Primary intraosseous meningioma: clinical, histological, and differential diagnostic aspects

TY - JOUR

T1 - Primary intraosseous meningioma: clinical, histological, and differential diagnostic aspects

AU - Butscheidt, Sebastian

AU - Ernst, Marielle

AU - Rolvien, Tim

AU - Hubert, Jan

AU - Zustin, Jozef

AU - Amling, Michael

AU - Martens, Tobias

PY - 2020/8

Y1 - 2020/8

N2 - OBJECTIVE: Primary intraosseous meningioma (PIM) is a rare manifestation of meningioma, a benign, neoplastic lesion of the meninges. Its characteristic appearance is hyperostosis, while no or only minimal dural changes can be observed. This study aims to characterize this rare entity from both a clinical and histopathological point of view in order to improve clinical management.METHODS: In the years 2009-2017, 26 cases of PIM were diagnosed using MRI and CT scans. In 16 cases the indication for resection was given, and specimens were further examined using a multilevel approach, including histological and immunohistochemical analyses. Additionally, the local database was searched for all cases of meningiomas, as well as osteosclerotic differential diagnoses-i.e., fibrous dysplasia, Paget's disease of bone, and other benign osteosclerotic lesions.RESULTS: In this study, PIM represented 2.4% of all meningiomas with a predominant occurrence in females (85%). Regarding the initial manifestation, PIMs show a slightly earlier onset than meningiomas. While most PIMs are located in the sphenoid bone, associated calcifications were visible in 58% of the cases on CT scans. Most of the cases were classified as WHO grade I (93%) and meningotheliomatous meningiomas (91%). Tumor growth was associated with an increased bone resorption followed by massive osteoid deposition and consecutive sclerosis. The frequently observed frayed appearance results from multiple bony canals, which contain blood vessels for the blood supply of the highly vascularized tumor tissue.CONCLUSIONS: PIM is a rare but important differential diagnosis for osteosclerotic lesions of the skull, especially in women. Tumor-induced, cellular-mediated bone resorption and formation may play a central role in the underlying pathogenesis.

AB - OBJECTIVE: Primary intraosseous meningioma (PIM) is a rare manifestation of meningioma, a benign, neoplastic lesion of the meninges. Its characteristic appearance is hyperostosis, while no or only minimal dural changes can be observed. This study aims to characterize this rare entity from both a clinical and histopathological point of view in order to improve clinical management.METHODS: In the years 2009-2017, 26 cases of PIM were diagnosed using MRI and CT scans. In 16 cases the indication for resection was given, and specimens were further examined using a multilevel approach, including histological and immunohistochemical analyses. Additionally, the local database was searched for all cases of meningiomas, as well as osteosclerotic differential diagnoses-i.e., fibrous dysplasia, Paget's disease of bone, and other benign osteosclerotic lesions.RESULTS: In this study, PIM represented 2.4% of all meningiomas with a predominant occurrence in females (85%). Regarding the initial manifestation, PIMs show a slightly earlier onset than meningiomas. While most PIMs are located in the sphenoid bone, associated calcifications were visible in 58% of the cases on CT scans. Most of the cases were classified as WHO grade I (93%) and meningotheliomatous meningiomas (91%). Tumor growth was associated with an increased bone resorption followed by massive osteoid deposition and consecutive sclerosis. The frequently observed frayed appearance results from multiple bony canals, which contain blood vessels for the blood supply of the highly vascularized tumor tissue.CONCLUSIONS: PIM is a rare but important differential diagnosis for osteosclerotic lesions of the skull, especially in women. Tumor-induced, cellular-mediated bone resorption and formation may play a central role in the underlying pathogenesis.

U2 - 10.3171/2019.3.JNS182968

DO - 10.3171/2019.3.JNS182968

M3 - SCORING: Journal article

C2 - 31226688

VL - 133

SP - 451

EP - 461

JO - J NEUROSURG

JF - J NEUROSURG

SN - 0022-3085

IS - 2

ER -