NCI Definition: A malignant neuroendocrine neoplasm composed of cells containing secretory granules that stain positive for NSE and chromogranin. The neoplastic cells are often round and form clusters or trabecular sheets. Representative examples are small cell carcinoma, large cell neuroendocrine carcinoma, and Merkel cell carcinoma. [1]

Neuroendocrine carcinomas most frequently harbor alterations in TP53, RB1, KMT2D, RET, and APC [2].

Most Commonly Altered Genes in Neuroendocrine Carcinoma

TP53 Mutation, TP53 Missense, TP53 c.217-c.1178 Missense, RB1 Mutation, and TP53 Exon 5 Mutation are the most common alterations in neuroendocrine carcinoma [2].

Top Alterations in Neuroendocrine Carcinoma

Significant Genes in Neuroendocrine Carcinoma






Disease Details

Poorly Differentiated Neuroendocrine Carcinoma, Neuroendocrine Cell Carcinoma, NEC, Neuroendocrine cancer, NOS, Metastatic neuroendocrine carcinoma, CARCINOMA, NEUROENDOCRINE CELL, MALIGNANT, Low-grade neuroendocrine carcinoma, Poorly Differentiated Carcinoma with Neuroendocrine Features, Metastatic high-grade poorly differentiated neuroendocrine carcinoma
Neuroendocrine Neoplasm
Thymic Neuroendocrine Carcinoma, Esophageal Neuroendocrine Carcinoma, Breast Neuroendocrine Carcinoma, Prostate Neuroendocrine Carcinoma, Endometrial Neuroendocrine Carcinoma, Vaginal Neuroendocrine Carcinoma, Thyroid Gland Medullary Carcinoma, Small Cell Carcinoma, Extrahepatic Bile Duct Neuroendocrine Carcinoma, Merkel Cell Carcinoma, Neuroendocrine Carcinoma of Unknown Primary, Large Cell Neuroendocrine Carcinoma, Neuroendocrine Carcinoma, NOS, Colorectal Neuroendocrine Carcinoma, Lung Neuroendocrine Carcinoma, Pancreatic Neuroendocrine Carcinoma, Anal Canal Neuroendocrine Carcinoma, Gastric Neuroendocrine Carcinoma, Cervical Neuroendocrine Carcinoma, Ovarian Neuroendocrine Carcinoma, and Head and Neck Neuroendocrine Carcinoma


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.