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Turcot Syndrome: A Synchronous Clinical Presentation of Glioblastoma Multiforme and Adenocarcinoma of the Colon

Turcot Syndrome: A Synchronous Clinical Presentation of Glioblastoma Multiforme and... Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2012, Article ID 720273, 6 pages doi:10.1155/2012/720273 Case Report Turcot Syndrome: A Synchronous Clinical Presentation of Glioblastoma Multiforme and Adenocarcinoma of the Colon 1 2 2 2 1, 2 Sabiq Dipro, Faisal Al-Otaibi, Adel Alzahrani, Anwar Ulhaq, and Essam Al Shail Alfaisal University, Riyadh 11533, Saudi Arabia Division of Neurological Surgery, Department of Neurosciences, King Faisal Specialist Hospital & Research Center, P.O. Box 3354, Riyadh 11211, Saudi Arabia Correspondence should be addressed to Faisal Al-Otaibi, faisalruwais@gmail.com Received 30 August 2012; Accepted 23 September 2012 Academic Editors: E. Itakura and Z. Madjd Copyright © 2012 Sabiq Dipro et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Turcot syndrome (TS) is a rare hereditary disorder clinically characterized by the occurrence of primary tumors of the colon and the central nervous system (CNS). Here we present the case of an 11-year-old boy with a synchronous clinical presentation of both glioblastoma multiforme (GBM) and colonic adenocarcinoma. A molecular genetic study revealed microsatellite instability in the DNA mismatch repair (MMR) gene. This patient ultimately survived for 13 months after clinical presentation. Based on this case study, the synchronous presentation of glioblastoma multiforme and adenocarcinoma of the colon might suggest a shorter survival rate for patients with Turcot syndrome. A literature review complements this paper. 1. Introduction 2. Case Report Turcot syndrome (TS) was originally described by Turcot An 11-year-old boy presented with headache for the past 5 in 1959 [1–3]. It is characterized by the development of days, vomiting, and history of a single tonic clonic seizure. He primary tumors of the central nervous system (CNS), such had no history of weakness, loss of consciousness, or memory as glioblastoma multiforme (GBM) and medulloblastoma, disturbance. He was also suffering from vague abdominal along with numerous adenomatous colorectal polyps and pain and loose motion over a period of one month prior colonic adenocarcinoma. TS usually develops in the patient’s to presentation. He had positive family history of colonic teens and can be genetically distinguished as either familial cancer. His sister had multiple cafe-au-lait ´ spots and multiple adenomatous polyposis (FAP) or hereditary nonpolyposis colonic polyps. colon carcinoma (HNPCC) [4]. The syndrome was divided During clinical examination, he was fully awake and into two types by Paraf and colleagues in 1997 [5]. TS oriented with a Glasgow coma scale (GCS) score of 15. His type I is characterized by the presence of glial tumors, pupils were bilaterally equal and reacting; no motor or sen- relatively few colonic polyps, and cancer. TS type II is sory deficit was noticed. His gait and posture were normal. characterized by thousands of colonic polyps and increased On his skin there were multiple cafe-au-lait ´ spots and areas of risk of medulloblastoma. hypopigmentation (Figure 1). Brain CT showed that he had Since the time when this syndrome was first identified, a4 × 4 cm left frontal cystic tumor with perifocal edema, there have only been isolated case reports or case series effacement of left lateral ventricle, and subfalcine herniation. with a limited number of patients being discussed in the The brain appeared tight. The tumor had an intense ring- literature. The synchronous clinical presentation of CNS enhancing rim. He underwent left-frontal craniotomy and tumors and colonic adenocarcinoma in this syndrome is gross total resection of the tumor. Histopathology showed extremely rare. In this paper, we present a case of TS GBM with giant-cell features (WHO grade IV) (Figure 1). with simultaneous clinical presentation of GBM and colonic Immunohistochemistry of the tumor showed positive p53 adenocarcinoma. and Ki-67 >90%. 2 Case Reports in Oncological Medicine (a) (b) (c) (d) Figure 1: T1 axial MRI brain depicting left frontal tumor with rim enhancement associated with midline shift, (a) and after resection MRI brain demonstrates no residual tumor and a resolved mass effect (b). Photograph of the patient skin cafe-au-lait ´ spots (c). (d) Histopathology shows high mitotic index and multinucleated giant cells representing GBM features. Hematoxylin and eosin stain 5 400. He continued to have abdominal pain and diarrhea and Nine months later, he presented with headache and developed melena and intermittent frank bleeding from was found to have mild right hemiparesis with an overall Karnofsky performance score of over 90. CT and MRI of the rectum. His blood cell count, platelets, and renal and the brain showed a left temporal ring-enhancing lesion coagulation profiles were within the normal range. Upper about 4 × 5 cm in size with uncal herniation (Figure 3). He GI endoscopy was normal; however, colonoscopy showed also had another lesion at the site of the previous surgery. multiple colonic polyps of various sizes with ulceration He underwent redo left temporal craniotomy and gross and active bleeding (Figure 2). Punch biopsy of the polyps total resection of the tumor. The biopsy report revealed revealed grade 2 adenocarcinoma (Figure 2). A molecular pathological features similar to those of GBM. He was genetic workup of the patient showed mutation in the DNA started on temozolomide as palliative therapy. Three months mismatch repair (MMR) gene with microsatellite instability. later, he had multiple recurrences and dissemination of the He was diagnosed with Turcot syndrome with cerebral GBM cerebral tumors on the left temporal, multifocal frontal, and and adenocarcinoma of the colon. For adenocarcinoma of parietal areas. At the same time, he had a massive progression the colon, he underwent laparoscopic total colectomy and of colonic carcinoma (Figure 3). He died 13 months after the ileocolic anastomosis. A month later, he had recurrent bleed- initial diagnosis. ing from the rectum and developed new rectal polyps. To address this issue, he underwent proctectomy and terminal 3. Discussion ileostomy. The patient received adjuvant radiotherapy for the brain cancer and chemoradiotherapy for the colonic Turcot syndrome is also known as brain-tumor polyposis cancer. and is characterized by the occurrence of primary tumors of Case Reports in Oncological Medicine 3 (a) (b) (c) (d) Figure 2: Colon fiberscopy demonstrates ulcerative adenoma (arrow; (a), (b)). Histopathology slides reveal the adenoma, hematoxylin and eosin stain 5 100. (c) and carcinoma invasion of the colon muscular layer, hematoxylin and eosin stain 5 200 (d). the CNS and multiple colorectal adenomas and/or colonic indices were found to range between 18% and 45%, and adenocarcinoma [5, 11]. From a genetic standpoint, TS can all three GBMs demonstrated positive nuclear p53. The first be divided into two subtypes: the first is manifested by two patients survived 44 and 55 months, respectively, and the MMR gene and PMS2 with a high risk of developing the last patient survived 29 months and was still alive when GBM, and the second type is manifested by the adenomatous the report was published. For our patient, the Ki-67 was polyposis coli gene germline mutation with a high risk of >90% and the tumor showed p53 positivity in addition developing medulloblastoma [5, 16]. However, this classifi- to MMR gene mutation. The tumor did exhibit giant-cell cation cannot be applied to all cases reported in the literature. features. The patient survived only 13 months after initial There are reports of cases with the occurrence of both GBM clinical presentation. The average survival rate of patients and medulloblastoma, as in the report from McLaughlin with TS and GBM was found to be 27 months; however, and colleagues [12]. Nevertheless, their patient was treated when the investigators excluded the two patients with the for medulloblastoma and received whole-brain radiation longest-term survival, the average survival rate dropped to therapy 10 years prior to the diagnosis of GBM. Therefore, 16 months [18, 19]. In contrast, the survival rate of sporadic radiation-induced GBM cannot be excluded. Moreover, the GBM has been found to be around 15 months [20]. GBM occurrence of tumors other than GBM and medulloblastoma and anaplastic astrocytoma are the most commonly reported has been reported [17]. Based on TS classification, our CNS tumor associated with