Infographic - Optimizing Neuroendocrine Tumor Outcomes

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Neuroendocrine Tumors are tough to spot, tougher to treat. This infographic, created from Aman Chauhan, MD, Associate Professor of Medical Oncology, Leader of th...


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Incidence Prevalence8.52 NENs per 100,000
(2021) 170,000 US patients
with NETs (2016) NETs are the solid
tumor with the
4TH HIGHEST
PREVALENCE
4TH HIGHEST
PREVALENCE
after colorectal,
lung and ovarian
Recorded
incidence has
NEARLY
DOUBLED
NEARLY
DOUBLED
in the last 25 years MEDIAN OVERALL
SURVIVAL Well-Differentiated
NETs Poorly-Differentiated
NECs 16.8 YEARS
Spectrum of Neuroendocrine Neoplasms NENs range from well-differentiated
neuroendocrine tumors to poorly-differentiated
neuroendocrine carcinomas (NECs) and are
graded according to Ki-67 and mitotic index
(grade 1 to grade 3) Patients with low-to-intermediate–grade
NETs can live for years, whereas high-grade,
poorly-differentiated NETs have a dismal
prognosis NETs can have dramatically different prognosis
depending on location, functionality, grade,
differentiation, extent of disease, and pace of
growth WD = well-differentiated; PD = poorly-differentiated G1 WD NET G2 WD NET G3 WD NET G3 PD NEC
GINET Well-Differentiated NET NEC Poorly-Differentiated NET Typical
Carcinoid Atypical
Carcinoid SCLC and
LCNEC NET Well-Differentiated NET NEC Poorly-Differentiated NET Lung SCLC = small cell lung cancer; LCNEC = large cell neuroendocrine carcinoma
Aman Chauhan, MD, Associate Professor of Medical Oncology,
Leader of the Neuroendocrine Tumor Program,
Co-Director of the Theranostics Program, University of Miami,
Sylvester Comprehensive Cancer Center
What Are
NETs?
Neuroendocrine tumors (NETs) are a diverse group
of cancers originating from neuroendocrine tissue
25%Lungs
14%No primary site
identified
8%Pancreas
53%All
gastrointestinal
(GI) NETs range from indolent
to aggressive and have
widely ranging treatment
according to location,
grade (G), and stage Most occur in the
gastrointestinal tract,
lungs, and pancreas, and
14% have no identifiable
primary site NETs can be functional or
nonfunctional. Functional
NETs produce hormones,
which can cause
symptoms Neuroendocrine tissues
occur throughout the
body, and NETs can occur
anywhere neuroendocrine
tissues are located
20%-30% of NETs are
functional and
produce hormones,
including:SEROTONIN VASOACTIVE INTESTINAL PEPTIDE GASTRIN GLUCAGON INSULIN
Nevertheless, NETs are considered rare cancers with limited treatment optionsExtent of Disease​ Low tumor burden/resectable​ High tumor burden/unresectable​ Widely metastatic​ Liver-dominant​
Pathology is key to determining prognosis and treatment
EVALUATION AND PROGNOSIS OF NETS
Characteristics of NETs​Pace of Growth​ Stable (lower Ki-67, lower mitotic
index)​ Progressive (higher Ki-67, higher
mitotic index)​ Primary Site (Gastrointestinal)​ Foregut​ Midgut Hindgut Pancreas Grade/Differentiation​ Well-Differentiated NET​ Poorly-Differentiated NEC​ Low-grade (G1)​ Intermediate-grade (G2)​ High-grade (G3)​ High-grade (G3)​ Hormone Status​ Functional​ Non-functional​ Somatostatin Receptor Expression​ High expression​ Low expression​
OPTIMIZING NEUROENDOCRINE
TUMOR OUTCOMES:
Closing the Gaps in Diagnosis and Care
INCIDENCE AND PREVALENCE OF
NEUROENDOCRINE NEOPLASMS (NENs)

