Turcot's syndrome and its mode of inheritance.

“…Moreover, some cases of TS appear to be inherited by an autosomal recessive mechanism. This is supported by instances of parental consanguinity and the disease involving siblings but not their parents [1,16,17]. It is worth noting that with our patient, one other sibling appeared to have a similar disease, although dying at young age of a brain tumour, before developing any colonic symptoms.…”

Section: Discussionmentioning

confidence: 61%

An unusual case of Turcot’s syndrome associated with ileal adenocarcinoma, intestinal non-Hodgkin’s lymphoma, and duodenal adenocarcinoma. Review of the classification and genetic basis of Turcot’s syndrome

et al. 2005

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A 38-year-old man with a history of colonic and small bowel polyposis and glioblastoma was investigated for dyspepsia. Upper GI endoscopy identified an abnormal area in the duodenum, confirmed by histology as high grade non-Hodgkin's B cell MALT lymphoma. Although cases of Turcot's syndrome (TS) (colonic polyposis and primary brain tumour occurring in the same patient) have been previously described, association with haematological malignancy is rare. This is the first report of intestinal lymphoma occurring in an adult with TS.

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“…Five of their 36 patients had adenomatous colonic polyps, although none had ulcerative colitis or a strong family history of colorectal carcinoma. Colonic polyps were present in one of our 5 colorectal cases and another (familial) case, although lacking polyps, had some features resembling Turcot's syndrome [8,16,17] or a syndrome described by Pratt et al [9] of multiple colorectal carcinomas, polyposis coli and neurofibromatosis associated with lymphangioma or haemangioma and lymphoma in offspring of consanguinous marriages. A genetic cause was likely in another of our patients with colonic carcinoma whose father had both rectal carcinoma and carcinoma of the kidney.…”

Section: Discussionmentioning

confidence: 81%

Malignant epithelial tumours in children: Incidence and aetiology

Al-Sheyyab

Muir

Cameron³

et al. 1993

Med. Pediatr. Oncol.

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The purpose of this study was to establish the incidence of carcinomas in children, changes in incidence over a 30-year period, and to identify features of possible aetiological significance. A total of 173 cases were identified, but after review of the histopathology, 30 patients were excluded because they were considered to have benign epithelial tumours or malignant tumours of nonepithelial origin. Seven other cases were excluded because pathology material was not available. Overall, in 28% of cases, the diagnoses were changed by pathology review. Thus, 136 children in the West Midlands Region diagnosed 1957-1986 were included, with carcinoid tumours (44) and tumours of skin (22), nasopharynx (14), salivary gland (13), adrenal cortex (13), thyroid (9), large bowel (5), other (16). Excluding carcinoids, the age-standardised incidence rate was 2.4 x 10(6) per year. Male:female ratio was 0.7:1 and 66% were aged > 10 years. Incidence increased from 1.5 to 3.3 x 10(6) per year. Genetic factors predisposing to carcinoma included tyrosinosis, MEN II and III, congenital adrenal hyperplasia and basal cell naevus syndrome. There was a case of Li-Fraumeni syndrome and several other patients had relevant family histories. Probable "environmental" causes included antenatal exposure to stilboestrol or hydroxyprogesterone hexanoate, stilboestrol given for premature menarche, neonatal hepatitis and prior radiotherapy. The aetiology of carcinomas in children is multifactorial, both genetic and environmental factors being important. The incidence is increasing.

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Turcot's syndrome and its mode of inheritance.

Cited by 65 publications

References 13 publications

Turcot syndrome: a case with spinal cord and colonic neoplasms

Turcot syndrome: a case with spinal cord and colonic neoplasms

An unusual case of Turcot’s syndrome associated with ileal adenocarcinoma, intestinal non-Hodgkin’s lymphoma, and duodenal adenocarcinoma. Review of the classification and genetic basis of Turcot’s syndrome

Malignant epithelial tumours in children: Incidence and aetiology

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