Mixed adeno-neuroendocrine carcinoma (MANEC) is a rare pathological diagnosis recently acknowledged by the World Health Organization in 2010. MANEC is a neoplasm characterized by significant histological heterogeneity and is characterized by the simultaneous presence of both adenocarcinomatous and neuroendocrine differentiation; their definition includes each component found in at least 30% of the tumor. Colorectal MANEC constitutes an uncommon type of malignant tumor. The true prevalence of colorectal MANEC has not been precisely defined and published studies are limited to case reports and small case series. The aim of the present review was to accumulate the existing evidence on colorectal MANEC with special attention to the clinicopathological characteristics, management and survival rates of patients diagnosed with this malignancy. A total of 20 studies (16 case reports and 4 retrospective cohorts) reported outcomes for patients with colorectal MANEC and were finally considered eligible for analysis. The results of the present study show that patients with early stage MANEC have more favorable survival compared to those diagnosed in advanced stages. Due to its neuroendocrine nature, which is characterized by rapid progression, MANEC is diagnosed in advanced stages in the majority of cases and thus potentially explains the poor survival rates. Because of its aggressive nature and high recurrence rate, adjuvant chemotherapy constitutes a critical part of the treatment and significantly improves survival. Further larger studies are needed in order to establish guidelines for the treatment of these rare lesions.
Keywords: chemotherapy; colorectal; mixed adeno-neuroendocrine carcinoma.