Adenocarcinoma of the colon developing on the basis of Crohn's disease in childhood.
Colorectal carcinoma rarely affects children and has a dismal prognosis with 5-year survival rates as low as 2.5%-7% despite apparently radical surgery. Here we report the case of an adenocarcinoma of the sigmoid colon in a 15-year-old girl preceded by uncertain abdominal complaints of 5 years' duration. Pathological work-up revealed a tumour with lymph node metastases (pT3NI). Immunohistochemical evidence of p53 overexpression by the tumour cells raised the suspicion of an underlying Li-Fraumeni syndrome. In addition, there were aphthoid ulceration, fissuration of the non-tumorous mucosa, along with a mixed transmural infiltrate composed of macrophages, eosinophils, and non-typical giant cells, which were compatible with simultaneous Crohn's disease. Anamnestic data concerning the occurrence of idiopathic inflammatory bowel disease or colorectal carcinoma in the patient's relatives were non-contributory. The present results suggest a possible relationship between Crohn's disease and colon cancer due to the defective p53 gene product.
Tiszlavicz
L
L
Department of Pathology, Medical Faculty of University of Szeged, Hungary.
Kapin
M
M
Várkonyi
A
A
Lõrincz
A
A
Bartyik
K
K
Várkonyi
T
T
D000230:Adenocarcinoma / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D000293:Adolescent; D003424:Crohn Disease / Q000150:complications* / Q000235:genetics; D005260:Female; D016158:Genes, p53; D006801:Humans; D016864:Li-Fraumeni Syndrome; D012811:Sigmoid Neoplasms / Q000209:etiology* / Q000235:genetics / Q000473:pathology