Microcystic meningioma of the calvarium: a series of 9 cases and review of the literature

• Published in: The American journal of surgical pathology Vol. 39; no. 4; p. 505
• Main Authors: Velázquez Vega, José E, Rosenberg, Andrew E
• Format: Journal Article
• Language: English
• Published: United States 01.04.2015
• Subjects: Aged; Aged, 80 and over; Biomarkers, Tumor - analysis; Biopsy; Female; Humans; Immunohistochemistry; Male; Meningeal Neoplasms - chemistry; Meningeal Neoplasms - pathology; Meningeal Neoplasms - surgery; Meningioma - chemistry; Meningioma - pathology; Meningioma - surgery; Middle Aged; Predictive Value of Tests; Skull - chemistry; Skull - pathology; Skull - surgery; Skull Neoplasms - chemistry; Skull Neoplasms - pathology; Skull Neoplasms - surgery; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Tumor Burden
• ISSN: 1532-0979
• DOI: 10.1097/PAS.0000000000000347

Meningiomas are one of the most common tumors that arise within the central nervous system; they represent up to 30% of all primary intracranial tumors. Extradural meningiomas are rare (<2% of all meningiomas), and most arise within the calvarium. Intraosseous calvarial meningiomas are usually easy to diagnose histologically if they are of the meningothelial type; however, they may cause diagnostic challenges when they manifest as unusual morphologic variants, such as the microcystic type. To address this issue we present a series of 9 cases of calvarial microcystic meningiomas arising in 7 female and 2 male patients; all patients were adults. The tumors had heterogenous findings on imaging studies and ranged in size from 1.1 to 4.3 cm in greatest dimension. The neoplasms were composed predominantly of stellate and spindle cells with long, thin interconnecting cytoplasmic processes arranged in a complex network. The resulting cellular architecture was "sieve-like" in appearance because of the formation of numerous small "cyst-like" spaces interposed between the cytoplasmic processes of the tumor cells. All of the neoplasms expressed the characteristic immunophenotype of meningiomas (EMA, PR positive). Most tumors were resected, and none of these have recurred during a follow-up period of 1 to 83 months (average 17 mo). The morphology of the tumors and their anatomic location generated problems in diagnosis, especially in 6 patients with a history of malignancy and for whom metastatic disease was suspected clinically. Intraosseous microcystic meningioma is uncommon, and this series, the largest reported to date, describes their clinicopathologic findings, biological behavior, and features that facilitate their accurate diagnosis.