Colorectal carcinoma at diagnosis of UC in a 17 year old

Abstract

IBD is a known risk factor for colorectal cancer (CRC). The degree of risk is related to the duration and extent of colitis, as well as the severity of inflammation over time. Other risk factors include PSC and a family history of sporadic CRC. ACG and AGA guidelines suggest surveillance based on duration of disease, not chronological age. Surveillance colonoscopies are recommended starting 8 years after onset of symptoms. We present a 17 year old female diagnosed with carcinoma in situ associated with UC at 1st colonoscopy for suspected IBD. She presented with non-bloody diarrhea of 3 years' duration. Physical exam and routine lab tests were unremarkable. A clinical diagnosis of IBS was suggested elsewhere and her symptoms improved with loperamide. Hematochezia triggered an investigation for IBD. The colonoscopy (6/2008) revealed pancolitis, with edematous and hyperemic mucosa, mucopurulent exudate and friability. No tumor was seen. Histology confirmed characteristic signs of chronic colitis with focal ulcerations, crypt branching and abscesses and dense acute and chronic inflammatory infiltrates. Biopsies (ascending, transverse, descending and rectum) showed signs of definite dysplasia, as well as the presence of P53 and k167 expression. In the descending, high grade dysplasia with carcinoma in situ was confirmed by two independent pathologists. A proctocolectomy with ileo-anal J pouch anastomosis was performed (8/2008). The pathology specimen confirmed a diagnosis of UC without skip lesions. Carcinoma in situ in the descending was found. Recurrent episodes of pouchitis have occurred despite probiotics, and were successfully treated with antibiotics. CRC in pediatric UC was first reported by Lagercrantz in 1949. Other cases were published over the ensuing 2 decades. In 1961 the Mayo Clinic reported that 10% of UC patients below 21 years of age died of CRC. Over the last 40 years improvements in the treatment and increased awareness of risk of CRC in IBD has impacted favorably on the incidence of CRC. Nevertheless, rare cases of CRC at or shortly after diagnosis of IBD exist. The first CRC associated with Crohn's disease in childhood was reported in 2001 in an adolescent with undiagnosed chronic abdominal pain for 5 years. Recently, an adolescent male with CRC after only 3 years of UC was reported in a Danish population-based study. Our case is exceptional in that CRC was found at the time of initial colonoscopy for suspected IBD. This emphasizes the fact that CRC can be present early after the onset of symptoms and may be delayed or missed when conducting surveillance strictly according to formal guidelines. It was recently suggested that surveillance with mucosal biopsies for dysplasia alone may be inadequate, as aneuploidy was found without dysplasia in patients as young as 16. Further research to optimize detection of pre-cancerous changes in high risk IBD patients is needed.