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Current Cancer Therapy Reviews

Editor-in-Chief

ISSN (Print): 1573-3947
ISSN (Online): 1875-6301

Systematic Review Article

Possible Considerations for the Management of Turcot’s Syndrome?

Author(s): Alexandrina Nikova*, Dimitar Ganchev and Theodossios Birbilis

Volume 15, Issue 2, 2019

Page: [146 - 154] Pages: 9

DOI: 10.2174/1573394714666180731094420

Price: $65

Abstract

Background: Turcot’s syndrome (TS) is a rare disease with known incidence of about 1-2 cases per year. It is, however, linked to high mortality due to the brain cancer. And because of this, we propose recommendations, aimed at preventing the mortality of the patients and to minimize the risk of undiagnosed Turcot’s syndrome.

Methods: The authors collected the worldwide published data on TS, from the year of its definition till 2018, all of which was published on the search engines, such as Medline, Medknow, Cohraine and Wiley.

Results: We included 97 patients, 57 from which are females and 40 males with median age of 22 years. The most common type of cancer is medulloblastoma, followed by glioblastoma and astrocytoma. We further divided the patients into two categories based on the first symptom of the disease and we made an algorithm of approaching these patients.

Conclusion: TS is a disease that affects mostly members of families with multiple genetic mutations and types of cancers. And because of the unknown mechanisms of inheritance, it is useful to establish guidelines for the approach of those patients, in order to minimize the high mortality rate.

Keywords: Brain tumor, Turcot's syndrome (TS), FAP, HNPCC, prevention, adenocarcinoma.

