Hostname: page-component-7c8c6479df-995ml Total loading time: 0 Render date: 2024-03-29T02:36:42.314Z Has data issue: false hasContentIssue false

Glioblastoma in a Patient with a Hereditary Cancer Syndrome

Published online by Cambridge University Press:  02 December 2014

J L Steckley
Affiliation:
Department of Clinical Neurological Sciences, London Health Sciences Center, University Hospital, London, Ontario, Canada
C J Watling
Affiliation:
Department of Clinical Neurological Sciences, London Health Sciences Center, University Hospital, London, Ontario, Canada
W. Ng
Affiliation:
Department of Clinical Neurological Sciences, London Health Sciences Center, University Hospital, London, Ontario, Canada
Rights & Permissions [Opens in a new window]

Extract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

A 68-year-old Caucasian male presented to emergency with a spontaneous sudden onset headache localized behind the left eye. Pain was severe and associated with vomiting, agitation, anxiety, memory loss, and word-finding difficulty. There was no preceding trauma and no meningeal symptoms. Further history was significant for mitral valve repair, atrial fibrillation treated with coumadin, colorectal cancer treated with right-sided hemicolectomy 17 years prior, and multiple skin tumors, which included squamous cell and basal cell carcinomas of his face and ears. Family history was significant for colon cancer in his father (at age 50), a sister (at age 40), and a niece (at age 38), as well as primary brain cancer in his paternal aunt.

Type
Peer Reviewed Letter
Copyright
Copyright © The Canadian Journal of Neurological 2008

References

1. Chung, DC, Rustgi, AK. The hereditary nonpolyposis colorectal cancer syndrome: genetics and clinical implications. Ann Intern Med. 2003; 138: 56070.Google Scholar
2. Lebrun, C, Olschwang, S, Jeannin, S, Vandenbos, F, Sobol, H, Frenay, M. Turcot syndrome confirmed with molecular analysis. Eur J Neurol. 2007; 14: 4702.Google Scholar
3. Turcot, J, Despres, JP, St. Pierre, F. Malignant tumors of the central nervous system associated with familial polyposis of the colon: report of two cases. Dis Colon Rectum. 1959; 2: 4658.Google Scholar
4. Melean, G, Sestini, R, Ammannati, F, Papi, L. Genetic insights into familial tumors of the nervous system. Am J Med Genet C Semin Med Genet. 2004; 129(1):7484.CrossRefGoogle Scholar
5. Hamilton, SR, Liu, B, Parsons, RE, Papadopoulos, N, Jen, J, Powell, SM, et al. The molecular basis of Turcot’s syndrome. New Eng J Med. 1995; 332: 83947.Google Scholar
6. Paraf, F, Jothy, S, Van Meir, EG. Brain tumor-polyposis syndrome: two genetic diseases? J Clin Oncol. 1997; 15: 274458.Google Scholar
7. Hegde, MR, Chong, B, Blazo, ME, Chin, LHE, Ward, PA, Chintagumpala, MM, et al. A homozygous mutation in MSH6 causes Turcot syndrome. Clin Cancer Res. 2005; 11(13):468993.CrossRefGoogle ScholarPubMed
8. Felton, KEA, Gilchrist, DM, Andrew, SE. Constitutive deficiency in DNA mismatch repair. Clin Genet. 2007; 71: 48398.Google Scholar
9. Felton, KEA, Gilchrist, DM, Andrew, SE. Constitutive deficiency in DNA mismatch repair: is it time for Lynch III? Clin Genet. 2007; 71: 499500.Google Scholar
10. Aarnio, M, Sankila, R, Pukkala, E, Salovaara, R, Aaltonen, LA, de la Chapelle, A, et al. Cancer risk in mutation carriers of DNA-mismatch-repair genes. Int J Cancer. 1999; 81(2):2148.Google Scholar
11. Muir, GG, Bell, AY, Barlow, KA. Multiple primary carcinomata of the colon, duodenum, and larynx associated with keratoacanthomata of the face. Brit J Surg. 1967; 54: 1915.Google Scholar
12. Torre, D. Multiple sebaceous tumors. Arch Derm. 1968; 98: 54951.Google Scholar
13. Ponti, G, Ponz de Leon M. Muir-Torre syndrome. Lancet Onc. 2004; 6: 9806.Google Scholar
14. Okamoto, H, Mineta, T, Nakahara, Y, Ichinose, M, Shiraishi, T, Tabuchi, K. Molecular analysis of astrocytoma associated with Turcot Syndrome type 1 - case report. Neurol Med Chir (Tokyo). 2004; 44: 1248.CrossRefGoogle ScholarPubMed
15. Watwe, V, Farrell, CD, LeVea, C, Javle M. Muir-Torre syndrome associated with Turcot syndrome. The Internet Journal of Gastroenterology. 2005; 3(2).Google Scholar
16. Houshmandi, SS, Gutmann, DH. All in the family: using inherited cancer syndromes to understand de-regulated cell signaling in brain tumors. J Cell Biochem. 2007; Nov 1;102(4):81119.Google Scholar
17. Creange, A, Zeller, J, Rostaing-Riggattieri, S, Brugieres, P, Degos, JD, Revuz, J, et al. Neurological complications of neurofibromatosis type 1 in adulthood. Brain. 1999; 122(3):47381.Google Scholar
18. Bahuau, M, Vidaud, D, Jenkins, RB, Bieche, I, Kimmel, DW, Assouline, B, et al. Germ-line deletion involving the INK4 locus in familial proneness to melanoma and nervous system tumors. Cancer Res. 1998; 58(11):2298303.Google Scholar
19. Wilkes, D, McDermott, DA, Basson, CT. Clinical phenotypes and molecular genetic mechanisms of Carney complex. Lancet Onc. 2005; 6(7):5018.Google Scholar
20. Maxwell, M, Shih, SD, Galanopoulos, T, Hedley-White, ET, Cosgrove, GR. Familial meningioma: analysis of expression of neurofibromatosis 2 protein merlin: report of 2 cases. J Neurosurg. 1998; 88(3):5629.Google Scholar
21. Hulsebos, TJ, Plomp, AS, Wolterman, RA, Robanus-Maandaq, EC, Baas, F, Wesseling, P. Germline mutation of INI1/SMARCB1 in familial schwannomatosis. Am J Hum Genet. 2007; 80(4): 80510.Google Scholar
22. Pannu, N, Lahermo, P, Onkamo, P, Ollikainen, V, Rantala, I, Helen, P, et al. A novel low-penetrance locus for familial glioma at 15q23-q26.3. Cancer Res. 2002; 62(13):3798802.Google Scholar