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DOI: 10.1055/s-0041-1736394
Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation
Funding None.
Abstract
Meningiomas are amongst the most common neoplasms of the central nervous system; however, “multiple meningiomas” or “meningiomatosis” account for < 10% of cases. The association with neurofibromatosis 2 is seen in ∼50% of cases. We report a case of 35-year-old female patient who presented with left eye proptosis, left forehead swelling, and multiple cranial nerve palsies predominantly on the left side. Imaging evaluation revealed innumerable en plaque meningiomas forming a sheet-like nodular thickening along the dura, causing marked hyperostosis and extending into the orbits, cavernous sinuses, sellar–suprasellar regions, various skull foramina, basal cisterns and into the cervical spinal canal causing mass effect on vital structures of the brain and cervical spinal cord. Similar lesions were found scattered in rest of the spine. We intend to highlight the role of imaging in accurately establishing the diagnosis and evaluating the extent and burden of disease in such rare cases.
Publication History
Article published online:
13 November 2021
© 2021. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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References
- 1 Koech F, Orege J, Ndiangui F, Macharia B, Mbaruku N. Multiple intracranial meningiomas: a review of the literature and a case report. Case Rep Surg 2013; 2013: 131962
- 2 Harbitz H. A case of multiple meningiomas combined with diffuse meningiomatosis. Acta Pathol Microbiol Scand 2010; 12: 24-37
- 3 Ohla V, Scheiwe C. Meningiomatosis restricted to the left cerebral hemisphere with acute clinical deterioration: case presentation and discussion of treatment options. Surg Neurol Int 2015; 6: 64
- 4 Akutsu H, Sugita K, Sonobe M, Matsumura A. Parasagittal meningioma en plaque with extracranial extension presenting diffuse massive hyperostosis of the skull. Surg Neurol 2004; 61 (02) 165-169 , discussion 169
- 5 Kim KS, Rogers LF, Goldblatt D. CT features of hyperostosing meningioma en plaque. AJR Am J Roentgenol 1987; 149 (05) 1017-1023
- 6 Araújo Pereira BJ, Nogueira de Almeida A, Pires de Aguiar PH, Paiva WS, Teixeira MJ, Nagahashi Marie SK. Multiple intracranial meningiomas: a case series and review of the literature. World Neurosurg 2019; 122: e1536-e1541
- 7 Chaurasia B, Hossain M, Barua K. et al. Meningiomatosis in non-neurofibromatosis patient: how we dealt with 8 tumors. J Neurol Stroke 2018; 8: 307-310
- 8 Koh YC, Yoo H, Whang GC, Kwon OK, Park HI. Multiple meningiomas of different pathological features: case report. J Clin Neurosci 2001; 8 (Suppl. 01) 40-43
- 9 Christiaans I, Kenter SB, Brink HC. et al. Germline SMARCB1 mutation and somatic NF2 mutations in familial multiple meningiomas. J Med Genet 2011; 48 (02) 93-97
- 10 Nagano T, Saiki I, Kanaya H. Multiple meningiomas in the posterior fossa. Surg Neurol 1985; 23 (04) 425-427
- 11 Hashimoto H, Hirabayashi H, Hiramatsu K, Morimoto T, Tsunoda S, Sakaki T. Malignant parasagittal meningioma appearing as a large subcutaneous mass [article in Japanese]. No Shinkei Geka 1994; 22 (08) 785-788
- 12 Neuss M, Westphal M, Hänsel M, Herrmann HD. Clinical and laboratory findings in patients with multiple meningiomas. Br J Neurosurg 1988; 2 (02) 249-256
- 13 Nakamura M, Roser F, Michel J, Jacobs C, Samii M. The natural history of incidental meningiomas. Neurosurgery 2003; 53 (01) 62-70 , discussion 70–71