oncology and supportive care
Neuroendocrine tumors
Neuroendocrine tumors – a life-limiting disease
Neuroendocrine tumors (NET) are slow-growing cancerous tumors originating from cells in the endocrine and nervous systems. The tumors can occur throughout the body, including the abdomen and lungs. It is a relatively rare and life-limiting disease that is often diagnosed late in disease progression.
Disease overview
Rising cases and varied symptoms – neuroendocrine tumors
The neuroendocrine tumors can occur throughout the body, including in the abdomen and lungs. Gastroenteropancreatic neuroendocrine tumors, GEP-NET, is the most common subgroup of NETs where the tumors are located in the gastrointestinal tract or pancreas.1
Steadily rising prevalence of neuroendocrine tumors
The incidence and prevalence of neuroendocrine tumors are steadily increasing in both North America, Asia and Europe, with the highest increase recorded in North America. There are today an estimated 350,000 patients with GEP-NET in the EU and US.1,2 Better utilization of healthcare resources, with more efficient and earlier diagnosis, is likely to be a contributing factor to the increase in disease incidence. In parallel with access to better treatment options, survival for patients with GEP-NET has improved over time.2-4
Heterogeneous disease and display of neuroendocrine tumor symptoms
NET can be present with or without symptoms. The most common syndrome of NET, carcinoid syndrome, is predominantly characterized by flushing, diarrhea, and asthma-like symptoms.3 Symptoms of NET can be very diverse, and depend on the tumor location, size and hormone produced by the affected cells. Often, a person has no symptoms until the tumor spreads, making NET hard to diagnose due to the slow growth rate of the tumor. Patients with GEP-NET are on average 63 years old at diagnosis, and the disease is equally common in women and men.5
Principles of neuroendocrine tumors treatment
The primary strategy in GEP-NET is surgical removal of the tumor. However, this is often not possible due to the location of the tumors and may not be curative, as metastases are commonly observed before or shortly after diagnosis.6 In such case, standard medical treatment is somatostatin receptor ligands (SRL), such as octreotide and lanreotide. Treatment with SRL aims to prevent tumor growth and further spread of the tumor, as well as to alleviate symptoms of an uncontrolled hormone production.7
References
- Das, S., et al. Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?. Current Oncology Reports. Mar 14; 23(4): 43, 2021.
- Sandvik O., et al. Epidemiology and classification of gastroenteropancreatic neuroendocrine neoplasms using current coding criteria. Br J Surg. 2016;103(3):226-32.
- Alwan H., et al. Incidence trends of lung and gastroenteropancreatic neuroendocrine neoplasms in Switzerland. Cancer Med. 2020;9(24):9454-61.
- Dasari, A., et al. Trends in the Incidence, Prevalence, and Survival Outcomes in Patients With Neuroendocrine Tumors in the United States. JAMA Oncology. 3(10):1335-1342, 2017.
- Sackstein PE, et al. Epidemiologic trends in neuroendocrine tumors: An examination of incidence rates and survival of specific patient subgroups over the past 20 years. Seminars in Oncology 45, 249-58, 2018.
- Globe Life Sciences rapport 2022, data on file.
- Díez M., et al. Gastroenteropancreatic neuroendocrine tumors: diagnosis and treatment. Ann Gastroenterol. 2013; 26(1): 29-36.
- INCA International Neuroendocrine Cancer Alliance. https://incalliance.org/ncd-2022/campaign-messages/
350,000
patientsin the EU and US are estimated to have GEP-NET2,3
55%
of patients with GEP-NET are initially misdiagnosed with for example irritable bowel syndrome (IBS), gastritis or anxiety5
Symptoms
- Redness of the skin (flushing)
- Diarrhea, stomach pain
- Asthma-like symptoms
- Carcinoid heart disease
Diagnosis
Diagnosis of NET is based on clinical symptoms, imaging studies and biochemical tests.
Management
Several factors help define the appropriate treatment of NET, including tumor location, invasiveness, hormone secretion, and proliferation. Surgery is often performed either as a cure or for symptom relief. In secretory tumors, somatostatin receptor ligands (SRL) are used to relieve symptoms by blocking the release of hormones.
