Small intestinal neuroendocrine cell pathobiology: 'carcinoid' tumors

Neuroendocrine tumors, particularly small intestinal tumors, also grouped as 'carcinoids', are defined by an increasing incidence and prevalence, a poor response to current therapies, and confusion regarding appropriate models for drug development. Despite these issues, approximately 350 studies were published in the last year.

Two sources of confusion are clearly apparent. First, pharmacotherapeutic studies using pancreatic tumor cell lines as models for small intestinal or 'carcinoid' tumor biology are considered appropriate. Second, there is continued inclusion and analysis of pancreatic endocrine tumors with small intestinal neuroendocrine tumors in clinical studies. One highlight of this year is additional data confirming the significant differences between pancreatic tumor cell lines and small intestinal cell lines, the different gene expressions, for example, PAX8, between these two tumor types, and the observations that these two tumors respond differently in clinical trials, for example, to mammalian target of rapamycin (mTOR) inhibitors. Other highlights include delineating the role of the tumor microenvironment in the development of fibrosis and developing a minimum pathology dataset and a prognostic nomogram that may have utility in stratifying patients for clinical studies.

A number of interesting studies have been published during 2009-2010, but critical areas remain that require resolution. Current data, for the most part, reflect amplification of previously held concepts with modest advances in novel information.