Carcinoid Syndrome - Market Insights, Epidemiology, and Market Forecast - 2034

Key Highlights

• Carcinoid syndrome occurs when a rare cancerous tumor called a carcinoid tumor secretes certain chemicals into your bloodstream, causing a variety of signs and symptoms. A carcinoid tumor, which is a type of neuroendocrine tumor, occurs most often in the gastrointestinal tract or the lungs.
• Only about 10% of the people with a carcinoid tumor will develop carcinoid syndrome. Major symptoms of this syndrome include hot, red facial flushing, diarrhea and wheezing.
• Very rarely, people with carcinoid syndrome have carcinoid crises, a potentially life-threatening condition that can happen if your carcinoid syndrome goes undiagnosed or untreated.
• The management of carcinoid syndrome centers on suppressing biogenic amine release and reducing tumor burden using multiple modalities. Somatostatin analogs are the cornerstone of medical management. Two commonly used analogs, octreotide and lanreotide, mimic the action of somatostatin. These drugs inhibit the secretion of biogenic amines, thereby controlling symptoms such as flushing and diarrhea.
• For patients with inoperable hepatic metastases, liver-directed therapies can reduce morbidity and improve quality of life.
• Telotristat is an FDA-approved agent to treat refractory diarrhea not responding to somatostatin analogs (SSA). It inhibits the enzyme tryptophan hydroxylase, which catalyzes a rate-limiting step in the conversion of tryptophan to serotonin.
• The carcinoid syndrome market currently has limited active players, with key companies like Crinetics Pharmaceuticals and Sanwa Kagaku Kenkyusho (developers of PALSONIFY [paltusotine]) pursuing innovative treatments and expanding their pipelines within this therapeutic area.

DelveInsight's “Carcinoid Syndrome – Market Insight, Epidemiology and Market Forecast – 2034” report delivers an in-depth analysis of Carcinoid Syndrome epidemiology, market, and clinical development in Carcinoid Syndrome. In addition to this, the report provides historical and forecasted epidemiology and market data as well as a detailed analysis of the Carcinoid Syndrome market trends in the United States, EU4 (Germany, France, Italy, and Spain), and the United Kingdom, and Japan.

The Carcinoid Syndrome market report provides real-world prescription pattern analysis, emerging drugs assessment, market share, and uptake/adoption pattern of individual therapies, as well as historical and forecasted Carcinoid Syndrome market size from 2020 to 2034 in 7MM. The report also covers current Carcinoid Syndrome treatment practices/algorithms and unmet medical needs to curate the best opportunities and assess the market’s underlying potential.

Geography Covered

• The United States
• EU4 (Germany, France, Italy, and Spain) and the United Kingdom
• Japan

Carcinoid Syndrome Disease Understanding and Treatment Algorithm

Carcinoid Syndrome Overview

Carcinoid syndrome is a rare condition caused by metastatic, well-differentiated neuroendocrine tumors, mainly from the midgut with liver metastases. These tumors secrete serotonin and other amines, leading to symptoms like flushing, diarrhea, bronchospasm, and complications such as carcinoid heart disease. Flushing (often triggered by stress, food, or alcohol) and chronic diarrhea are the most common and debilitating features.

Carcinoid syndrome is caused by neuroendocrine tumors that metastasize to the liver, with the majority of these tumors originating from the midgut, particularly the appendix and small intestine. Neuroendocrine tumors without liver metastases typically do not produce carcinoid syndrome, as the liver metabolizes the biogenic amines produced by the primary tumor.

Carcinoid Syndrome Diagnosis

The diagnostic evaluation of carcinoid syndrome involves initial testing for biochemical markers to confirm the presence of the syndrome, followed by tumor localization and staging through radiographic and endoscopic studies. The 24-hour urinary 5-HIAA test is the initial diagnostic test for carcinoid syndrome. 5-HIAA is the primary end product of serotonin metabolism, and this test demonstrates approximately 90% sensitivity and specificity.

When biochemical markers confirm the diagnosis of carcinoid syndrome, imaging studies are essential for tumor localization and staging. Cross-sectional imaging techniques such as triple-phase computed tomography (CT) scans, magnetic resonance imaging (MRI), and somatostatin receptor scintigraphy are commonly employed. Due to its high sensitivity, abdominal CT with triphasic imaging of the liver is often the diagnostic modality of choice. MRI is sometimes preferred for its superior sensitivity in detecting liver metastases. Bronchoscopy with biopsy can be performed for direct visualization and histological confirmation of bronchial neuroendocrine tumors.

