Mixed neuroendocrine and adenocarcinoma of gastrointestinal tract: A complex diagnosis and therapeutic challenge

Abstract

In this editorial we comment on the manuscript describing a case of adenocarcinoma mixed with a neuroendocrine carcinoma of the gastroesophageal junction. Mixed neuroendocrine and non-neuroendocrine neoplasms of the gastrointestinal system are rare heterogeneous group of tumors characterized by a high malignant potential, rapid growth, and poor prognosis. Due to the rarity of these cancers, the standard therapy is poorly defined. The diagnosis of these tumors is based on combination of morphological features, immunohistochemical and neuroendocrine and epithelial cell markers. Both endocrine and epithelial cell components can act independently of each other and thus, careful grading of each component separately is required. These cancers are aggressive in nature and the potential of each component has paramount importance in the choice of treatment and response. Regardless of the organ of origin, these tumors portend poor prognosis with increased proportion of neuroendocrine component. Multidisciplinary services and strategies are required for the management of these mixed malignancies to provide the best oncological outcomes. The etiopathogenesis of these mixed tumors remains obscure but poses interesting question. We briefly discuss a few salient points in this editorial.

Keywords: Mixed adenocarcinoma and neuroendocrine carcinoma, Mixed neuroendocrine and non-neuroendocrine neoplasm, Mixed adeno-neuroendocrine cancer, Cell reprograming, Tumor plasticity

Core Tip: Mixed neuroendocrine and non-neuroendocrine neoplasms and mixed adeno-neuroendocrine cancer of the gastrointestinal system are a rare heterogeneous group of tumors with high malignant potential, rapid growth, and poor prognosis. The occurrence of cells with both neuroendocrine and non-neuroendocrine morphological features co-exist in varying amounts, and these tumors occur in almost all organs. The origin of these mixed cancers is not clearly defined but may be the result of certain genetic alterations and growth stimulation by the neuroendocrine peptides produced by neuroendocrine component. Regardless of the organ of origin, these tumors portend poor prognosis with increased proportion of neuroendocrine component. Multidisciplinary services and strategies are required for the management of these mixed malignancies to provide the best oncological outcomes.