Primary linitis plastica of the entire colon in a background of ulcerative colitis: a case report
© Feinberg et al.; licensee BioMed Central. 2015
Received: 21 August 2014
Accepted: 23 December 2014
Published: 4 February 2015
We present a case report of colorectal cancer arising in a young patient with ulcerative colitis of only 6 years duration. The pathology was unusual with extensive pancolonic involvement in a lintitis plastica fashion. This case represents a clinical example where colon cancer occurred prior to the onset of recommended screening according to guidelines regarding patients with ulcerative colitis.
Colorectal cancer is a serious outcome associated with ulcerative colitis. However, this risk is correlated with the duration of inflammation. Current data estimate that the risk of colorectal cancer in ulcerative colitis is 2% at 10 years, 8% at 20 years, and 18% at 30 years . Signet ring cell carcinoma is an uncommon form of cancer in the colon and rectum, representing 1% of cancers in one series . We present a case of poorly differentiated colonic adenocarcinoma with focal signet ring cell features involving virtually the entire colon in a linitis plastica fashion, which may be only the second such case recorded in the literature . Additionally, our patient was a 20-year-old female with only a 6-year history of ulcerative colitis.
The patient completed twelve cycles of adjuvant FOLFOX over 6 months. She is currently disease free.
Colonic linitis plastica is extremely rare, estimated at less than 0.1% of colon cancers . Secondary linitis plastica of the colon is more common and the treating team should consider investigating for a primary tumor.
It is difficult to determine when to initiate screening colonoscopy for colorectal cancer detection in patients with ulcerative colitis. Prior reports of low incidence of malignancy among patients without longstanding colitis  lend to establishing guidelines that use duration of disease as a criterion. This is problematic since there is a distinction between the initiation of symptoms and the official diagnosis. Nonetheless, major American and international associations have published guidelines that recommend screening for dysplasia should begin after 8 years of disease activity in ulcerative colitis [5-7]. There are specific stipulations for patients with pancolitis or primary sclerosing cholangitis, but none of the guidelines recommend initiating screening prior to the 8-year mark. This case challenges those guidelines, as the patient presented with extensive colonic neoplasia after just 6 years of disease activity.
Despite a low overall risk of colorectal cancer in patients with ulcerative colitis of relatively short duration, there is evidence that malignancies may be missed in that initial window. A retrospective Dutch nationwide population-based analysis found that approximately 20% of colorectal cancer cases in patients with ulcerative colitis presented prior to the recommended initiation of screening colonoscopy . Thus, it may not be adequate to use duration of disease as an isolated clinical marker for screening guidelines. Other factors such as severity of disease  and presence of pseudopolyps  contribute to increase the risk of developing colorectal cancer. It is also possible that the immunosuppressive effect of long term anti-tumor necrosis factor therapy may have been a predisposing factor. We propose initiating screening for colorectal cancer in ulcerative colitis earlier in patients that have these worrisome features. In those without reasonable symptom control, colonoscopy should be performed regularly. Furthermore, altering the medical treatment or surgical intervention should be considered. In our patient, optimal care would have entailed subtotal colectomy prior to diagnosing malignancy simply on the basis of refractory disease with severe malnutrition.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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