TS. In a review of 33 reported patient fit the clinical and molecular genetic features of TS cases, 21 reports were for GBM and anaplastic astrocytoma type I. [21]. The pathological differentiation between sporadic GBM Most of the reported TS cases in the literature are and TS-associated GBM has not been adequately addressed characterized by different time intervals between the clinical in the literature. Recently, Lusis and colleagues reported on presentation of CNS tumors and the colonic adenocarci- three cases involving patients with TS-associated GBM [11]. noma [18, 22]. The presence of symptomatic CNS GBM and The histopathological and molecular genetic features in all colorectal cancers at the same time carries more management three cases consisted of giant-cell GBM, and the patients difficulties and, more important, might shorten the survival in two of the cases had sarcomatous changes. The Ki-67 rate. Our patient presented with CNS and gastrointestinal 4 Case Reports in Oncological Medicine Table 1: Summary of selected reported cases with high-grade glioma and adenocarcinoma of the colon. Interval between the Survival rate after the No. of diagnosis of high-grade Authors (year) CNS tumors Other associated tumors diagnosis of high-grade cases glioma and colonic glioma adenocarcinoma (i) Adenocarcinoma of the ampullary region Agostini et al. Right frontal giant-cell 1 (ii) Adenomas of the 9months Not reported (2005) [6] GBM transverse and sigmoid colon Castillo and Multiple colonic polyps Wilson 1 Left frontoparietal GBM Same time as diagnosis Not reported with adenocarcinoma (2002) [7] (i) Ovarian Still alive at the age of 25 Chung et al. Right parietotemporal cystadenocarcinoma 1 12 years at the time of reporting (2012) [8] GBM (ii) Adenocarcinoma in the case sigmoid colon Recurring grade 3 Eguchi et al. Adenocarcinoma of the 1 astrocytoma in the right Same time as diagnosis One year (1993) [9] colon parietal lobe (i) Recurring colon cancer (ii) Carcinoma of the right Malignant astrocytoma Kleinerman et al. groin 1 (grade III) of the frontal 12 years One year (2012) [10] (iii) Nodular basal cell of lobe the right nose and the left cheek Two of the patients died after 44 and 55 months of diagnosis, and the Lusis et al. 3, 6, and 6 months, 3 GBM Colorectal adenocarcinoma third was still alive at the (2010) [11] respectively time of writing the article. Survived >29 months since diagnosis (i) Medulloblastoma McLaughlin et al. Adenocarcinoma of the 1 (ii) Right parietal GBM About 10 years A few months (1998) [12] colon Radin et al. GBM of the cervical Adenocarcinoma of the 1 4 months 6 months (1984) [13] spinal cord descending colon The patient died from brain herniation due to Schroder et al. GBM of the left frontal 1 Carcinoma of the jejunum 5 years GBM acute presentation. (1983) [14] lobe The diagnosis was made from autopsy Initial diagnosis was grade Takayama et al. I astrocytoma of left Adenocarcinoma of the 1 6months 18months (1989) [15] frontal lobe, and the ascending colon recurrence was GBM symptoms simultaneously. We elected to treat the CNS cases. Table 1 summarizes selected studies demonstrating the tumor first due to the presence of mass effects that were survival rates of TS after the time of high-grade glioma causing increased intracranial pressure. He was operated diagnosis. on for the GBM recurrence; however, a few months later he had a disseminated GBM involving almost the entire 4. Conclusion left cerebrum that was not amenable to further surgical intervention. This indicates highly malignant disease. The Simultaneous clinical presentation of CNS GBM and ade- presence of GBM in TS may suggest a lower survival nocarcinoma of the colon in TS might be an indicator of a rate in contrast to other CNS tumors in most reported shorter survival rate. Moreover, the presence of GBM may Case Reports in Oncological Medicine 5 Rt Lt Rt Lt (a) (b) (c) Figure 3: MRI brain shows the dissemination of the GBM in the left hemisphere ((a) (b)). (c) MRI pelvis (sagittal) demonstrates extensive colorectal adenocarcinoma recurrence. also negatively affect the survival rate in contrast to other [3] T. Mori, H. Nagase, A. Horii et al., “Germ-line and somatic mutations of the APC gene in patients with Turcot syndrome types of TS-associated CNS tumors. and analysis of APC mutations in brain tumors,” Genes Chromosomes and Cancer, vol. 9, no. 3, pp. 168–172, 1994. Conflict of Interests [4] M. Miyaki, J. Nishio, M. Konishi et al., “Drastic genetic insta- bility of tumors and normal tissues in Turcot syndrome,” The authors declare that they have no conflict of interests. Oncogene, vol. 15, no. 23, pp. 2877–2881, 1997. [5] F. Paraf, S. Jothy, and E. G. Van Meir, “Brain tumor-polyposis syndrome: two genetic diseases?” Journal of Clinical Oncology, References vol. 15, no. 7, pp. 2744–2758, 1997. [6] M. Agostini, M. G. Tibiletti, E. Lucci-Cordisco et al., “Two [1] J. Turcot, J. P. Despres, and F. St Pierre, “Malignant tumors of PMS2 mutations in a Turcot syndrome family with small the central nervous system associated with familial,” Diseases bowel cancers,” American Journal of Gastroenterology, vol. 100, of the Colon and Rectum, vol. 2, pp. 465–468, 1959. no. 8, pp. 1886–1891, 2005. [2] C.M.J.Tops, H. F. A. Vasen, G. vanBerge Henegouwen et [7] R. Castillo and M. M. G. Wilson, “Turcot syndrome in an al., “Genetic evidence that turcot syndrome is not allelic to elderly adult,” Journal of Clinical Gastroenterology, vol. 34, no. familial adenomatous polyposis,” American Journal of Medical 4, pp. 449–450, 2002. Genetics, vol. 43, no. 5, pp. 888–893, 1992. 6 Case Reports in Oncological Medicine [8] H.J.Chung,S.T.Oh, J. G. Kim, andW.K.Kang, “Turcot syndrome: a case report in an unsuspected setting,” Journal of Gastrointestinal Surgery, vol. 16, no. 2, pp. 411–414, 2012. [9] G. Eguchi, M. Shigemori, Y. Sugita, S. Kuramoto, and M. Uegaki, “A case of Turcot syndrome (glioma polyposis),” Neu- rological Surgery, vol. 21, no. 3, pp. 247–250, 1993. [10] R. Kleinerman, J. Marino, and E. Loucas, “Muir-Torre Syn- drome / Turcot Syndrome overlap? A patient with sebaceous carcinoma, colon cancer, and a malignant astrocytoma,” Der- matology Online Journal, vol. 18, no. 5, p. 3, 2012. [11] E. A. Lusis, S. Travers, S. C. Jost, and A. Perry, “Glioblastomas with giant cell and sarcomatous features in patients with turcot syndrome type 1: a clinicopathological study of 3 cases,” Neu- rosurgery, vol. 67, no. 3, pp. 811–817, 2010. [12] M. R. McLaughlin, S. M. Gollin, C. M. Lese, and A. L. Albright, “Medulloblastoma and glioblastoma multiforme in a patient with Turcot syndrome: a case report,” Surgical Neurology, vol. 49, no. 3, pp. 295–301, 1998. [13] D. R. Radin, K. C. Fortgang, C. S. Zee, V. G. Mikity, and J. M. Halls, “Turcot syndrome: a case with spinal cord and colonic neoplasms,” American Journal of Roentgenology, vol. 142, no. 3, pp. 475–476, 1984. [14] S. Schroder, D. Moehrs, J. von Weltzien, R. Winkler, and H. F. Otto, “The Turcot syndrome. Report of an additional case and review of the literature,” Diseases of the Colon and Rectum, vol. 26, no. 8, pp. 533–538, 1983. [15] H. Takayama, K. Nakagawa, S. Onozuka et al., “Nonfamilial Turcot syndrome presenting with astrocytoma—case report,” Neurologia Medico-Chirurgica, vol. 29, no. 7, pp. 606–609, [16] F. Paraf, “Turcot syndrome: evolution of knowledges,” Gas- troenterologie Clinique et Biologique, vol. 18, no. 3, pp. 297– 299, 1994. [17] T. Tamiya, S. Hamazaki, Y. Ono et al., “Ganglioglioma in a patient with Turcot syndrome,” Journal of Neurosurgery, vol. 92, no. 1, pp. 170–175, 2000. [18] S. R. Hamilton, B. Liu, R. E. Parsons et al., “The molecular basis of Turcot’s syndrome,” The New England Journal of Medicine, vol. 332, no. 13, pp. 839–847, 1995. [19] A. Merlo, C. Rochlitz, and R. Scott, “Survival of patients with Turcot’s syndrome and glioblastoma,” The New England Journal of Medicine, vol. 334, no. 11, pp. 736–737, 1996. [20] B. K. Kleinschmidt-DeMasters, L. Meltesen, L. McGavran, and K. O. Lillehei, “Characterization of glioblastomas in young adults,” Brain Pathology, vol. 16, no. 4, pp. 273–286, 2006. [21] Z. A. B. Jamjoom, S. Sadiq, A. B. Mofti, I. Al-Mofleh, and D. Ajarim, “Turcot syndrome: report of a case and review of the literature,” International Surgery, vol. 74, no. 1, pp. 45–50, [22] T. L. Chan, S. T. Yuen, L. P. Chung et al., “Germline hMSH2 and differential somatic mutations in patients with Turcot’s syndrome,” Genes Chromosomes Cancer, vol. 25, no. 2, pp. 75– 81, 1999. 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Turcot Syndrome: A Synchronous Clinical Presentation of Glioblastoma Multiforme and Adenocarcinoma of the Colon

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Copyright © 2012 Sabiq Dipro et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2012, Article ID 720273, 6 pages doi:10.1155/2012/720273 Case Report Turcot Syndrome: A Synchronous Clinical Presentation of Glioblastoma Multiforme and Adenocarcinoma of the Colon 1 2 2 2 1, 2 Sabiq Dipro, Faisal Al-Otaibi, Adel Alzahrani, Anwar Ulhaq, and Essam Al Shail Alfaisal University, Riyadh 11533, Saudi Arabia Division of Neurological Surgery, Department of Neurosciences, King Faisal Specialist Hospital & Research Center, P.O. Box 3354, Riyadh 11211, Saudi Arabia Correspondence should be addressed to Faisal Al-Otaibi, faisalruwais@gmail.com Received 30 August 2012; Accepted 23 September 2012 Academic Editors: E. Itakura and Z. Madjd Copyright © 2012 Sabiq Dipro et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Turcot syndrome (TS) is a rare hereditary disorder clinically characterized by the occurrence of primary tumors of the colon and the central nervous system (CNS). Here we present the case of an 11-year-old boy with a synchronous clinical presentation of both glioblastoma multiforme (GBM) and colonic adenocarcinoma. A molecular genetic study revealed microsatellite instability in the DNA mismatch repair (MMR) gene. This patient ultimately survived for 13 months after clinical presentation. Based on this case study, the synchronous presentation of glioblastoma multiforme and adenocarcinoma of the colon might suggest a shorter survival rate for patients with Turcot syndrome. A literature review complements this paper. 1. Introduction 2. Case Report Turcot syndrome (TS) was originally described by Turcot An 11-year-old boy presented with headache for the past 5 in 1959 [1–3]. It is characterized by the development of days, vomiting, and history of a single tonic clonic seizure. He primary tumors of the central nervous system (CNS), such had no history of weakness, loss of consciousness, or memory as glioblastoma multiforme (GBM) and medulloblastoma, disturbance. He was also suffering from vague abdominal along with numerous adenomatous colorectal polyps and pain and loose motion over a period of one month prior colonic adenocarcinoma. TS usually develops in the patient’s to presentation. He had positive family history of colonic teens and can be genetically distinguished as either familial cancer. His sister had multiple cafe-au-lait ´ spots and multiple adenomatous polyposis (FAP) or hereditary nonpolyposis colonic polyps. colon carcinoma (HNPCC) [4]. The syndrome was divided During clinical examination, he was fully awake and into two types by Paraf and colleagues in 1997 [5]. TS oriented with a Glasgow coma scale (GCS) score of 15. His type I is characterized by the presence of glial tumors, pupils were bilaterally equal and reacting; no motor or sen- relatively few colonic polyps, and cancer. TS type II is sory deficit was noticed. His gait and posture were normal. characterized by thousands of colonic polyps and increased On his skin there were multiple cafe-au-lait ´ spots and areas of risk of medulloblastoma. hypopigmentation (Figure 1). Brain CT showed that he had Since the time when this syndrome was first identified, a4 × 4 cm left frontal cystic tumor with perifocal edema, there have only been isolated case reports or case series effacement of left lateral ventricle, and subfalcine herniation. with a limited number of patients being discussed in the The brain appeared tight. The tumor had an intense ring- literature. The synchronous clinical presentation of CNS enhancing rim. He underwent left-frontal craniotomy and tumors and colonic adenocarcinoma in this syndrome is gross total resection of the tumor. Histopathology showed extremely rare. In this paper, we present a case of TS GBM with giant-cell features (WHO grade IV) (Figure 1). with simultaneous clinical presentation of GBM and colonic Immunohistochemistry of the tumor showed positive p53 adenocarcinoma. and Ki-67 >90%. 2 Case Reports in Oncological Medicine (a) (b) (c) (d) Figure 1: T1 axial MRI brain depicting left frontal tumor with rim enhancement associated with midline shift, (a) and after resection MRI brain demonstrates no residual tumor and a resolved mass effect (b). Photograph of the patient skin cafe-au-lait ´ spots (c). (d) Histopathology shows high mitotic index and multinucleated giant cells representing GBM features. Hematoxylin and eosin stain 5 400. He continued to have abdominal pain and diarrhea and Nine months later, he presented with headache and developed melena and intermittent frank bleeding from was found to have mild right hemiparesis with an overall Karnofsky performance score of over 90. CT and MRI of the rectum. His blood cell count, platelets, and renal and the brain showed a left temporal ring-enhancing lesion coagulation profiles were within the normal range. Upper about 4 × 5 cm in size with uncal herniation (Figure 3). He GI endoscopy was normal; however, colonoscopy showed also had another lesion at the site of the previous surgery. multiple colonic polyps of various sizes with ulceration He underwent redo left temporal craniotomy and gross and active bleeding (Figure 2). Punch biopsy of the polyps total resection of the tumor. The biopsy report revealed revealed grade 2 adenocarcinoma (Figure 2). A molecular pathological features similar to those of GBM. He was genetic workup of the patient showed mutation in the DNA started on temozolomide as palliative therapy. Three months mismatch repair (MMR) gene with microsatellite instability. later, he had multiple recurrences and dissemination of the He was diagnosed with Turcot syndrome with cerebral GBM cerebral tumors on the left temporal, multifocal frontal, and and adenocarcinoma of the colon. For adenocarcinoma of parietal areas. At the same time, he had a massive progression the colon, he underwent laparoscopic total colectomy and of colonic carcinoma (Figure 3). He died 13 months after the ileocolic anastomosis. A month later, he had recurrent bleed- initial diagnosis. ing from the rectum and developed new rectal polyps. To address this issue, he underwent proctectomy and terminal 3. Discussion ileostomy. The patient received adjuvant radiotherapy for the brain cancer and chemoradiotherapy for the colonic Turcot syndrome is also known as brain-tumor polyposis cancer. and is characterized by the occurrence of primary tumors of Case Reports in Oncological Medicine 3 (a) (b) (c) (d) Figure 2: Colon fiberscopy demonstrates ulcerative adenoma (arrow; (a), (b)). Histopathology slides reveal the adenoma, hematoxylin and eosin stain 5 100. (c) and carcinoma invasion of the colon muscular layer, hematoxylin and eosin stain 5 200 (d). the CNS and multiple colorectal adenomas and/or colonic indices were found to range between 18% and 45%, and adenocarcinoma [5, 11]. From a genetic standpoint, TS can all three GBMs demonstrated positive nuclear p53. The first be divided into two subtypes: the first is manifested by two patients survived 44 and 55 months, respectively, and the MMR gene and PMS2 with a high risk of developing the last patient survived 29 months and was still alive when GBM, and the second type is manifested by the adenomatous the report was published. For our patient, the Ki-67 was polyposis coli gene germline mutation with a high risk of >90% and the tumor showed p53 positivity in addition developing medulloblastoma [5, 16]. However, this classifi- to MMR gene mutation. The tumor did exhibit giant-cell cation cannot be applied to all cases reported in the literature. features. The patient survived only 13 months after initial There are reports of cases with the occurrence of both GBM clinical presentation. The average survival rate of patients and medulloblastoma, as in the report from McLaughlin with TS and GBM was found to be 27 months; however, and colleagues [12]. Nevertheless, their patient was treated when the investigators excluded the two patients with the for medulloblastoma and received whole-brain radiation longest-term survival, the average survival rate dropped to therapy 10 years prior to the diagnosis of GBM. Therefore, 16 months [18, 19]. In contrast, the survival rate of sporadic radiation-induced GBM cannot be excluded. Moreover, the GBM has been found to be around 15 months [20]. GBM occurrence of tumors other than GBM and medulloblastoma and anaplastic astrocytoma are the most commonly reported has been reported [17]. Based on TS classification, our CNS tumor associated with TS. In a review of 33 reported patient fit the clinical and molecular genetic features of TS cases, 21 reports were for GBM and anaplastic astrocytoma type I. [21]. The pathological differentiation between sporadic GBM Most of the reported TS cases in the literature are and TS-associated GBM has not been adequately addressed characterized by different time intervals between the clinical in the literature. Recently, Lusis and colleagues reported on presentation of CNS tumors and the colonic adenocarci- three cases involving patients with TS-associated GBM [11]. noma [18, 22]. The presence of symptomatic CNS GBM and The histopathological and molecular genetic features in all colorectal cancers at the same time carries more management three cases consisted of giant-cell GBM, and the patients difficulties and, more important, might shorten the survival in two of the cases had sarcomatous changes. The Ki-67 rate. Our patient presented with CNS and gastrointestinal 4 Case Reports in Oncological Medicine Table 1: Summary of selected reported cases with high-grade glioma and adenocarcinoma of the colon. Interval between the Survival rate after the No. of diagnosis of high-grade Authors (year) CNS tumors Other associated tumors diagnosis of high-grade cases glioma and colonic glioma adenocarcinoma (i) Adenocarcinoma of the ampullary region Agostini et al. Right frontal giant-cell 1 (ii) Adenomas of the 9months Not reported (2005) [6] GBM transverse and sigmoid colon Castillo and Multiple colonic polyps Wilson 1 Left frontoparietal GBM Same time as diagnosis Not reported with adenocarcinoma (2002) [7] (i) Ovarian Still alive at the age of 25 Chung et al. Right parietotemporal cystadenocarcinoma 1 12 years at the time of reporting (2012) [8] GBM (ii) Adenocarcinoma in the case sigmoid colon Recurring grade 3 Eguchi et al. Adenocarcinoma of the 1 astrocytoma in the right Same time as diagnosis One year (1993) [9] colon parietal lobe (i) Recurring colon cancer (ii) Carcinoma of the right Malignant astrocytoma Kleinerman et al. groin 1 (grade III) of the frontal 12 years One year (2012) [10] (iii) Nodular basal cell of lobe the right nose and the left cheek Two of the patients died after 44 and 55 months of diagnosis, and the Lusis et al. 3, 6, and 6 months, 3 GBM Colorectal adenocarcinoma third was still alive at the (2010) [11] respectively time of writing the article. Survived >29 months since diagnosis (i) Medulloblastoma McLaughlin et al. Adenocarcinoma of the 1 (ii) Right parietal GBM About 10 years A few months (1998) [12] colon Radin et al. GBM of the cervical Adenocarcinoma of the 1 4 months 6 months (1984) [13] spinal cord descending colon The patient died from brain herniation due to Schroder et al. GBM of the left frontal 1 Carcinoma of the jejunum 5 years GBM acute presentation. (1983) [14] lobe The diagnosis was made from autopsy Initial diagnosis was grade Takayama et al. I astrocytoma of left Adenocarcinoma of the 1 6months 18months (1989) [15] frontal lobe, and the ascending colon recurrence was GBM symptoms simultaneously. We elected to treat the CNS cases. Table 1 summarizes selected studies demonstrating the tumor first due to the presence of mass effects that were survival rates of TS after the time of high-grade glioma causing increased intracranial pressure. He was operated diagnosis. on for the GBM recurrence; however, a few months later he had a disseminated GBM involving almost the entire 4. Conclusion left cerebrum that was not amenable to further surgical intervention. This indicates highly malignant disease. The Simultaneous clinical presentation of CNS GBM and ade- presence of GBM in TS may suggest a lower survival nocarcinoma of the colon in TS might be an indicator of a rate in contrast to other CNS tumors in most reported shorter survival rate. Moreover, the presence of GBM may Case Reports in Oncological Medicine 5 Rt Lt Rt Lt (a) (b) (c) Figure 3: MRI brain shows the dissemination of the GBM in the left hemisphere ((a) (b)). (c) MRI pelvis (sagittal) demonstrates extensive colorectal adenocarcinoma recurrence. also negatively affect the survival rate in contrast to other [3] T. Mori, H. Nagase, A. Horii et al., “Germ-line and somatic mutations of the APC gene in patients with Turcot syndrome types of TS-associated CNS tumors. and analysis of APC mutations in brain tumors,” Genes Chromosomes and Cancer, vol. 9, no. 3, pp. 168–172, 1994. Conflict of Interests [4] M. Miyaki, J. Nishio, M. 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