Article Sidebar

pdf
Date Published: 21/04/2025
Abstract Views: 69
Views pdf: 31
DOI: https://doi.org/10.51298/vmj.v549i1.13614
Issue
Vol. 549 No. 1 (2025)
Section
Các bài báo
How to Cite
Nguyễn Đức, L., & Nguyễn Thái, H. (2025). TREATMENT OF GLIOBLASTOMA IN PATIENTS WITH LYNCH SYNDROME: A RARE CASE REPORT. Vietnam Medical Journal, 549(1). https://doi.org/10.51298/vmj.v549i1.13614

TREATMENT OF GLIOBLASTOMA IN PATIENTS WITH LYNCH SYNDROME: A RARE CASE REPORT

Liên Nguyễn Đức, Học Nguyễn Thái

Main Article Content

Abstract

Gliomas occur in 1-6% of patients with Lynch syndrome, the most common brain tumor being glioblastoma (accounting for 56% of brain tumors in patients with Lynch syndrome). Brain tumors occur in 14% of cases with MSH2 mutations, so brain MRI should be performed every 6 to 12 months to detect brain tumors early in families with Lynch syndrome carrying MSH2 mutations. Treatment of glioblastoma in patients with Lynch syndrome includes maximal resection of the brain tumor and Stupp chemotherapy and radiotherapy, and immunotherapy has been shown to increase survival in individual case reports. Case report: We reported on 2 cases diagnosed with glioblastoma in patients with a history of colon cancer treatment, with PMS2 gene mutation, both patients underwent total brain tumor resection and concurrent chemoradiotherapy according to Stupp protocol. The disease was stable and did not recur at the time of follow-up examination 12-24 months after surgery. 

Article Details

Keywords

glioblastomas, Lynch syndrome, surgery

References

1. Hampel, H.; Frankel, W.L.; Martin, E.; Arnold, M.; Khanduja, K.; Kuebler, P.; Clendenning, M.; Sotamaa, K.; Prior, T.; Westman, J.A.; et al. Feasibility of screening for Lynch syndrome among patients with colorectal cancer. J. Clin. Oncol. 2008, 26, 5783–5788.
2. De Jong, A.E.; Morreau, H.; van Puijenbroek, M.; Eilers, P.H.; Wijnen, J.; Nagengast, F.M.; Griffioen, G.; Cats, A.; Menko, F.H.; Kleibeuker, J.H. The role of mismatch repair gene defects in the development of adenomas in patients with HNPCC. Gastroenterology 2004, 126, 42–48.
3. Nakase K, Matsuda R, Sasaki S, Nakagawa I. Lynch Syndrome-Associated Glioblastoma Treated With Concomitant Chemoradiotherapy and Immune Checkpoint Inhibitors: Case Report and Review of Literature. Brain Tumor Res Treat. 2024 Jan;12(1):70-74
4. 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: 465–468.
5. Paraf F, Jothy S, Van Meir EG. Brain tumor-polyposis syndrome: two genetic diseases? J Clin Oncol 1997; 15: 2744–2758.
6. Therkildsen C, Ladelund S, Rambech E, Persson A, Petersen A, Nilbert M. Glioblastomas, astrocytomas and oligodendrogliomas linked to Lynch syndrome. Eur J Neurol. 2015 Apr;22(4):717-24. doi: 10.1111/ ene.12647. Epub 2015 Jan 19. PMID: 25648859.
7. De Rosa M, Fasano C, Panariello L, Scarano MI, Belli G, Iannelli A, Ciciliano F, Izzo P. Evidence for a recessive inheritance of Turcot's syndrome caused by compound heterozygous mutations within the PMS2 gene. Oncogene. 2000 Mar 23;19(13):1719-23. doi: 10.1038/sj.onc. 1203447. PMID: 10763829.
8. Vasen HF, Stormorken A, Menko FH, Nagengast FM, Kleibeuker JH, Griffioen G, et al. MSH2 mutation carriers are at higher risk of cancer than MLH1 mutation carriers: a study of hereditary nonpolyposis colorectal cancer families. J Clin Oncol (2001) 19:4074–80. doi: 10.1200/JCO.2001.19.20.4074.
9. Barrow E, Robinson L, Alduaij W, Shenton A, Clancy T, Lalloo F, et al. Cumulative lifetime incidence of extracolonic cancers in Lynch syndrome: a report of 121 families with proven mutations. Clin Genet (2009) 75:141–9. doi: 10.1111/j.1399-0004.2008.01125.x
10. Georgescu, M.-M. (2024). Adult glioblastoma with Lynch syndrome-associated mismatch repair deficiency forms a distinct high-risk molecular subgroup. Free Neuropathology, 5, 32.