Archives of Head and Neck Surgery
Archives of Head and Neck Surgery
Case Report Tumors of the lip and oral cavity

Ectopic palatine tonsil meningioma in type 2 neurofibromatosis: a few times documented phenomenon

Juan Miguel Alemán-Iñiguez, Christian Valencia Padilla

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Type 2 neurofibromatosis is a hereditary neurocutaneous entity, characterized by associations with cranial and spinal meningiomas; extra neural cases are infrequent. There are oral meningiomas reports, however their presence at the palatine tonsils have not been described. This is a patient, with type 2 neurofibromatosis inherited from her father. The guest, presented progressive odyno-dysphagia whose etiology was WHO I meningioma in palatine tonsils, while the cranial image showed same lineage meningiomas that were surgically treated. This rare phenomenon in the context of an uncommon disease and the events that would explain the arrival of menigiomatous tissue to the palate are discussed.


meningioma; neurofibromatosis 2; palatine tonsil.


1. Slattery WH. Neurofibromatosis type 2. Otolaryngol Clin North Am. 2015;48(3):443-60. PMid:26043141.

2. Goutagny S, Kalamarides M. Medical treatment in neurofibromatosis type 2: review of the literature and presentation of clinical reports. Neurochirurgie. 2018;64(5):370-4. PMid:28162254.

3. Dirks M, Butman J, Kim H, Wu T, Morgan K, Tran A, Lonser R, Asthagiri A. Longterm natural history of neurofibromatosis type 2: associated intracranial tumors. J Neurosurg. 2012;117(1):109-17. PMid:22503123.

4. Evans DG. Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis. 2009;4(1):16. PMid:19545378.

5. Ruttledge M, Andermann A, Phelan C, Claudio J, Han F, Chretien N, Rangaratnam S, MacCollin M, Short P, Parry D, Michels V, Riccardi VM, Weksberg R, Kitamura K, Bradburn JM, Hall BD, Propping P, Rouleau GA. Type of mutation in the neurofibromatosis type 2 gene (NF2) frequently determines severity of disease. Am J Hum Genet. 1996;59(2):331-42. PMid:8755919.

6. Evans DG, Bowers N, Huson SM, Wallace A. Mutation type and position varies between mosaic and inherited NF2 and correlates with disease severity. Clin Genet. 2013;83(6):594-5. PMid:22989157.

7. Friedrich R, Hagel C. Expansive extracranial growth of intracranial meningioma in neurofibromatosis type. Anticancer Res. 2016;36(6):3161-7. PMid:27272842.

8. Friedman CD, Costantino PD, Teitelbaum B, Berktold RE, Sisson GA Sr. Primary extracranial meningiomas of the head and neck. Laryngoscope. 1990;100(1):41-8. PMid:2104554.

9. Akhmametyeva E, Mihaylova M, Luo H, Kharzai S, Welling D, Chang L. Regulation of the neurofibromatosis 2 gene promoter expression during embryonic development. Dev Dyn. 2006;235(10):2771-85. PMid:16894610.

10. Vital RB, Hamamoto Filho PT, Lapate RL, Martins VZ, de Oliveira Lima F, Romero FR, Zanini MA. Calvarial ectopic meningothelial meningioma. Int J Surg Case Rep. 2015;10:69-72. PMid:25805612.

11. Molina Montes L, Montes de Oca Fernández F, Gamboa Mutuberría B. Embriología y anatomía de la cavidad oral y faringe. In: Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello, editor. Cavidad oral y faringe. 1. ed. Alcalá de Henares: Hospital Universitario Príncipe de Asturias. Libro virtual de formación. Capítulo 67; p. 2.

12. Zaifullah S, Yunus MR, See GB. Diagnosis and treatment of branchial cleft anomalies in UKMMC: a 10-year retrospective study. Eur Arch Otorhinolaryngol. 2013;270(4):1501-6. PMid:23053382.

13. Shetty C, Avinash KR, Auluck A, Mupparapu M. Extracranial meningioma of the parapharyngeal space: report of a case and review of the literature. Dentomaxillofac Radiol. 2007;36(2):117-20. PMid:17403892.

14. Albsoul N, Rawashdeh B, Albsoul A, Abdullah M, Golestani S, Rawshdeh A, Mohammad M, Alzoubi M. A rare case of extracranial meningioma in parapharyngeal space presented as a neck mass. Int J Surg Case Rep. 2015;11:40-3. PMid:25912007.

15. Kumar S, Hasija S, Goyal R, Kataria SP, Sen R, Wadhera R. Ectopic parapharyngeal meningioma: diagnosis of a rare entity on FNAC. J Surg Case Rep. 2012;2012(12):rjs024. PMid:24968421.

16. Amer HW, Hafed L, Ibrahim S, Shaker S. Case report: rare site for intraoral meningioma. F1000 Res. 2020;9:95. PMid:32850120.

17. Evans JJ, Jeun SS, Lee JH, Harwalkar JA, Shoshan Y, Cowell JK, Golubic M. Molecular alterations in the neurofibromatosis type 2 gene and its protein rarely occurring in meningothelial meningiomas. J Neurosurg. 2001;94(1):111-7. PMid:11147878.

18. Durand A, Champier J, Jouvet A, Labrousse F, Honnorat J, Guyotat J, Fèvre-Montange M. Expression of c-Myc, neurofibromatosis Type 2, somatostatin receptor 2 and erb-B2 in human meningiomas: relation to grades or histotypes. Clin Neuropathol. 2008;27(5):334-45. PMid:18808065.

19. Coy S, Rashid R, Stemmer-Rachamimov A, Santagata S. An update on the CNS manifestations of neurofibromatosis type 2. Acta Neuropathol. 2020;139(4):643-65. PMid:31161239.

20. Grill J, Dhermain F, Habrand JL. Risks of radiation therapy in patients with neurofibromatosis. Int J Radiat Oncol Biol Phys. 2009;75(2):632. PMid:19735889.

21. Miao R, Wang H, Jacobson A, Lietz A, Choy E, Raskin K, Schwab J, Deshpande V, Nielsen GP, DeLaney TF, Cote GM, Hornicek FJ, Chen YE. Radiation-induced and neurofibromatosis-associated malignant peripheral nerve sheath tumors (MPNST) have worse outcomes than sporadic MPNST. Radiother Oncol. 2019;137:61-70. PMid:31078939.

22. Evans DG, Birch JM, Ramsden RT, Sharif S, Baser ME. Malignant transformation and new primary tumours after therapeutic radiation for benign disease: substantial risks in certain tumour prone syndromes. J Med Genet. 2005;43(4):289-94. PMid:16155191.

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