Graphical Abstract
[1]
Turcot J, Desprks 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: 465-8.
[2]
Hamilton SR, Liu B, Parsons RE, et al. The molecular basis of Turcot Syndrome. N Engl J Med 1995; 332: 839-47.
[3]
Van Meir EG. Turcot’s syndrome: Phenotype of brain tumors, survival and mode of inheritance. Int J Cancer 1998; 75: 162-4.
[4]
Jamjoom ZAB, Sadiq S, Mofti AB, Al-Mofleh I, Ajarim D. Turcot Syndrome: Report of a case and review of the literature. Int Surg 1989; 74: 45-50.
[5]
Chowdhary UM, Boehme DH. AI-Jishi M. Turcot’s syndrome (glioma-polyposis). Case report. J Neurosurg 1985; 63: 804-7.
[6]
Lewis JH, Ginsberg AL, Toomey KE. Turcot’s syndrome: Evidence for autosomal dominant inheritance. Cancer 1983; 51: 524-8.
[7]
Itoh H, Ohsato K, Yao T, Iida M, Watanabe H. Turcot’s syndrome and its mode of inheritance. Gut 1979; 20: 414-9.
[8]
Matsui T, Hayashi N, Yao K, et al. A father and son with Turcot’s syndrome: evidence for autosomal dominant inheritance: Report of two cases. Dis Colon Rectum 1998; 41: 797-801.
[9]
Mullins KJ, Rubio A, Myers SP, Korones DN, Pilcher WH. Malignant Ependymomas in a Patient with Turcot’s Syndrome: Case report and management guidelines. Surg Neurol 1998; 49: 290-4.
[10]
Tithecott GA, Filler TR, Sherman PM. Turcot’s syndrome: A diagnostic consideration in a child with primary adenocarcinoma of the colon. J Pediatr Surg 1989; 24: 1189-91.
[11]
Niizuma K, Fujimura M, Kumabe T, Tominaga T. Malignant transformation of high-grade astrocytoma associated with neurocysticercosis in a patient with Turcot syndrome. J Clin Neurosci 2007; 14: 53-5.
[12]
Li FP, Little JB, Bech-Hansen NT, et al. Acute leukemia after radiotherapy in a patient with Turcot’s syndrome. Am J Med 1983; 74: 343-8.
[13]
Newton HB, Rosenblum MK, Malkin MG. Turcot’s Syndrome. Cancer 1991; 68: 1633-9.
[14]
Everson RB, Fraument J. Familial glioblastoma with hepatic focal nodular hyperplasia. Cancer 1976; 38: 310-3.
[15]
Kawanami K, Ohno M, Matsuura K, Soejima T, Watanabe H, Enjoji M. Turcot’s syndrome. Report of an autopsy case. Stomach Intestine 1976; 11: 1075-82.
[16]
Binder MK, Zablen MA, Fleischer DE, Sue DY, Dwyer RM, Hanelin L. Colon polyps, sebaceous cysts, gastric polyps and malignant brain tumor in a family. Dig Dis 1978; 23: 460-6.
[17]
Michels VV, Stevens C. Basal cell carcinoma in a patient with intestinal polyposis. Clin Genet 1982; 22: 80-2.
[18]
Kleinerman R, Marino J, Loucas E. Muir-Torre Syndrome/Turcot Syndrome overlap? A patient with sebaceous carcinoma, colon cancer, and a malignant astrocytoma. Dermatol Online J 2012; 18: 3.
[19]
Arico M, Parigi GB, Locatelli D, Bragheri R, Lornbardi F, Zangrand A. Turcot’s syndrome with intestinal lymphoma in a child: An unusual case of triple tumor. Med Pediatr Oncol 1990; 18: 252-5.
[20]
Torres CF, Korones DN, Pilcher W. Multiple ependymomas in a patient with Turcot’s syndrome. Med Pediatr Oncol 1997; 28: 59-61.
[21]
Ikeda J, Sawamura Y, Van Meir EG. Pineoblastoma presenting in familial adenomatous polyposis (FAP): Random association, FAP variant or Turcot syndrome? Br J Neurosurg 1998; 12: 576-8.
[22]
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: 124-8.
[23]
Kropilak M, Jagelman DG, Fazio VW, Lavery IL, McGannon E. Brain tumors in familial adenomatous polyposis. Dis Colon Rectum 1989; 32: 778-2.
[24]
Chan TL, Yuen ST, Chung LP, et al. Germline hMSH2 and differential somatic mutations in patients with Turcot’s syndrome. Genes Chromosomes Cancer 1999; 25: 75-81.
[25]
Hamoudi AB, Ertel I, Newton WA Jr, Reiner CB, Clatworthy W. Multiple neoplasms in adolescent child associated with IGA deficiency. Cancer 1974; 33: 1134-44.
[26]
Cohen SB. Familial polyposis coli and its extracolonic manifestations. J Med Genet 1982; 19: 193-203.
[27]
