NÖROFİBROMATOZİS TİP-2 OLGUSUNDA İNTRAVENTRİKÜLER MENENJİOM

Öz

GİRİŞ: Nörofıbromatozis (NF) tip-2 nadir görülen bir hastalıktır (1/210 000). Ventrikül içi menenjiomlar da nadir görülen tümörlerdir (%0,5-3). Nadir görülen bu iki patolojinin birlikteliği radyolojik ve klinik açıdan tartışılmıştır. OLGU: 63 y kadın hasta. Baş ağrısı , her iki kulakta 10 yıldır ilerleyici işitme kaybı şikayetleriyle başvurdu. Hastanın kranial MR tetkiki sonucunda her iki internal akustik kanal içinde milimetrik boyutlarda patolojik kontrast tutulumu (schwannom?), sol lateral ventrikül içinde, patolojik kontrast tutan yaklaşık 32x28x25 mm düzgün sınırlı kitle (menenjiom ?) tespit edildi. Nörolojik muayenesinde bilateral işitmede azalma dışında patolojik bulgu yoktu. Sol parietalde ciltaltında 1,5x1 cm fibromatöz lezyon vardı. Transkortikal insizyonla sol lateral ventriküldeki kitle total olarak boşaltıldı. Patoloji sonucu fibroblastik menenjiom olarak geldi. SONUÇ: İşitme kaybı ve baş ağrısı şikayeti ile gelen hastalarda nadirde olsa NF tip 2 ve eşlik eden ventrikül içi tümör akılda bulundurulmalıdır. Dr. İsmail İŞTEMEN, Dr. Salim ŞENTÜRK, Dr. Ömer FarukTÜRKOĞLU, Dr. Etem BEŞKONAKLI

Anahtar Kelimeler

• Nörofıbromatozis
• tip-2
• intraventriküler
• menenjiom.

INTRAVENTRICULAR MENENGIOMA IN A NEUROFIBROMATOSIS TYPE-2 CASE

Öz

INTRODUCTION: Neurofibromatosis (NF) type-2 is a rare disease(1/210 000). Also intraventricular menengiomas are rare too (%0,5-3). A rare combination of these two pathologies are discussed in the radiological and clinical aspect. CASE: 63 Y, female patient. She was admitted with headache and progressive hearing loss in both ears for 10 years. As a result of the patient’s cranial MRI, pathological contrast enhancements in the millimeter sizes (schwannoma?) were detected in the both of internal acoustic canal, and pathological contrast-enhancing mass with smooth margins of about 32x28x25 mm (menenjiom?) was detected in the left lateral ventricle. Neurological examination findings were normal except for bilateral decreased hearing. There was a 1,5x1 cm fibromatous lesion under the skin at the left parietal zone, ventricular mass was totally removed with transcortical incision. Histopathological result was reported as fibroblastic meningioma. RESULT: Hearing loss and headache can occur in patients with a complaint from the intraventricular tumors associated with NF type 2 and should be considered.

Kaynakça

1. 1. Nakamura M, Roser F, Bundschuh O, Vorkapic P, Samii M. Intraventricular meningiomas: a review of 16 cases with reference to the literature. Surg Neurol 2003; 59:491-503, discussion 503-494.
2. 2. Chaskis C, Buisseret T, Michotte A, D’Haens J. Meningioma of the fourth ventricle presenting with intermittent behaviour disorders: a case report and review of the literature. J Clin Neurosci 2001;8(4A):59-62.
3. 3. Delfıni R, Acqui M, Oppido PA, Capone R, Santoro A, Ferrante L. Tumors of the lateral ventricles. Neurolsurg Rev 1991 ;114(2): 127-33.
4. 4. Huang PP, Doyle WK, Abbott IR. Atypical meningioma of the third ventricle in a 6 year-old boy. Neurosurgery 1993;33(2):312-5.
5. 5. Murai Y, Yoshida D, Ikeda Y, Teramoto A, Kojima T, Ikakura K. Spontaneous intraventricular hemorrhage caused by lateral ventricular meningioma— case report. Neurol Med Chir (Tokyo) 1996;36(8):586-9.
6. 6. Vialta J, Gaston F, Rubio E, Barba JM, Castano CH, Bosch J, Conde C, Poch JM. Intraventricular meningiomas. Neurologia 1992;7(3):94-6.
7. 7. Yoshida K, Onozuka S, Kawase T, Ikeda E. Lateral ventricular meningioma encapsulated by the duralike membrane. Neuropathology 2000;20(1):56-9.
8. 8. Ceylan S, İlbay K, Kuzeyli K, Kalelioglu M, Akturk F, Ozoran Y Intraventricular meningioma of the fourth ventricle. Clin Neurol Neurosurg 1992; 94:181-184.

9. 9. Lang I, Jackson A, Strang FA. Intraventricular hemorrhage caused by intraventricular meningioma: CT appearance. AJNR Am J Neuroradiol 1995; 16:1378-1381.
10. 10. Bernard T Lyngdoh, Pramod J. Giri, Sanjay Behari *, Deepu Banerji, Devendra K. Chhabra, Vijendra K. Jain. Intraventricular meningiomas: A surgical challenge Journal of Clinical Neuroscience 2007; 14: 442-448.
11. 11. D Gareth R Evans,M Sainio, Michael E Baser, Neurofibromatosis type 2, J Med Genet 2000;37:897-904.
12. 12. Evans DGR, Huson SM, Don-nai D, Neary W, Blair V, Newton V, Harris R. A clinical study of type 2 neurofibromatosis.Q J Med 1992;84:603-18.
13. 13. Parry DM, Eldridge R, Kaiser-Kupfer Ml, Bouzas EA, Pikus A, Patronas N. Neurofibromatosis 2 (NF2): clinical characteristics of 63 affected individuals and clinical evidence for heterogeneity. Am J Med Genet 1994;52:450-1.
14. 14. Mautner VF, Lindenau M, Baser ME, Kluwe L, Gottschalk J. Skin abnormalities in neurofibromatosis 2. Arch Dermatol 1997;133:1539-43.
15. 15. Evans DGR, Huson SM, Donnai D, NearyW, Blair V.Teare D, Newton V, Strachan T, Ramsden R, Harris R. A genetic study of type 2 neurofibromatosis in the United Kingdom. I. Prevalence, mutation rate, fitness, and confirmation of maternal transmission effect on severity. JMed Genet 1992; 29:841-6.
16. 16. DGR Evans, C Watson, A King, A J Wallace, M E Baser, Multiple meningiomas: differential involvement of the NF2 gene in children and adults, J Med Genet 2005;42:45-48.
17. 17. Meng Liu . Yuan Wei . Yuguang Liu . Shugan Zhu . Xingang Li Intraventricular meninigiomas: a report of 25 cases Neurosurg Rev 2006; 29; 36-40.