Download PDF
CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2021; 31(03): 768-771
DOI: 10.1055/s-0041-1736394
Case Report

Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation

Smily Sharma
1   Department of Radiodiagnosis and Imaging, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
,
Pankaj Sharma
1   Department of Radiodiagnosis and Imaging, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
,
Amit Kumar
1   Department of Radiodiagnosis and Imaging, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
› Author Affiliations Funding None.
› Further Information
Also available at
   Permissions and Reprints

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.

Keywords

en plaque meningioma - meningiomatosis - neurofibromatosis 2


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/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 

  • 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
    PubMedGoogle Scholar
  • 2 Harbitz H. A case of multiple meningiomas combined with diffuse meningiomatosis. Acta Pathol Microbiol Scand 2010; 12: 24-37
    CrossrefGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 5 Kim KS, Rogers LF, Goldblatt D. CT features of hyperostosing meningioma en plaque. AJR Am J Roentgenol 1987; 149 (05) 1017-1023
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 10 Nagano T, Saiki I, Kanaya H. Multiple meningiomas in the posterior fossa. Surg Neurol 1985; 23 (04) 425-427
    CrossrefPubMedGoogle Scholar
  • 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
    PubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar