Biomaterial Database

Turcot's syndrome. A review. 1988

L Jarvis, and N Bathurst, and D Mohan, and D Beckly

Department of Radiodiagnosis, Freedom Fields Hospital, Plymouth, Devon, United Kingdom.

Turcot's syndrome represents the association between familial multiple polyposis coli and neural tumors. The syndrome is reviewed with reference to genetics, colonic and central nervous system manifestations. This evidence suggests that Turcot's syndrome represents one manifestation of the pleiotropic autosomal dominant gene responsible for familial polyposis coli and the associated extracolonic manifestations of Gardner's syndrome. A diagnosis of Turcot's syndrome should be restricted to those patients with familial polyposis coli associated with gliomas or medulloblastomas. A further case is presented that is believed to be the first report of such a case in the United Kingdom.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008527	Medulloblastoma	A malignant neoplasm that may be classified either as a glioma or as a primitive neuroectodermal tumor of childhood (see NEUROECTODERMAL TUMOR, PRIMITIVE). The tumor occurs most frequently in the first decade of life with the most typical location being the cerebellar vermis. Histologic features include a high degree of cellularity, frequent mitotic figures, and a tendency for the cells to organize into sheets or form rosettes. Medulloblastoma have a high propensity to spread throughout the craniospinal intradural axis. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2060-1)	Arachnoidal Cerebellar Sarcoma, Circumscribed,Medulloblastoma, Desmoplastic,Medullomyoblastoma,Sarcoma, Cerebellar, Circumscribed Arachnoidal,Medulloblastoma, Adult,Medulloblastoma, Childhood,Melanocytic Medulloblastoma,Adult Medulloblastoma,Adult Medulloblastomas,Childhood Medulloblastoma,Childhood Medulloblastomas,Desmoplastic Medulloblastoma,Desmoplastic Medulloblastomas,Medulloblastoma, Melanocytic,Medulloblastomas,Medulloblastomas, Adult,Medulloblastomas, Childhood,Medulloblastomas, Desmoplastic,Medulloblastomas, Melanocytic,Medullomyoblastomas,Melanocytic Medulloblastomas
D009378	Neoplasms, Multiple Primary	Two or more abnormal growths of tissue occurring simultaneously and presumed to be of separate origin. The neoplasms may be histologically the same or different, and may be found in the same or different sites.	Neoplasms, Synchronous,Neoplasms, Synchronous Multiple Primary,Multiple Primary Neoplasms,Multiple Primary Neoplasms, Synchronous,Synchronous Multiple Primary Neoplasms,Synchronous Neoplasms,Multiple Primary Neoplasm,Neoplasm, Multiple Primary,Neoplasm, Synchronous,Primary Neoplasm, Multiple,Primary Neoplasms, Multiple,Synchronous Neoplasm
D011125	Adenomatous Polyposis Coli	A polyposis syndrome due to an autosomal dominant mutation of the APC genes (GENES, APC) on CHROMOSOME 5. The syndrome is characterized by the development of hundreds of ADENOMATOUS POLYPS in the COLON and RECTUM of affected individuals by early adulthood.	Polyposis Coli, Familial,Polyposis Syndrome, Familial,Adenomatous Polyposis Coli, Familial,Adenomatous Polyposis of the Colon,Familial Adenomatous Polyposis,Familial Adenomatous Polyposis Coli,Familial Adenomatous Polyposis of the Colon,Familial Intestinal Polyposis,Familial Multiple Polyposi,Familial Multiple Polyposis,Familial Multiple Polyposis Syndrome,Familial Polyposis Coli,Familial Polyposis Syndrome,Familial Polyposis of the Colon,Hereditary Polyposis Coli,Myh-Associated Polyposis,Polyposis Coli,Polyposis, Adenomatous Intestinal,Adenomatous Intestinal Polyposes,Adenomatous Intestinal Polyposis,Adenomatous Polyposes, Familial,Adenomatous Polyposis Colus,Adenomatous Polyposis, Familial,Coli, Adenomatous Polyposis,Coli, Familial Polyposis,Coli, Hereditary Polyposis,Coli, Polyposis,Colus, Adenomatous Polyposis,Colus, Familial Polyposis,Colus, Hereditary Polyposis,Colus, Polyposis,Familial Adenomatous Polyposes,Familial Intestinal Polyposes,Familial Multiple Polyposes,Familial Multiple Polyposus,Familial Polyposis Colus,Familial Polyposis Syndromes,Hereditary Polyposis Colus,Intestinal Polyposes, Familial,Intestinal Polyposis, Adenomatous,Intestinal Polyposis, Familial,Multiple Polyposes, Familial,Multiple Polyposi, Familial,Multiple Polyposis, Familial,Multiple Polyposus, Familial,Myh Associated Polyposis,Myh-Associated Polyposes,Polyposes, Familial Adenomatous,Polyposes, Familial Multiple,Polyposes, Myh-Associated,Polyposi, Familial Multiple,Polyposis Coli, Adenomatous,Polyposis Coli, Hereditary,Polyposis Colus,Polyposis Colus, Adenomatous,Polyposis Colus, Familial,Polyposis Colus, Hereditary,Polyposis, Familial Adenomatous,Polyposis, Familial Multiple,Polyposis, Myh-Associated,Polyposus, Familial Multiple
D002528	Cerebellar Neoplasms	Primary or metastatic neoplasms of the CEREBELLUM. Tumors in this location frequently present with ATAXIA or signs of INTRACRANIAL HYPERTENSION due to obstruction of the fourth ventricle. Common primary cerebellar tumors include fibrillary ASTROCYTOMA and cerebellar HEMANGIOBLASTOMA. The cerebellum is a relatively common site for tumor metastases from the lung, breast, and other distant organs. (From Okazaki & Scheithauer, Atlas of Neuropathology, 1988, p86 and p141)	Benign Cerebellar Neoplasms,Cerebellar Cancer,Malignant Cerebellar Neoplasms,Cerebellar Neoplasms, Benign,Cerebellar Neoplasms, Malignant,Cerebellar Neoplasms, Primary,Cerebellar Tumors,Neoplasms, Cerebellar,Neoplasms, Cerebellar, Benign,Neoplasms, Cerebellar, Malignant,Neoplasms, Cerebellar, Primary,Primary Neoplasms, Cerebellum,Benign Cerebellar Neoplasm,Cancer, Cerebellar,Cerebellar Cancers,Cerebellar Neoplasm,Cerebellar Neoplasm, Benign,Cerebellar Neoplasm, Malignant,Cerebellar Neoplasm, Primary,Cerebellar Tumor,Cerebellum Primary Neoplasm,Cerebellum Primary Neoplasms,Malignant Cerebellar Neoplasm,Neoplasm, Benign Cerebellar,Neoplasm, Cerebellar,Neoplasm, Cerebellum Primary,Neoplasm, Malignant Cerebellar,Primary Cerebellar Neoplasm,Primary Cerebellar Neoplasms,Primary Neoplasm, Cerebellum,Tumor, Cerebellar
D005799	Genes, Dominant	Genes that influence the PHENOTYPE both in the homozygous and the heterozygous state.	Conditions, Dominant Genetic,Dominant Genetic Conditions,Genetic Conditions, Dominant,Condition, Dominant Genetic,Dominant Gene,Dominant Genes,Dominant Genetic Condition,Gene, Dominant,Genetic Condition, Dominant
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D013577	Syndrome	A characteristic symptom complex.	Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes