Zafer AYDIN, Esra ERDEN, Ertekin ARASIL, Ayşe KARATAŞ, Ağahan ÜNLÜ, Kağan TUN, Gökmen KAHİLOĞULLARI

Turcot sendromu: Olgu sunumu

Turcot sendromu; genetik geçişli, kolonda familyal adenomatöz polipozisle beraber, santral sinir sisteminin nöro-epitelial tümörünün birlikteliği ile olan bir sehdromdur. Bu sendromda; glial tümörler ve medulloblastomalar santral sinir sisteminde en sık görülen tümörlerdir. Bu olgu sunumunda; kolon tümörü nedeniyle opere edilen ve takiben glioblastoma multiforme tanısı alarak öpere edilen genç bir Turcot sendromu hastası sunularak; nöroşirürjikal önemi olan bu sendrom literatür bilgileri ışığı altında tartışılmıştır.

Turcot syndrome: A case report

Turcot Syndrome is a genetic disease presented by familial adenomatous polyposis in colon and neuroepitelial neoplasms in central nervous system. Glial tumors and medulloblastoma are the most common intracranial neoplasms seen in this syndrome. in this case repon, a young patient with Turcot Syndrome iş presented whom underwent surgery because of a colon tümör. A glioblastoma multiforme was diagnosed at follow up period. The neuro-surgical significance of this syndrome is discussed.

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1. Gardner E.J., Richards R.C.: Multipl Cutaneus and Subcutaneus Lesions Occoring Simultaneously with Hereditary Polyposis and Osteomatosis;Am j. Human Genetic;1953;5, 139-47
2. Jamjoom Z.A., Sadiq S., Mofti A.B., Al-Mofleh I., Ajarim D.:Turcot Syndrome:Report of a Case and Review of the Literature:lnt. Surg. 1989;74:45-50
3. Mc. Laughlin M.R., Gollin S.M., Leşe CM., Albright A.L.: Medullablastoma and Glioblastoma Multifor- me in A Patient with Turcot Syndrome:A Case Report:Surg. Neurol. 1998;49:295-301
4. TurcotJ., Despres J.P., St. Pierre: Malignant Tumors of the CNS Associated with Familial Polyposis of the Colon:Report of Two Cases: Dis Colon Rec- tum: 959;2:465-8
5. Crail H.W.: Multipl Primary Malignancies Arising in the Rectum, Brain and Thyroid;U.S. Naval Med. Bull;1949;49:123-8
6. Costa O.L., Sılva D.M., Colnago F.A.,Viera M.S., Musso C.:Turcot Syndrome;Autosomal Dominant or Recessive Transmission:Dis Colon Rectum:May 1987:391-4
7. Krokowicz P.:The Turcot Syndrome:Acta Chir Scand: 1979; 145:113-5 8. Paraf B.F., Jothy S., Van Meir E.G.; Brain Tumor- Polyposis Syndrome: Two Genetic Diseases;J. Of Clinical Oncology:July 1997, Vol.15,No:72744-58
9. Kattar M.M., Kupsky W.J., Shimoyama R.K., Vo T.D., Olson M.W., Bargar G.R., Sarkar F.H.; Clonal Analysis of Gliomas-.Human Pathology: Oct.1997; Vol.28 No.10:1166-1179
10. Lewis J.H., Ginsberg A.L., Toomey K.E.: Turcot's Syndrome. Evidence for Autosomal Dominant In- heritance, Cancer; 1983: 51;524-8
11. Rutz H.P., De Tribolet N.,Calmes J.M., Chapuis G.; Long-Time Survival of a Patient with Glioblastoma and Turcot's Syndrome;J. Neurosurgery 1991;74: 813-5
12. Kropilak M., Jagelman D.G., Fazio V.W., Lavery I.L., Mc. Gannon E.: Brain Tumors in Familial Adenomatous Polyposis; Dis Colon Rectum: Sept. 1989 Vol.32. No:9;778-82
13.Taylor D.M., Perry J., Zlatescu M.C..: The hPMS2 exon 5 mutation and malignant glioma; J- Neurosur¬gery 1999; 90:946-50