Clinical case of five-year local control following radiosurgery of intracranial tumors in a patient with type II neurofibromatosis
Keywords:neurofibromatosis type II, intracranial tumor, stereotactic radiosurgery
Type II neurofibromatosis (NF2) is a rare autosomal dominant hereditary disease characterized by the presence of many benign tumors of the nervous system. Stereotactic radiosurgery (SRS) is a generally accepted therapeutic option for sporadic vestibular schwannoma and small and medium-sized meningiomas, which allows treating several tumors simultaneously during a single irradiation session. At the same time, opinions on the use of radiosurgery as the primary treatment for patients with NF2 remain controversial. This article deals with a clinical case of the results of radiosurgical treatment of a 50-year-old patient with NF2, with a follow-up period of 5 years. The final results of SRS in ensuring local control of NF2 associated with vestibular schwannoma and meningiomas should be evaluated not earlier than 24–36 months after irradiation. The advantages of radiosurgical treatment of patients with NF2 include non-invasiveness, the possibility of irradiating several tumors in a single irradiation session, treatment under outpatient conditions, ensuring local control of tumor growth, minimizing of the neurological deficit associated with the treatment toxicity, ensuring the quality of life of patients.
1. Blakeley JO, Evans DG, Adler J, Brackmann D, Chen R, Ferner RE, Hanemann CO, Harris G, Huson SM, Jacob A, Kalamarides M, Karajannis MA, Korf BR, Mautner VF, McClatchey AI, Miao H, Plotkin SR, Slattery W 3rd, Stemmer-Rachamimov AO, Welling DB, Wen PY, Widemann B, Hunter-Schaedle K, Giovannini M. Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2. Am J Med Genet A. 2012 Jan;158A(1):24-41. [CrossRef] [PubMed] [PubMed Central]
2. Rouleau GA, Merel P, Lutchman M, Sanson M, Zucman J, Marineau C, Hoang-Xuan K, Demczuk S, Desmaze C, Plougastel B, et al. Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2. Nature. 1993 Jun 10;363(6429):515-21. [CrossRef] [PubMed]
3. Trofatter JA, MacCollin MM, Rutter JL, Murrell JR, Duyao MP, Parry DM, Eldridge R, Kley N, Menon AG, Pulaski K, et al. A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell. 1993 Nov 19;75(4):826. [CrossRef] [PubMed]
4. Ruggieri M, Praticò AD, Serra A, Maiolino L, Cocuzza S, Di Mauro P, Licciardello L, Milone P, Privitera G, Belfiore G, Di Pietro M, Di Raimondo F, Romano A, Chiarenza A, Muglia M, Polizzi A, Evans DG. Childhood neurofibromatosis type 2 (NF2) and related disorders: from bench to bedside and biologically targeted therapies. Acta Otorhinolaryngol Ital. 2016 Oct;36(5):345-367. [CrossRef] [PubMed] [PubMed Central]
5. Asthagiri AR, Parry DM, Butman JA, Kim HJ, Tsilou ET, Zhuang Z, Lonser RR. Neurofibromatosis type 2. Lancet. 2009 Jun 6;373(9679):1974-86. [CrossRef] [PubMed]
6. Campian J, Gutmann DH. CNS Tumors in Neurofibromatosis. J Clin Oncol. 2017 Jul 20;35(21):2378-2385. [CrossRef] [PubMed] [PubMed Central]
7. Chen LH, Zhang HT, Xu RX, Zhang L, Li WD, Sun K. Microsurgery for patients diagnosed with neurofibromatosis type 2 complicated by vestibular schwannomas: Clinical experience and strategy for treatments. Medicine (Baltimore). 2018 Apr;97(17):e0270. [CrossRef] [PubMed] [PubMed Central]
8. Goutagny S, Bah AB, Henin D, Parfait B, Grayeli AB, Sterkers O, Kalamarides M. Long-term follow-up of 287 meningiomas in neurofibromatosis type 2 patients: clinical, radiological, and molecular features. Neuro Oncol. 2012 Aug;14(8):1090-6. [CrossRef] [PubMed] [PubMed Central]
9. Klijn S, Verheul JB, Beute GN, Leenstra S, Mulder JJ, Kunst HP, Hanssens PE. Gamma Knife radiosurgery for vestibular schwannomas: evaluation of tumor control and its predictors in a large patient cohort in The Netherlands. J Neurosurg. 2016 Jun;124(6):1619-26. [CrossRef] [PubMed]