Patients with significant elevation of serum serotonin or urinary 5-HIAA (≥5 times the upper limit of normal), symptoms of carcinoid heart disease, or those undergoing major surgery should undergo echocardiographic evaluation to assess for cardiac involvement.

Further details related to country-based variations in diagnosis are provided in the report

Carcinoid Syndrome Treatment

The management of carcinoid syndrome focuses on controlling the release of biogenic amines and reducing tumor burden through a multimodal approach. Somatostatin analogs remain the mainstay of medical therapy. In surgical settings, elective cholecystectomy is often considered to prevent gallbladder complications related to long-term SSA use. While small gastric or rectal neuroendocrine tumors may be curatively treated with endoscopic resection, they rarely cause carcinoid syndrome. For patients with inoperable liver metastases, liver-directed therapies can alleviate symptoms and enhance quality of life.

Somatostatin analogs help reduce excessive hormone production and may slow tumor growth, especially when cancer has spread. Peptide Receptor Radionuclide Therapy (PRRT) is a targeted treatment for advanced neuroendocrine tumors that express somatostatin receptors. It uses radiolabeled somatostatin analogs (e.g., lutetium-177) to deliver localized radiation to tumor cells. PRRT is most effective for well-differentiated, low-to-intermediate grade tumors, particularly those of gastrointestinal or pancreatic origin.

In the event of a carcinoid crisis, immediate management includes a bolus of octreotide followed by continuous IV infusion, along with fluid resuscitation and vasopressor support as needed.

Carcinoid Syndrome Epidemiology

The Carcinoid Syndrome cancer epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented as total incident population of carcinoid tumor, total incident population of carcinoid syndrome, gender-specific incident population of carcinoid syndrome, origin-specific incident population of carcinoid syndrome, and total treated cases of carcinoid syndrome in the 7MM, covering the United States, EU4 (Germany, France, Italy, and Spain), and the United Kingdom, and Japan from 2020 to 2034.

• A carcinoid tumor is a rare type of neuroendocrine tumor that most often occurs in the gastrointestinal tract or the lungs, with an estimated incidence of fewer than 3 cases per 100,000 individuals each year.
• Fewer than 10% of people with carcinoid tumors develop symptoms. The incidence of symptoms, however, may vary based on the location of the tumor.
• Approximately 40% of all gastrointestinal carcinoid tumors are located in the small intestine, while pulmonary carcinoid tumors represent about 1–2% of all lung cancers.
• Carcinoid tumors are more common in women than in men.

Carcinoid Syndrome Drug Chapters

The drug chapter segment of the Carcinoid Syndrome report encloses a detailed analysis of Carcinoid Syndrome’s late-stage (Phase III) pipeline drugs. It also deep dives into Carcinoid Syndrome’s pivotal clinical trial details, recent and expected market approvals, patent details, the latest news, and recent deals and collaborations.

Marketed Drugs

SOMATULINE AUTOGEL /SOMATULINE DEPOT (lanreotide): Ipsen Biopharmaceuticals

SOMATULINE AUTOGEL /SOMATULINE DEPOT is made of the active substance lanreotide and is a long-acting somatostatin analogue that inhibits the secretion of growth hormone and certain hormones secreted by the digestive system. The new electronic auto-injector is designed to enhance the treatment experience for individuals living with carcinoid syndrome, along with gastroenteropancreatic neuroendocrine tumors and acromegaly.

In September 2017, the US Food and Drug Administration (FDA) approved a supplemental indication for SOMATULINE DEPOT for the treatment of carcinoid syndrome; when used, it reduces the frequency of short-acting somatostatin analogue rescue therapy.

SANDOSTATIN LAR DEPOT (octreotide acetate): Novartis

SANDOSTATIN LAR DEPOT (octreotide acetate) is a somatostatin analog indicated for patients with metastatic carcinoid tumors who experience severe diarrhea and flushing, and who have previously responded to and tolerated subcutaneous Sandostatin injections. Additionally, it is also approved for long-term maintenance therapy in acromegalic patients who have had an inadequate response to surgery and/or radiotherapy, or for whom surgery and/or radiotherapy is not an option, and long-term treatment of the profuse watery diarrhea associated with VIP-secreting tumors.

In October 2024, Teva Pharmaceuticals launched the first and only generic version of SANDOSTATIN LAR DEPOT in the United States.

Emerging Drugs

PALSONIFY (paltusotine): Crinetics Pharmaceuticals and Sanwa Kagaku Kenkyusho

Paltusotine is the first oral, once-daily, selectively-targeted SST2 agonist and is currently in Phase III clinical development for carcinoid syndrome associated with neuroendocrine tumors. Additionally, it is also in investigational Phase III studies for acromegaly. Phase II clinical trials (NCT07087054) began in August 2025 for patients with carcinoid syndrome caused by well-differentiated neuroendocrine tumors.

