About GEP-NETs

Frequency

NETs Are Being Diagnosed With Increasing Frequency

Neuroendocrine tumors (NETs) arise from neuroendocrine cells, which are specialized cells that secrete hormones and other substances and are found throughout the body. These cancers may or may not secrete substances at levels high enough to cause symptoms.^1-3

Although classified as a rare disease, NETs are being diagnosed with increasing frequency.^4,5

The reported incidence of NETs has increased:

6.98/100,000

New cases/year as of 2012⁴

~171,000

Projected people living with the disease in the United States as of 2014⁴

~6.4-fold increase

Over the last few decades
(from 1973 to 2012)⁴

Chart showing number of patients diagnosed by primary site in each time period.

Diagnosis at earlier stages of the disease is thought to be responsible for this increase, even though NETs are still often diagnosed at an advanced or metastatic stage.^7,8

NETs are generally considered as indolent (slowly growing) tumors; however, the prognosis varies widely, according to⁴:

Primary tumor site
Tumor differentiation and grade
Stage

From 1973 through 2012, the annual age-adjusted incidence of NETs has increased compared with that of other malignant neoplasms.⁴

Classification

Although NETs can arise from a wide variety of organs and tissues, most NETs are gastroenteropancreatic NETs (GEP-NETs), originating in the gastrointestinal tract and pancreas.⁴

GEP-NETs have traditionally been divided into¹⁰:

Foregut tumors: Esophagus, stomach, proximal duodenum, liver, and pancreas
Midgut tumors: Distal duodenum, ileum, jejunum, ascending colon, and proximal two-thirds of transverse colon
Hindgut tumors: Distal one-third of transverse colon, descending colon, sigmoid colon, and rectum

Foregut: Stomach 8.9%, Pancreas 12.1%. Midgut: Small Intestine 30.8%. Hindgut: Colon 17.6%, Appendix 5.7%, Rectum 26.3%

Delay in Diagnosis

Many patients with GEP-NETs are diagnosed with metastatic disease, which may result in increased disease burden and worse survival outcomes.^4,8,11

Approximately 60% of patients with GEP-NETs present with regional spread or distant metastases at time of diagnosis

of patients with GEP-NETs present with regional spread or distant metastases at time of diagnosis^8,a

^aBased on 837 assessable patients from the National Cancer Registry for Gastroenteropancreatic Neuroendocrine Tumors (RGETNE) in Spain. In this cohort, 14% of patients had regional spread and 44% had distant metastases.⁸

Mortality

Advanced GEP-NETs May Be Fatal, Even for Patients With Low-Grade Disease⁴

Chart showing survival rates among distant stage grade ½ NETs diagnosed from 2000 to 2012.

Most patients are diagnosed with locally advanced or metastatic disease due to the slow-growing nature of the tumor, lack of symptoms, or nonspecific symptoms^11,12
GEP-NETs may be fatal in patients with advanced, nonresectable disease^4,7

GEP-NETs, gastroenteropancreatic neuroendocrine tumors.

Next: Disease Progression

References: 1. National Center for Advancing Translational Sciences. Neuroendocrine tumor. Accessed April 8, 2021. https://rarediseases.info.nih.gov/diseases/13445/neuroendocrine-tumor 2. National Cancer Institute. Neuroendocrine tumor. Accessed April 8, 2021. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/neuroendocrine-tumor 3. American Society of Clinical Oncology. Neuroendocrine Tumors: Introduction. Accessed June 23, 2021. https://www.cancer.net/cancer-types/neuroendocrine-tumors/introduction 4. Dasari A, Shen C, Halperin D, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017;3(10):1335-1342. 5. US Food and Drug Administration. Rare Diseases at FDA. Accessed June 23, 2021. https://www.fda.gov/patients/rare-diseases-fda 6. Man D, Wu J, Shen Z, Zhu X. Prognosis of patients with neuroendocrine tumor: a SEER database analysis. Cancer Manag Res. 2018;10:5629-5638. 7. Sackstein PE, O’Neil DS, Neuget AI, Chabot J, Fojo T. Epidemiologic trends in neuroendocrine tumors: an examination of incidence rates and survival of specific patient subgroups over the past 20 years. Semin Oncol. 2018;45(4):249-258. 8. Garcia-Carbonero R, Capdevila J, Crespo-Herrero G, et al. Incidence, patterns of care and prognostic factors for outcome of gastroenteropancreatic neuroendocrine tumors (GEP-NETs): results from the National Cancer Registry of Spain (RGETNE). Ann Oncol. 2010;21(9):1794-1803. 9. Lutathera. Prescribing information. Novartis Pharmaceuticals Corp. 10. Campana D, Tomassetti P. Incidence, epidemiology, aetiology and staging, classification, clinical presentation/signs and symptoms, diagnosis, staging procedures/investigation. In: PET/CT in Neuroendocrine Tumors, Clinicians’ Guides to Radionuclide Hybrid Imaging. 1st ed. Springer International; 2016:1-5. 11. Singh S, Granberg D, Wolin E, et al. Patient-reported burden of a neuroendocrine tumor (NET) diagnosis: results from the first global survey of patients with NETs. J Glob Oncol. 2017;3(1):43-53. 12. Vinik AI, Woltering EA, Warner RRP, et al. NANETS consensus guidelines for the diagnosis of neuroendocrine tumor. Pancreas. 2010;39(6):713-734.

About GEP-NETs

NETs Are Being Diagnosed With Increasing Frequency

Advanced GEP-NETs May Be Fatal, Even for Patients With Low-Grade Disease⁴

Important Safety Information

Indication

About GEP-NETs

NETs Are Being Diagnosed With Increasing Frequency

Advanced GEP-NETs May Be Fatal, Even for Patients With Low-Grade Disease4

Advanced GEP-NETs May Be Fatal, Even for Patients With Low-Grade Disease⁴