Baehring J, Hui P, Piepmeier J, Bannykh SI. Anaplastic oligoastrocytoma in Turcot syndrome. J Neurooncol 2009; 95: 293-8.
[28]
Mihalatos MI, Danielides I, Fountzilas G, et al. Novel mutations of the APC gene in familial adenomatous polyposis in Greek patients. Cancer Genet Cytogenet 2003; 141: 65-70.
[29]
Ma SC, Hu YS, Yang J, Zhou XN. Turcot’s syndrome associated with intestinal non-Hodgkin’s lymphoma: Case report and review of literature. Clin Neurol Neurosurg 2013; 115: 117-20.
[30]
Shibata C, Sasaki I, Naito H, et al. Turcot syndrome with colonic obstruction and small intestinal invagination: Report of a case. Surg Today Jpn J Surg 1999; 29: 785-8.
[31]
Mori T, Nagase H, Horii A, et al. Germ-line and somatic mutations of the apc gene in patients with Turcot syndrome and analysis of apc mutations in brain tumors. Genes Chromosomes Cancer 1994; 9: 168-72.
[32]
Rochlitz CF, Heide I, Neubauer EKA, et al. Molecular alterations in a patient with Turcot’s syndrome. Br J Cancer 1993; 68: 519-23.
[33]
Castillo R, Wilson MMG. Turcot syndrome in an elderly adult. J Clin Gastroenterol 2002; 34: 449-50.
[34]
Dipro S, Al-Otaibi F, Alzahrani A, Ulhaq A, Shail EA. Turcot syndrome: A synchronous clinical presentation of glioblastoma multiforme and adenocarcinoma of the colon. Case Rep Oncol Med 2012; 2012720273
[35]
Peserico L, Merli GA, Cortesi N, Canossi GC, Crisi G. The Turcot syndrome. Case report. Ital J Neurol Sci 1981; 4: 391-4.
[36]
Scapa E, Umlas J, Loewenstein MS, Zamcheck N. Non-familial Turcot’s syndrome associated with Crohn’s disease and duodenal ulcer in one kindred. Am J Gastroenterol 1983; 78: 411-2.
[37]
Kamiya J, Muto T, Morioka K. Turcot syndrome. Report of a case. Stomach Intestine 1982; 17: 913-7.
[38]
Schroder S, Moehrs D, Weltzien J, Winkler R, Otto HF. The Turcot syndrome. Report of an additional case and review of the literature. Dis Colon Rectum 1983; 26: 533-8.
[39]
Herrera-Ornelas L, Ochi H, Petrelli N, Mittelman A, Sandberg AA. Non-familial Turcot’s syndrome associated with Turner’s syndrome, multiple carcinomas of the tongue, and cancer of the colon. J Surg Oncol 1984; 27: 251-4.
[40]
Minami T, Shinomura Y, Tarui S, et al. Turcot’s syndrome: Report of an adult case. Dig Endosc 1992; 4: 68-75.
[41]
Anseline PF. Turcot’s syndrome. Aust N Z J Surg 1992; 62: 587-90.
[42]
Costa OL, Silva DM, Colnago F, Vieira MS, Musso C. Turcot syndrome: Autosomal dominant or recessive transmission? Dis Colon Rectum 1987; 30: 391-4.
[43]
Suzul M, Yoshimi N, Ham A, Morishita Y, Tanaka T, Mori H. Genetic alterations in a patient with Turcot’s syndrome. Pathol Int 1998; 48: 126-33.
[44]
Todd DW, Christoferson LA, Leech RW, Rudolf L. A family affected with intestinal polyposis and gliomas. Ann Neurol 1981; 10: 390-2.
[45]
Baughman FA Jr, List CF, Williams JR, Muldoon JP, Segarra JM, Volkel JS. The gliomapolyposis syndrome. N Engl J Med 1969; 281: 1345-6.
[46]
Radin DR, Fortgang K, Zee CS, Mikity VG, Halls JM. Turcot syndrome: A case with spinal cord and colonic neoplasms. AJR 1984; 142: 475-6.
[47]
Skomolowski M, Taxier M, Wise W Jr. Turcot syndrome type 2: Medulloblastoma with multiple colorectal adenomas. Clin Gastroenterol Hepatol 2012; 10(10): A24.
[48]
McLaughlin MR, Gollin SM, Lese CM, Albright AL. Medulloblastoma and glioblastoma multiforme in a patient with Turcot syndrome: A case report. Surg Neurol 1998; 49: 295-301.
[49]
Barel D, Cohen IJ, Mor C, et al. Mutations of the adenomatous polyposis coli and p53 genes in a child with Turcot’s syndrome. Cancer Lett 1998; 132: 119-2.
[50]
Chung HJ, Oh ST, Kim JG, Kang WK. Turcot Syndrome: A case report in an unsuspected setting. J Gastrointest Surg 2012; 16: 411-4.
[51]
Lebrun C, Olschwang C, Jeannin S, Vandenbos F, Sobol H, Frenay M. Turcot syndrome confirmed with molecular analysis. Eur J Neurol 2007; 14: 470-2.
