Activation of Hedgehog Pathway in Neuroendocrine Tumor

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Introduction: Neuroendocrine tumors (NETs) are rare tumors and often follow an indolent course, especially the tumors of low-to-intermediate grade. The genetic etiology of sporadic NET is largely unknown. It is well-known that a significant number of patients with NETs may have harbored the slow-growing lesions since very a young age, suggesting it could result from alterations of pathways involved in development. Hedgehog (Hh) signaling has been found to be important in embryonic patterning, stem cell maintenance and tissue repair; and is normally silenced in most mature adult tissue. However, it frequently undergoes gain-of-function mutations or reactivation due to ligand overproduction and has been linked to the etiology of a variety of tumors.

Aim(s): In this study, the role of Hh pathway in neuroendocrine tumorigenesis was examined.

Materials and methods: The expression status of Hh pathway in NETs was determined by immunohistochemistry; and its role in tumorigenesis was studied using a specific inhibitor in a xenograft mouse model.

Conference: 9th Annual ENETSConcerence (2012)

Presenting Author:

Authors: Dong M, Sangai T, Rashid A, Meric-Bernstam F, Yao J,

Keywords: neuroendocrine tumor, Hedgehog pathway, therapeutic target,