In March 2024, Crinetics Pharmaceuticals announced positive topline results from its Phase II (NCT05361668) study of oral paltusotine in carcinoid syndrome. The drug showed rapid, sustained symptom relief—reducing flushing by 63%, excess bowel movements by 60%, flushing severity by 61%, and urgency by 64%. Benefits were seen within 2 weeks and lasted 8 weeks. Paltusotine was well-tolerated, with no serious treatment-related adverse events. Biomarker data supported its activity.

Carcinoid Syndrome Drug Class Insights

Somatostatin Analogs

Somatostatin analogs treat carcinoid syndrome by mimicking the natural hormone somatostatin and binding to receptors on neuroendocrine tumor cells, thereby inhibiting the release of serotonin and other substances that cause symptoms like flushing and diarrhea. These drugs—such as octreotide and lanreotide—are highly effective at controlling hormone-related symptoms in most patients and can also slow tumor growth, which improves overall quality of life and may prolong progression-free survival.

Carcinoid Syndrome Market Outlook

Carcinoid syndrome presents a significant challenge in clinical management due to its debilitating symptoms. Currently, several therapeutic options are available for alleviating these symptoms, which can significantly impact the quality of life of affected patients. The primary treatment modality involves somatostatin analogs, such as octreotide and lanreotide, which have been shown to provide substantial symptom relief in many cases. However, for patients who experience refractory symptoms, additional treatment options exist, including telotristat ethyl, which explicitly targets serotonin production, and everolimus, an mTOR inhibitor that can help control tumor growth. Furthermore, liver-directed therapies and chemotherapy may be considered, depending on the extent of the disease and patient-specific factors. This multifaceted approach underscores the importance of effective personalized treatment plans in managing carcinoid syndrome.

To date, there are only a few efficacious treatments for carcinoid syndrome, especially regarding carcinoid heart disease and mesenteric fibrosis. Therefore, there is a critical need for developing new preclinical models to detect innovative specific molecular targets and to provide the basis for promising clinical studies.

With limited players currently active in the carcinoid syndrome market, the future treatment landscape is moving toward more personalized and targeted approaches. Companies such as Crinetics Pharmaceuticals and Sanwa Kagaku Kenkyusho are advancing their lead candidates through various stages of clinical development, aiming to explore their potential in the treatment of carcinoid syndrome.

Carcinoid Syndrome drug uptake

This section focuses on the uptake rate of potential drugs expected to be launched in the market during 2025–2034, which depends on the competitive landscape, safety, and efficacy data, along with the order of entry.

Further detailed analysis of emerging therapies drug uptake in the report…

Carcinoid Syndrome Pipeline Development Activities

The report provides insights into different therapeutic candidates in (Phase III). It also analyzes key players involved in developing targeted therapeutics.

Pipeline Development Activities

The report covers information on collaborations, acquisitions and mergers, licensing, and patent details for Carcinoid Syndrome cancer therapies.

KOL Views

To keep up with the real-world scenario in current and emerging market trends, we take opinions from Key Industry leaders working in the domain through primary research to fill the data gaps and validate our secondary research. Industry Experts were contacted for insights on the evolving treatment landscape, patient reliance on conventional therapies, patient therapy switching acceptability, and drug uptake, along with challenges related to accessibility, including MDs, Medical/scientific writers, Professors, and Others.

DelveInsight’s analysts connected with 10+ KOLs to gather insights; however, interviews were conducted with 5+ KOLs in the 7MM. Centers such as Louisiana State University Health Science Center-Shreveport, etc., were contacted. Their opinion helps understand and validate current and emerging therapy treatment patterns or Carcinoid Syndrome market trends.

Qualitative Analysis

We perform Qualitative and Market Intelligence analysis using various approaches, such as SWOT Analysis and Conjoint Analysis. In the SWOT analysis, strengths, weaknesses, opportunities, and threats in terms of gaps in disease diagnosis, patient awareness, physician acceptability, competitive landscape, cost-effectiveness, and geographical accessibility of therapies are provided.

Conjoint Analysis analyzes multiple approved and emerging therapies based on relevant attributes such as safety, efficacy, frequency of administration, route of administration, and order of entry. Scoring is given based on these parameters to analyze the effectiveness of therapy.

In efficacy, the trial’s primary and secondary outcome measures are evaluated; for instance, one of the most important primary outcome measures is event-free survival and overall survival.

Further, the therapies’ safety is evaluated, wherein the acceptability, tolerability, and adverse events are majorly observed, and it sets a clear understanding of the side effects posed by the drug in the trials. In addition, the scoring is also based on the probability of success and the addressable patient pool for each therapy. According to these parameters, the final weightage score and the ranking of the emerging therapies are decided.

Market Access and Reimbursement

Reimbursement may be referred to as the negotiation of a price between a manufacturer and a payer that allows the manufacturer access to the market. It is provided to reduce the high costs and make the essential drugs affordable. Health technology assessment (HTA) plays an important role in reimbursement decision-making and recommending the use of a drug. These recommendations vary widely throughout the seven major markets, even for the same drug. In the US healthcare system, both Public and Private health insurance coverage are included. Also, Medicare and Medicaid are the largest government-funded programs in the US. The major healthcare programs, including Medicare, Medicaid, Health Insurance Program (CHIP), and the state and federal health insurance marketplaces, are overseen by the Centers for Medicare & Medicaid Services (CMS). Other than these, Pharmacy Benefit Managers (PBMs) and third-party organizations that provide services and educational programs to aid patients are also present.

The report further provides detailed insights on the country-wise accessibility and reimbursement scenarios, cost-effectiveness scenario of currently used therapies, programs making accessibility easier and out-of-pocket costs more affordable, insights on patients insured under federal or state government prescription drug programs, etc.

Scope of the Report

• The report covers a segment of key events, an executive summary, a descriptive overview of Carcinoid Syndrome, explaining its causes, signs and symptoms, pathogenesis, and currently available therapies.
• Comprehensive insight has been provided into the epidemiology segments and forecasts, the future growth potential of the diagnosis rate, and disease progression along treatment guidelines.
• Additionally, an all-inclusive account of both the current and emerging therapies, along with the elaborative profiles of late-stage and prominent therapies, will have an impact on the current treatment landscape.
• A detailed review of the Carcinoid Syndrome market, historical and forecasted market size, market share by therapies, detailed assumptions, and rationale behind our approach is included in the report, covering the 7MM drug outreach.
• The report provides an edge while developing business strategies, by understanding trends, through SWOT analysis and expert insights/KOL views, patient journey, and treatment preferences that help in shaping and driving the 7MM Carcinoid Syndrome market.

Carcinoid Syndrome Report Insights

• Patient Population
• Therapeutic Approaches
• Carcinoid Syndrome Pipeline Analysis
• Carcinoid Syndrome Market Size and Trends
• Existing and future Market Opportunity

Carcinoid Syndrome Report Key Strengths

• Ten-Years Forecast
• 7MM Coverage
• Carcinoid Syndrome Epidemiology Segmentation
• Key Cross Competition
• Conjoint analysis
• Drugs Uptake and Key Market Forecast Assumptions

Carcinoid Syndrome Report Assessment

• Current Treatment Practices
• Unmet Needs
• Pipeline Product Profiles
• Market Attractiveness
• Qualitative Analysis (SWOT and Conjoint Analysis)

FAQs

• What is the historical and forecasted Carcinoid Syndrome patient pool/patient burden in the United States, EU4 (Germany, France, Italy, and Spain), and the United Kingdom, and Japan?
• How would the market drivers, barriers, and future opportunities affect the market dynamics and subsequent analysis of the associated trends?
• What are the current and emerging options for the treatment of Carcinoid Syndrome?
• What are the recent novel therapies, targets, mechanisms of action, and technologies developed to overcome the limitations of existing therapies?
• Patient acceptability in terms of preferred treatment options as per real-world scenarios?
• What are the country-specific accessibility issues of expensive, recently approved therapies?
• How many key players are developing therapies for Carcinoid Syndrome?
• Which drug is the major contributor to the Carcinoid Syndrome market by 2034?

Reasons to buy

• The report will help in developing business strategies by understanding the latest trends and changing treatment dynamics driving the Carcinoid Syndrome market.
• Insights on patient burden/disease incidence, evolution in diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• Understand the existing market opportunities in varying geographies and the growth potential over the coming years.
• Distribution of historical and current patient share based on real-world prescription data along with reported sales of approved products in the US, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan.
• Identifying strong upcoming players in the market will help devise strategies to help get ahead of competitors.
• Detailed analysis and ranking of class-wise potential emerging therapies under the conjoint analysis section to provide visibility around leading classes.
• Highlights of access and reimbursement policies of approved therapies, barriers to accessibility of expensive off-label therapies, and patient assistance programs.
• To understand Key Opinion Leaders’ perspectives around the accessibility, acceptability, and compliance-related challenges of existing treatment to overcome barriers in the future.
• Detailed insights on the unmet needs of the existing market so that the upcoming players can strengthen their development and launch strategy.

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