Diagnosis and Management of Rectal Neuroendocrine Tumors (NETs)

Abstract

Rectal neuroendocrine tumors (NETs) are rare, with an incidence of 0.17%, but they represent 12% to 27% of all NETs and 20% of gastrointestinal NETs. Although rectal NETs are uncommon tumors, their incidence has increased over the past few years, and this is probably due to the improvement in detection rates made by advanced endoscopic procedures. The biological behavior of rectal NETs may be different: factors predicting the risk of metastases have been identified, such as size and grade of differentiation. The tendency for metastatic diffusion generally depends on the tumor size, muscular and lymphovascular infiltration, and histopathological differentiation. According to the current European Neuroendocrine Tumor Society (ENETS) guidelines, tumors that are smaller than 10 mm and well differentiated are thought to have a low risk of lymphovascular invasion, and they should be completely removed endoscopically. Rectal NETs larger than 20 mm have a higher risk of involvement of muscularis propria and high metastatic risk and are candidates for surgical resection. There is controversy over rectal NETs of intermediate size, 10-19 mm, where the metastatic risk is considered to be 10-15%: assessment of tumors endoscopically and by endoanal ultrasound should guide treatment in these cases towards endoscopic, transanal, or surgical resection.

Keywords: endoscopic mucosal resection; endoscopic submucosal dissection; endoscopy; rectal neuroendocrine tumor; surgery; transanal endoscopic microsurgery.

Scheme 1

Proposed algorithm of diagnostic assessment of Rectal NETs.

Scheme 2

Proposed algorithm of treatment of Rectal NETs based on tumor size, grading and presence or not of metastasis.