10. Plotkin SR, Stemmer-Rachamimov AO, Barker FG 2nd, Halpin C, Padera TP, Tyrrell A, Sorensen AG, Jain RK, di Tomaso E. Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med. 2009 Jul 23;361(4):358-67. [CrossRef] [PubMed] [PubMed Central]
11. Prasad D, Steiner M, Steiner L. Gamma surgery for vestibular schwannoma. J Neurosurg. 2013 Dec;119 Suppl:745-59. [CrossRef] [PubMed]
12. Mallory GW, Pollock BE, Foote RL, Carlson ML, Driscoll CL, Link MJ. Stereotactic radiosurgery for neurofibromatosis 2-associated vestibular schwannomas: toward dose optimization for tumor control and functional outcomes. Neurosurgery. 2014 Mar;74(3):292-300; discussion 300-1. [CrossRef] [PubMed]
13. Massager N, Delbrouck C, Masudi J, De Smedt F, Devriendt D. Hearing preservation and tumour control after radiosurgery for NF2-related vestibular schwannomas. B-ENT. 2013;9(1):29-36. [PubMed]
14. Zemskova OV, Chuvashova OYu. Modern approaches to vestibular schwannomas treatment in patients with neurofibromatosis type 2 using stereotactic radiosurgery. Ukr Neurosurg J. 2014;(3):66-71. Ukrainian. [CrossRef]
15. Tsao MN, Sahgal A, Xu W, De Salles A, Hayashi M, Levivier M, Ma L, Martinez R, Rйgis J, Ryu S, Slotman BJ, Paddick I. Stereotactic radiosurgery for vestibular schwannoma: International Stereotactic Radiosurgery Society (ISRS) Practice Guideline. J Radiosurg SBRT. 2017;5(1):5-24. [PubMed] [PubMed Central]
16. Maniakas A, Saliba I. Neurofibromatosis type 2 vestibular schwannoma treatment: a review of the literature, trends, and outcomes. Otol Neurotol. 2014 Jun;35(5):889-94. [CrossRef] [PubMed]
17. Daniel RT, Tuleasca C, George M, Pralong E, Schiappacasse L, Zeverino M, Maire R, Levivier M. Preserving normal facial nerve function and improving hearing outcome in large vestibular schwannomas with a combined approach: planned subtotal resection followed by gamma knife radiosurgery. Acta Neurochir (Wien). 2017 Jul;159(7):1197-1211. [CrossRef] [PubMed] [PubMed Central]
18. Verbova LN, Gudkov VV, Onishchenko PM, Fedirko VO, Lisyany AN, Tsyurupa DM, Chuvashova OYu, Gryazov AB, Zemskova OV, Kruchok IV, Skobskaya OE, Kiseleva IG, Malysheva TA. Treatment of large and giant vestibular schwannomas and its results. Experimental and Clinical Medicine. 2015;4(69):110-118. Russian.
19. Hayhurst C, Zadeh G. Tumor pseudoprogression following radiosurgery for vestibular schwannoma. Neuro Oncol. 2012 Jan;14(1):87-92. [CrossRef] [PubMed] [PubMed Central]
20. Sharma MS, Singh R, Kale SS, Agrawal D, Sharma BS, Mahapatra AK. Tumor control and hearing preservation after Gamma Knife radiosurgery for vestibular schwannomas in neurofibromatosis type 2. J Neurooncol. 2010 Jun;98(2):265-70. [CrossRef] [PubMed]
21. Phi JH, Kim DG, Chung HT, Lee J, Paek SH, Jung HW. Radiosurgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2: tumor control and hearing preservation. Cancer. 2009 Jan 15;115(2):390-8. [CrossRef] [PubMed]
22. Subach BR, Kondziolka D, Lunsford LD, Bissonette DJ, Flickinger JC, Maitz AH. Stereotactic radiosurgery in the management of acoustic neuromas associated with neurofibromatosis Type 2. J Neurosurg. 1999 May;90(5):815-22. [CrossRef] [PubMed]
23. Rowe JG, Radatz MW, Walton L, Soanes T, Rodgers J, Kemeny AA. Clinical experience with gamma knife stereotactic radiosurgery in the management of vestibular schwannomas secondary to type 2 neurofibromatosis. J Neurol Neurosurg Psychiatry. 2003 Sep;74(9):1288-93. [CrossRef] [PubMed] [PubMed Central]
24. Mathieu D, Kondziolka D, Flickinger JC, Niranjan A, Williamson R, Martin JJ, Lunsford LD. Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: an analysis of tumor control, complications, and hearing preservation rates. Neurosurgery. 2007 Mar;60(3):460-8; discussion 468-70 [CrossRef] [PubMed]
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