SYMPTOMSAbdominal Pain Nausea Diarrhea Indigestion Weight Loss Flushing Persistent Cough Vomiting Wheezing NET patients report having
originally been misdiagnosed
1 in 21 in 2 from first symptoms to
correct NET diagnosis
4.3 years4.3 yearsIt takes a median of CORRECT DIAGNOSIS NETsNETs
Treatment of NETs
EVALUATIONImaging
Biochemical
evaluation
Molecular
profiling
Genetic
counseling Local/
Locoregional Surgery Locally
Advanced
Metastatic Systemic
Therapy Liver-Directed
Therapy No role for adjuvant chemotherapy Immunotherapy
(NECs only) Chemotherapy Somatostatin Analogues Octreotide
Lanreotide Lu-dotatate
177 Radioligand Therapy Targeted Therapy Cabozantinib
Everolimus
Sunitinib Liver-directed radioembolization Hepatic arterial embolization Histotripsy Liver resection Thermal ablation
THERAPY
SEQUENCING:
THERAPY
SEQUENCING:Somatostatin
analogues 1 Lu-dotatate
177
(first line for Ki-67 >10%) 2 Cabozantinib, capecitabine/temozolomide
(CAPTEM), everolimus, sunitinib (pNET) 3
1980 1982 1988 2011 2014 2016 2017 2018 2020 2025 SSTR-positive if uptake in
measurable lesions is greater in liver Ga-dotatate, Ga-dotatoc,
68 68
Cu-dotatate
64 SSTR-PET/CT or SSTR-PET/MRI, or
octreotide SPECT/CT only if SSTR-PET
is not available Symptoms are
highly variable, from
asymptomatic to
profoundly disabling Others can cause
mechanical
complications
(bleeding, obstruction)
or hormonal effects
Many NETs are
incidentally
discovered
NETs is a chronic indolent
disease in many
This can be anot a
Prioritize quality of life
alongside longevity
NETs are
andReferral to a NET multidisciplinary
program is vital
Accessing a specialized
center is NOT always EASY
Accessing a specialized
center is NOT always EASY
Peptide Receptor
Radionuclide
Therapy (PRRT)
at certain locationsis a significant milestone in NET
treatment, but access can be a
DIAGNOSTIC TOOLSDIAGNOSTIC TOOLSWell-Differentiated Poorly-Differentiated High-grade
(G3) >20% >20 High-grade
(G3) Intermediate-grade
(G2) Low-grade
(G1) Grade Ki-67 Index Mitotic Index
(mitoses/2 mm)
2 Functional
Imaging >20% 3%-20% <3% >20 2-20 <2 Octreoscan SPECT or SSTR PET-positive FDG PET-positive References
Chauhan A, Chan K, Halfdanarson TR, et al (2024). Critical updates in neuroendocrine tumors: version 9 American Joint Committee on Cancer staging system for gastroenteropancreatic neuroendocrine
tumors. CA: A Cancer Journal for Clinicians, 74(4):359-367. DOI:10.3322/caac.21840
Chauhan A, Kohn E & Del Rivero J (2020). Neuroendocrine tumors-less well known, often misunderstood, and rapidly growing in incidence. JAMA Oncol, 6(1):21-22. DOI:10.1001/jamaoncol.2019.4568
Corbett V, Arnold S, Anthony L & Chauhan A (2021). Management of large cell neuroendocrine carcinoma. Front Oncol, 11:653162. DOI:10.3389/fonc.2021.653162
Dasari A, Shen C, Halperin D, et al (2017). Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol, 3(10):1335-1342.
DOI:10.1001/jamaoncol.2017.0589
Dasari A, Wallace K, Halperin DM, et al (2025). Epidemiology of Neuroendocrine Neoplasms in the US. JAMA Netw Open, 8(6):e2515798.
National Comprehensive Cancer Network (2025). Clinical practice guidelines in oncology: Neuroendocrine and Adrenal Tumors. Version 2. 2025. Available at: https://www.nccn.org/guidelines/guidelines-
detail?category=1&id=1448
Oronsky B, Ma PC, Morgensztern D & Carter CA (2017). Nothing but NET: A review of neuroendocrine tumors and carcinomas. Neoplasia, 19(12):991–1002. DOI:10.1016/j.neo.2017.09.002
Perez K, Del Rivero J, Kennedy EB, et al (2025). Symptom management for well-differentiated gastroenteropancreatic neuroendocrine tumors: ASCO Guideline. JCO Oncology Practice, [Online Ahead of Print].
10.1200/OP-25-00133
Singh S, Granberg D, Wolin E, et al (2016). Patient-reported burden of a neuroendocrine tumor (NET) diagnosis: results from the first global survey of patients with NETs. J Glob Oncol, 3(1):34-53.
DOI:10.1200/JGO.2015.002980
Wolin EM, Leyden J, Goldstein G, et al (2017). Patient-reported experience of diagnosis, management, and burden of neuroendocrine tumors. Pancreas, 46(5):639-647. DOI:10.1097/MPA.0000000000000818 This educational activity is supported by a medical education grant from Exelixis, Inc. GEP-NET = gastroenteropancreatic NET
DIAGNOSISDIAGNOSIS
PHYSICIAN AWARENESS IS IMPORTANTPHYSICIAN AWARENESS IS IMPORTANT
If you don’t suspect it, you can’t detect it!If you don’t suspect it, you can’t detect it!
PATIENT PERSPECTIVESINCORRECT DIAGNOSIS Stomach Ulcers Irritable Bowel Syndrome Obstructed Bowel Pneumonia Gastritis Asthma Anxiety or Depression Menopause FDG = fluorodeoxyglucose; PET = positron emission tomography; SPECT = single photon emission computed tomography; SSTR = somatostatin receptor; Ga = gallium; Cu = copper FUNCTIONAL
IMAGING IS KEY Co-management with a high-volume
multidisciplinary NETs center is highly encouraged Clinical trials preferred for any line
NEUROENDOCRINE ONCOLOGYNEUROENDOCRINE ONCOLOGY
ADVANCES INADVANCES INOctreotide Lanreotide in
GEP-NET Telotristat Ga-Dotatate
PET
68 Streptozocin Everolimus
Sunitinib in
pNET Everolimus
in Lung/GI NET Capecitabine/
Temozolomide Lu-Dotatate
177 Cu-Dotatate PET
64 Cabozanitinib pNET = pancreatic neuroendocrine tumor Lu = lutetium
Targeted therapies
(cabozantinib,
everolimus) are effective
but can have side effects
and
are key