Primary signet ring cell carcinoma of colon and rectum often presents as long segment stenosis in young patients

Abstract

Background: Primary signet ring cell carcinoma of the colon and rectum (PSRCCR) is rare, usually diagnosed at advanced stage with poor outcomes. We aimed to find possible diagnostic clues in order to help diagnosis. Methods: A retrospective study of PSRCCR patients from 1993 to 2018 was reviewed at a single tertiary center. Colorectal adenocarcinoma patients as control group with 1:4 ratio was also enrolled. Results: 18 patients with PSRCCR were identified. The prevalence rate was 0.16% (18 of 11,515). The mean age was 50.2 years-old in PSRCCR group and 63 years-old in non-SRCC colorectal cancer patients ( p < 0.001). Diagnosis tool depends on CFS were much less in PSRCCR group than control group (44.4 % vs 93 %, p < 0.001). SRCC patients had higher level of CEA (68.3 vs 17.7 ng/mL, p =0.004) and lower level of Albumin (3.4 vs 4.3 g/dL, p < 0.001). The majority of PSRCCR tumor configuration was ulcerative and infiltrative. More PSRCCR pathology presented as high-grade carcinoma (66.7 vs 1.4 %, p < 0.001) and lymphovascular invasion (77.8 vs 44.4 %, p = 0.011) than control group. More PSRCCR patients were diagnosed at advanced stage (88.8 vs 40.3 %, p = 0.001). Higher mortality was also noticed in PSRCCR group than control group (72.2 vs 20.8 %, p < 0.001). Conclusion: For young patients with long segment colonic stenosis and ulcerative/ infiltrative mucosa but endoscopic biopsy failed to identify malignant cells, earlier operation or non-colon site biopsy is suggested for diagnosing the PSRCCR.