Doğan KÖSE, Erdal KALKAN, Yahya PAKSOY, Hatice TOY, Nurcan Üçüncü ERGÜN, YAVUZ KÖKSAL

Bir çocukta serebellumun disembriyoplastik nöroepiteliyal tümörü

Disembriyoplastik nöroepitelyial tümörler (DNT) tipik olarak supratentorial yerleşen benign neoplazmalardır. Bilinç ve postural tonus kaybı şikayetleri ile bir lokal hastaneye başvuran 12 yaşındaki erkek hasta saptanan serebellar kitlesi nedeniyle hastanemize refere edildi. Hikayesinden benzer şikayetlerinin 3 ve 10 yaşlarında da tekrar ettiği ancak o dönemde pediatric kardiyolog ve pediatric nörolog tarafından yapılan incelemelerde herhangi bir anomaliye rastlanmadığı öğrenildi. Hastanın fizik muayenesi ve rutin laboratuvar tetkikleri normaldi. Serebellumda saptanan kitle total olarak çıkarıldı. Alınan dokunun histopatolojik değerlendirmesi ile hastaya DNT tanısı konuldu. Hasta halen, 5 yıldır, herhangi bir nüks belirtisi olmadan ayaktan takip edilmektedir. Bu yazıda serebellum yerleşimli DNT'ye sahip bir çocuk vakanın literatürle paylaşılması amaçlanmıştır.

Dysembryoplastic neuroepithelial tumor of cerebellum in a child

Dysembryoplastic neuroepithelial tumors are benign neoplasms with typical supratentorial location. 12 year old patient, who applied to a local hospital with complaints of loss of consciousness and postural tonus, was referred to our hospital with cerebellar mass diagnosis. According to the anamnesis, similar complaints also appeared when the patient was 3 and 10 years old, however no anomaly was found in the examinations done by pediatric cardiologist and pediatric neurologist in that period. Physical examination and routine laboratory examinations of the patient were normal. The mass identified in the cerebellum was totally removed. The patient was diagnosed as dysembryoplastic neuroepithelial tumor by histopathological evaluation of the tissue. The patient has still been under outpatient follow-up and in complete remission without any evidence of recurrence for 5 years. In this report a child patient having dysembryoplastic neuroepithelial tumor with cerebellar location is aimed to be shared with the literature.

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1.Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, et al. Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. Neurosurgery 1988; 23:545-56.
2.Rosemberg S, Vieira GS. Dysembryoplastic neuroepithelial tumor. An epidemiological study from a single institution. Arq Neuropsiquiatr 1998; 56:232-6.
3.Daumas-Duport C, Pietsch T, Lantos PL. Dysembryoplastic neuroepithelial tumour. In: Kleihues P, Cavenee WK eds. Pathology and genetics of tumours of the nervous system. 2nd ed. Lyon: IARC, 2000; 103-6.
4.Thom M, Gomez-Anson B, Revesz T, et al. Spontaneous intralesional haemorrhage in dysembryoplastic neuroepithelial tumours: a series of five cases. J Neurol Neurosurg Psychiatry 1999; 67:97-101.
5.Onguru O, Deveci S, Sirin S, et al. Dysembryoplastic neuroepithelial tumor in the left lateral ventricle. Minim Invasive Neurosurg 2003; 46:306-9.
6.Whittle IR, Dow GR, Lammie GA, et al. Dsyembryoplastic neuroepithelial tumour with discrete bilateral multifocality: further evidence for a germinal origin. Br J Neurosurg 1999; 13:508-11.
7.Kuchelmeister K, Demirel T, Schlörer E, et al. Dysembryoplastic neuroepithelial tumour of the cerebellum. Acta Neuropathol 1995; 89:385-90.
8.Leung SY, Gwi E, Ng HK, et al. Dysembryoplastic neuroepithelial tumor. A tumor with small neuronal cells resembling oligodendroglioma. Am J Surg Pathol 1994; 18:604-14.
9.Yasha TC, Mohanty A, Radhesh S, et al. Infratentorial dysembryoplastic neuroepithelial tumor associated with Arnold-Chiari malformation. Clin Neuropathol 1998; 17:305-10.
10.Fujimoto K, Ohnishi H, Tsujimoto M, et al. Dysembryoplastic neuroepithelial tumor of the cerebellum and brainstem. Case report. J Neurosurg 2000; 93:487-9.
11.Litrico S, Desjardins T, Dran G, et al. Infratentorial localisation of a dysembryoplastic neuroepithelial tumor. A case report. Neurchirurgie 2004; 50:47-52.
12.Tailor JK, Kim AH, Folkerth RD, et al. The development of ring-shaped contrast enhancement in a case of cerebellar dysembryoplastic neuroepithelial tumor: case report. Neurosurgery 2008; 63:609-10.
13.Vaquero J, Saldaña C, Coca S, et al. Complex form variant of dysembryoplastic neuroepithelial tumor of the cerebellum. Case Rep Pathol, 2012: 718651. doi: 10.1155/2012/718651.
14.O'Brien DF, Farrell M, Delanty N, et al. The Children's Cancer and Leukaemia Group guidelines for the diagnosis and management of dysembryoplastic neuroepithelial tumours. Br J Neurosurg 2007; 21:539-49.
15.Sarrazin JL, Hélie O, Lévêque C, et al. Tumeurs de la fosse cérébrale postérieure de l'adulte. Encycl Med Chir, Radiodiagnostic-NeuroradiologieAppareil Locomoteur 1999; 10:16.
16.Campos AR, Clusmann H, von Lehe M, et al. Simple and complex dysembryoplastic neuroepithelial tumors (DNT) variants: clinical profile, MRI, and histopathology. Neuroradiology 2009; 51:433-43.
17.Yu AH, Chen L, Li YJ, et al. Dysembryoplastic neuroepithelial tumors: magnetic resonance imaging and magnetic resonance spectroscopy evaluation. Chin Med J 2009; 122:2433-7.
18.Chan CH, Bittar RG, Davis GA, et al. Long-term seizure outcome following surgery for dysembryoplastic neuroepithelial tumor. J Neurosurg 2006; 104:62-9.
19.Schittenhelm J, Mittelbronn M, Wolff M, et al. Multifocal dysembryoplastic neuroepithelial tumor with signs of atypia after regrowth. Neuropathology 2007; 27:383-9.
20.Ray WZ, Blackburn SL, Casavilca-Zambrano S, et al. Clinicopathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature. J Neurooncol 2009; 94:283-92.