Neuroendocrine tumours are malignant growths which originate from the neuroendocrine cells. The tumour may grow slowly or aggressively and spread to other parts of the body. They most commonly occur in the gastrointestinal tract, lung and pancreas, but may also develop in many other organs, including the breast, prostate, thymus and skin. Generally, no visible symptom of the disease can be seen and the tumour is detected incidentally. When symptoms arise, they can vary based on the location of the tumour. In some rare cases of the disease, fluctuating blood sugar levels or skin flushing may happen. Serotonin, chromogranin and several other proteins and hormones can serve as biomarkers for NETs owing to capability of neuroendocrine cells to produce them.
Neuroendocrine tumours can either be suspected through a clinical symptom or can be identified as an incident finding. NETs are termed as ‘functioning’ if they symptoms can be visible in clinical diagnosis as a release of hormones. While, NETs are termed as ‘nonfunctioning’ if they do not produce a biologically active hormone.
Neuroendocrine tumours diagnosis is a multidisciplinary effort wherein medical oncologists, surgeons, interventional radiologists and pathologists are involved. The diagnostic picture is combined result of pathology testing, hormonal testing, and diagnostic and functional imaging. Biochemical testing required for diagnosis can either be syndrome specific or non-specific depending upon the type of NET.
Neuroendocrine Tumors: Epidemiology Insights
Report Ocean will focus on epidemiology segmentation at global level depending on the following characteristics:
- Total Prevalent Population of Neuroendocrine Tumors
- Total Diagnosed Prevalent Population of Neuroendocrine Tumors
- Site-specific Diagnosed Prevalent Population of Neuroendocrine Tumors
- Age-specific Diagnosed Prevalent Population of Neuroendocrine Tumors
- Gender-specific Diagnosed Prevalent Population of Neuroendocrine Tumors
- Stage-wise Diagnosed Prevalent Population of Neuroendocrine Tumors
- Grade-wise Diagnosed Prevalent Population of Neuroendocrine Tumors
Neuroendocrine tumours holds for only 0.5% of all malignancies. It is a quite rare disorder with an incidence of approximately 2 in per 100,000 cases. Neuroendocrine tumors (NETs) have a low incidence but relatively high prevalence. According to the reports of Kentucky Cancer Registry (KCR) and Surveillance, Epidemiology, and End Results Program (SEER), between 1995-2012, the incidence of NETs increased from 3.1 to 7.1 per 100,000 cases and from 3.96 to 6.61 per 100,000 cases, respectively.
The cases of neuroendocrine tumours are site specific and it was found in literature studies that it occurs mostly in the gastrointestinal tract with 62-67% incidence and the lung with 22-27% incidence. The disease is predominant in females with few exceptions. This female preponderance may be due to the appendiceal location. As per age, in approximately 40% cases, patients aged 50-64 years had the highest prevalence of this disorder.
Neuroendocrine tumours can either be completely resectable and are considered as early stage or they can either locally advanced and unresectable or metastatic which are known to have reached advanced stage. NETs are usually diagnosed at an early stage, according to a study out of total diagnosed cases more than 42% were diagnosed at early stage. This staging has a profound impact on the treatment choice as early stage can be cured with surgical resection alone.
Neuroendocrine tumours can also be represented by grade. In 2010, the WHO validated this crucial role of proliferative rate, thus classified gastroenteropancreatic (GEP) NETs into two categories: well differentiated known as Grade 1 and Grade 2 and poorly differentiated category referred as Grade 3. On statistical analysis of a study, it was found that 76.5% cases are Grade 3, 68.75% cases are of Grade 2 and 51.6% belongs to Grade 1 category.
Market for Neuroendocrine Tumors
Neuroendocrine tumors are a rare kind of tumor. It is estimated that more than 12,000 people are affected by the disease each year in the U.S. The value of market was approximately US$ 3.5 Bn in 2017, and is expected to increase during the forecast period. The worldwide market for NET can be segmented by site, by treatment type, by diagnostic tools and by end user.
- Treatment Type
- Radiation therapy
- Medication therapy
- Diagnostic tools
- End user
- Specialty Centers
The market is driving owing to combined contributions of various factors such as increasing prevalence of neuroendocrine carcinoma, rising awareness among people, the growing number of government initiatives and technological advancements in the field of medicine and research. Moreover, the continuous efforts undertaken by the pharmaceutical companies to develop targeted therapy for the disease further increases the market growth. However, lack of understanding of the disease along with high development cost and long duration of approval and post-approval formalities impede the market growth to some extent.
On the basis of site, the gastric segment has the dominant share followed by lungs. In the treatment domain, Radiofrequency ablation found to be the only cure for pancreatic NETs, thereby expected to register notable growth. Another suitable treatment is chemotherapy but sometimes surgical excision combined with chemotherapy is used in case of slowly growing tumors.
Among the diagnostic tools, diagnostic imaging techniques dominate the market owing to the advanced technology which make these to detect very tiny and clinically insignificant malignant tumors. Further, among end-users, the requirement to visit and stay in the hospital during NETs treatment facilitates the growth of this segment.
North America has the dominant share in market in 2017 due to supportive health related insurance policies in the region. Moreover, high R&D expenditure in combination with sophisticated healthcare facilities are other factors driving the North American market. Asia Pacific market is expected to witness significant growth in the forecast period due to increasing investments by the regional governments in order to deal with the rising incidence of NETs in the region.
Companies across the globe are thoroughly working toward the development of new treatment therapies for NET. Some of the key players in the therapeutic market of NET at a global level are Novartis AG, Amgen Inc., Boehringer Ingelheim GmbH, Dauntless Pharmaceuticals, Chiasma Inc., Aegis Therapeutics, Ispen, Progenics Pharmaceuticals, Inc., Tarveda Therapeutics, Pfizer, Jubilant Life Sciences Ltd. (Jubilant DraxImage), AbbVie Inc. and others. Novartis has the highest number of ongoing clinical trials followed by Ipsen and Pfizer.
Afinitor (Everolimus) developed by Novartis AG is the medication used to treat adults with a type of cancer known as neuroendocrine tumor (NET) of the stomach and intestine (gastrointestinal) or lung. It is suitable for advanced stage NET that cannot be cured by surgery. This oral medicine has been approved in February, 2016 by the U. S. Food and Drug Administration.
Sutent (Sunitinib Malate) developed by Pfizer is an oral, small-molecule that was approved by the FDA to treat the portion of patients whose NET has progressed and cannot be treated with surgery. Sunitinib inhibits cellular signaling by targeting multiple receptor tyrosine kinases (RTKs).
Somatuline Depot (Lanreotide) developed by Ipsen Biopharmaceuticals Inc. is a medication used in the management of acromegaly and symptoms caused by neuroendocrine tumors. This subcutaneous injection has been approved by the FDA and has biological activity similar to naturally occurring somatostatin.
Surufatinib has completed phase III of clinical studies at Hutchison Medipharma Limited. It is an orally administered antineoplastic class of drug that has been already registered for approval to treat neuroendocrine tumors.
177Lu-edotreotide PRRT provides targeted molecular therapy in patients with inoperable, progressive neuroendocrine tumours of gastroenteric or pancreatic origin. The drug is in phase III of clinical trials at ITM Isotopen Technologien Muenchen GmbH.
Anlotinib is in phase III clinical trials at Advenchen Laboratories, LLC. It is a new, orally administered tyrosine kinase inhibitor in clinical development. It can inhibit both tumor angiogenesis and tumor cell proliferation.
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