the skull, are rare. The authors reported a complex case of intradiploic intraosseous metaplastic meningioma of the left medial wall and orbital roof with the left frontal sinus invasion and left ethmoidal body bone substitution. The authors also conducted a systematic review concerning diagnosis and management of patients affected by purely calvarial intradiploic meningiomas along with a focus on fronto-orbito-ethmoidal ones.

Methods: A literature search was conducted using PubMed and Scopus databases according to preferred reporting items for systematic reviews and meta-analysis statement and with the following Mesh terms: Intradiploic, intraosseous, calvarial, and meningioma. Eligibility criteria were limited by the nature of existing literature on intradiploic meningiomas, consisting of only case series, and case reports.

Results: A total of 128 published studies were identified through our search. 41 studies were included in this systematic review, 59 patients with a female/male ratio of 1.2/1. The mean age of the patients is of 47.69 years (range 3-84 years). Only seven out of 59 patients (11.9%) presented a complex intradiploic meningioma located in fronto-orbito-ethmoidal region like our case. In almost all patients, a gross-total resection was performed (96.6%) and only in two patients (3.4%) a subtotal resection was achieved.

Conclusion: The authors shared this successfully treated case to add to the overall clinical experience in the management of this rare subtype tumor, with the hope that more studies are conducted to further address the mechanism of intradiploic meningiomas development.

Keywords: Anterior skull base; Calvarial; Intradiploic; Intraosseous; Meningioma.

Figure 1:

Preferred reporting items for systematic reviews and meta-analysis flow diagram summarizing our searches and selection of studies included in the systematic review of intradiploic meningioma.

Figure 2:

Preoperative coronal (a) and sagittal (b) head computed tomography scan images with bone algorithm and 3D-reconstruction (c) in series showing a left fronto-orbitoethmoidal intradiploic lesion, with frontal sinus involvement.

Figure 3:

Preoperative coronal (up) and axial (down) brain T1-weighted Magnetic resonance imaging images after Gadolinium administration showing a left inhomogeneous multiloculated intradiploic fronto-orbito-ethmoidal lesion.

Figure 4:

Routine histology showing neoplastic proliferation of medium-sized spindle tumor cells with no atypia, forming fascicles in metaplastic bone tissue and spreading in normal trabecular bone (up); neoplastic cells with epithelial membrane antigen immunoreactivity (Epithelial Membrane Antigen) (down).

Figure 5:

Postoperative axial head computed tomography scan images in series (a), with bone algorithm (b) and with 3D-reconstruction (c) showing a proper reconstruction of the left orbital roof and the medial orbital wall with split calvarial bone graft and bone defect reconstruction with methyl-methacrylate paste, obtaining good cosmetic and functional results.

Figure 6:

Axial head computed tomography scan (a) and coronal T2-CISS 3D magnetic resonance imaging (b) showing the presence of pneumocephalus with a small defect in the posterior ethmoidal plane close to the spheno-ethmoidal suture with pneumocephalus.

Figure 7:

Three-month postoperative sagittal T2-CISS 3D magnetic resonance imaging showing the posterior ethmoidal space, filled with free fatty tissue and no residual or recurrent disease.