[52]
Schneider T, Victor S. The Turcot Syndrome (Glioma polyposis) and its neurosurgical significance. Case report. Neurosurg Rev 1993; 16: 327-30.
[53]
Grips E, Wentzensen N, Sutter C, et al. Glioblastoma multiforme als manifestation des Turcot-syndroms. Nervenarzt (German) 2002; 73: 177-82.
[54]
Koontz NA, Hess CP. AJR teaching file: Brain tumor in a patient with familial adenomatous polyposis. AJR 2010; 195: S25-8.
[55]
Gadish T, Tulchinsky H, Deutsch AA, Rabau M. Pinealoblastoma in a patient with familial adenomatous polyposis: Variant of Turcot syndrome type 2? Report of a case and review of the literature. Dis Colon Rectum 2005; 48(12): 2243-6.
[56]
Gorovoy IR, de Alba Campomanes A. A potential life-saving diagnosis-recognizing Turcot syndrome. J AAPOS 2014; 18: 186-8.
[57]
Bulus H, Tas A, Koklu S. Gastrointestinal stromal tumor in Turcot syndrome. Gastroenterol Nurs 2015; 38: 126-7.
[58]
Dora V, Diego G, R’omulo B, Natalia O. First Case Report of Turcot Syndrome Type 1 in Colombia. Case Rep Oncol Med 2012; 2012356384
[59]
Ozerov SS, Zakharov IV, Talypov SR, et al. Turcot syndrome. A rare case and literature review. Vopr Neirokhir 2013; 77: 49-53.
[60]
Krokowicz P. The Turcot syndrome. Acta Chir Scand 1979; 145: 113-5.
[61]
Smoll NR, Drummond KJ. The incidence of medulloblastomas and primitive neurectodermal tumours in adults and children. J Clin Neurosci 2012; 19: 1541-4.
[62]
Di Maggio FM, Minafra L, Forte GI, et al. Portrait of inflammatory response to ionizing radiation treatment. J Inflamm (London, England) 2015; 12: 14.
[63]
Wong GH. Protective roles of cytokines against radiation: Induction of mitochondrial MnSOD. BBA Mol Basis Dis 1995; 1271: 205-9.
[64]
Schaue D, Kachikwu EL, McBride WH. Cytokines in radiobiological responses: A review. Radiat Res 2012; 178: 505-23.
[65]
Neta R. Modulation of radiation damage by cytokines. J Stem Cells 2009; 15: 87-94.
[66]
Desai S, Kumar A, Laskar S, Pandey BN. Cytokine profile of conditioned medium from human tumor cell lines after acute and fractionated doses of gamma radiation and its effect on survival of bystander tumor cells. Cytokine 2013; 61: 54-62.
[67]
Tops CMJ, Vasen HFA, van Berge Henegouwen G, et al. Genetic evidence that Turcot syndrome is not allelic to familial adenomatous polyposis. Am J Med Genet 1992; 43: 888-93.
[68]
Tops CMJ, van de Klift HM, van der Luijt RB, et al. Non-allelic heterogeneity of familial adenomatous polyposis. Am J Med Genet 1993; 47: 563-7.
[69]
Pino MS, Chung DC. The Chromosomal instability pathway in colon cancer. Gastroenterology 2010; 138: 2059-72.
[70]
Kinzler KW, Vogelstein B. Lessons from hereditary colorectal cancer. Cell 1996; 87: 159-70.
[71]
Haigis KM, Kendall KR, Wang Y, et al. Differential effects of oncogenic K-Ras and N-Ras on proliferation, differentiation and tumor progression in the colon. Nat Genet 2008; 40: 600-8.
[72]
Deng J, Miller SA, Wang HY, et al. β-catenin interacts with and inhibits NF-B in human colon and breast cancer. Cancer Cell J 2002; 2: 323-34.
[73]
Boland CR, Goel A. Microsatellite instability in colorectal cancer. Gastroenterology 2010; 138(6): 2073-87.
[74]
Rehana K, Tabish SA, Gojwari T, Ahmad R, Abdul H. Unit cost of CT scan and MRI at a large tertiary care teaching hospital in North India. Health 2013; 5: 2059-63.
[75]
Haslam R, El-Khassawneh S, Sucher U. The cost of colonoscopy – a worldwide view. Gut 2013; 62(Suppl. 2): A1-A50.
[76]
Holmquist L, Russo A, Elixhauser A. Hospitalizations for Brain Cancer 2006.Statistical brief #68. Available form:. https://www.ncbi.nlm.nih.gov/pubmed/21595138
[77]
Russo A, Stocks C. Hospitalizations for colon cancer 2006.HCUP: Statistical brief #69. Available form:. https://www.ncbi.nlm.nih.gov/pubmed/21595136

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