Question A 58-year-old, non-smoker male patient with no family history was followed up since 2002 for ulcerative colitis (UC) located in the rectosigmoid. He was initially treated by salicylates alone until November 2008. Mercaptopurine was started in August 2009 for steroid dependence with primary failure despite adequate 6 thioguanine nucleotides (6 TGN) level. He was included in February 2010 in the PURSUIT trial testing Golimumab with only partial response. A combination therapy with Mercaptopurine and Infliximab was then started in December 2010 with a long-term clinical, biological and endoscopic steroid-free remission. A pan-chromoendoscopy performed in June 2016 showed a complete mucosal healing with no histological activity or dysplasia on multiple random biopsies. Regular monitoring of Infliximab trough levels and 6-TGN were appropriate. A rectosigmoidoscopy was carried in September 2017 for a relapse (figure 1A), scored UCEIS 3. Rectal biopsies showed a polymorphic inflammatory infiltrate with no dysplasia. Bacterial cultures were negative. A new Infliximab infusion was administered without clinical improvement. He was hospitalised in October 2017 for poor general state, fever and persistent bloody diarrhoea. A CT scan of the chest, abdomen and pelvis found multiple mesenteric and peri-aortic lymphadenopathies, peritoneal infiltration with bilateral pyelocaliceal dilatation and major rectal thickening (figure 1B). The rectosigmoidoscopy revealed a stenosis from 5 to 20 cm from the anal verge passable by the fiberscope only (figure 1C). Biopsies were performed (figure 1D).
Question What is the diagnosis?
- ulcerative colitis
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Biopsies of the stenosis revealed an adenocarcinoma with signet-ring cells leading to the diagnosis of metastatic rectal linitis plastica. Molecular analysis revealed a KRAS codon 12 gene mutation with no microsatellite instability. He underwent emergency double J catheter stenting for malignant extrinsic ureteral obstruction. Chemotherapy by folinic acid, fluorouracil and oxaliplatin was initiated. Unfortunately, febrile neutropenia occurred at day 8 of cycle 1 with septic shock leading to patient death.
Colorectal linitis plastica is usually found in the rectosigmoid and represents less than 1% of rectal adenocarcinomas.1 It is usually characterised by diffuse rectal thickening infiltrating all the layers with the presence of signet-ring cells at pathological examination. At equivalent tumour staging, these lesions are of poorer prognosis than sporadic ones. Metastatic spreading seems more frequent with preferential lymph nodes and peritoneal involvement. Chemoresistance has been suggested compared with non-linitic adenocarcinoma.2 Few cases of rectal linitis in patients with UC have been reported.3 Our case is unusual by the rapid onset of the tumour in a patient beneficiating of a regular endoscopic surveillance. Moreover, a rectosigmoidoscopy with multiples rectal biopsies did not show any macroscopic or microscopic suspect lesions 3 weeks before. We cannot exclude that combination therapy may have contributed to the rapidly fatal issue of this rare tumour.
Contributors NH: writing and data collection J-MG: writing and manuscript coordination. CG: pathological examination and histological pictures providing. M-LT-M and MA: copy editing of the manuscript.
Competing interests None declared.
Patient consent Next of kin consent obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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