Clinical and Molecular Characteristics of Rare Malignant Tumors of Colon and Rectum

SIMPLE SUMMARY: Tumors of colon and rectum other than adenocarcinomas represent a neglected issue from clinical and laboratory points of view because of their rarity. In this review, we summarize and describe the rare histologic entities occurring in colon and rectum. Clinical and pathologic characteristics, prognostic behavior, treatments, and altered genes are reported to provide readers with a paramount and comparative perspective. In relation to this, we propose that improvements in registries and multidisciplinary research are warranted to ameliorate their management. ABSTRACT: The most frequent form of colorectal cancer is represented by adenocarcinoma being about 98% of tumor histological types. However, other rare histotypes can be found in colon and rectum (adenosquamous, goblet cell adenocarcinoma, lymphoma, medullary carcinoma, melanoma, mesenchymal, neuroendocrine, plasmacytoma, signet ring, squamous tumors). Altogether, these forms account for less than 2% of colorectal tumors. There are no specific diagnostic or therapeutic recommended approaches and most of the information available from literature derives from small and retrospective clinical series. In the present study, we provide a paramount and updated view on clinical and biologic characteristics of rare colorectal tumors.