Signet ring cell carcinoma of the colon in a 10 year-old boy

Irene Irene; Ketut Ariawati

doi:10.14238/pi51.2.2011.116-22

Irene Irene Department of Child Health, Udayana University Medical School/Sanglah Hospital, Denpasar, Bali
Ketut Ariawati Department of Child Health, Udayana University Medical School/Sanglah Hospital, Denpasar, Bali

DOI: https://doi.org/10.14238/pi51.2.2011.116-22

Keywords: signet ring cell carcinoma, SRCC, colon, familial polyposis, ulcerative colitis

Abstract

Signet ring cell carcinoma (SRCC), a variantÂ of adenocarcinoma, is defined by the presenceÂ of more than 50% of tumor cells withÂ prominent intracytoplasmic mucin.1 PrimaryÂ gastrointestinal malignancies constitute only 1% ofÂ pediatric neoplasms and 0.34% take the form of colonÂ carcinoma. It is a very rare disease in persons underÂ 17 years of age, 94% of which is found in children>Â 9 years of age. [.4 In contrast to adults, approximately
50% of colon carcinoma cases in children are theÂ highly malignant mucin'producing adenocarcinomaÂ type.4 The typical signet-ring cell has a large mucinÂ vacuole that fills the cytoplasm and pushes the nucleiÂ to the side, thereby giving the cells their signatureÂ histologic appearance.S,6 Carcinoma of the colonÂ during childhood has been associated with familial
polyposis and ulcerative colitis. However, carcinomaÂ arising de novo is the most common type.2,3 RiskÂ factors include a high caloric diet rich in animal fat,Â sedentary lifestyle, smoking, alcohol consumption, lowÂ vegetable fibre consumption, chronic inflammatoryÂ bowel disease, ulcerative colitis, Crohn's disease,Â and polymorphism in key enzymes of injuriousÂ compounds.s

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