FRANKLY SPEAKING ABOUT CANCER: NEUROENDOCRINE & CARCINOID TUMORS (NETS)

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FRANKLY SPEAKING ABOUT CANCER: NEUROENDOCRINE & CARCINOID TUMORS (NETS) Gilda s Club Quad Cities November 5 th, 2018 Joseph Dillon, MD Neuroendocrine Tumor Clinic University of Iowa Hospitals & Clinics

Diffuse (Neuro)Endocrine System Thymus Skin (Merkel) Stomach Cervix/Uterus/Ovary (modified)

¼ = 1,000,000 cells Neuroendocrine Cell

¼ = 1,000,000 cells Neuroendocrine Cell

Neuroendocrine Cell Neuroendocrine Hormones ¼ = 1,000,000 cells

Neuroendocrine Cell Neuroendocrine Hormones Somatostatin Receptor ¼ = 1,000,000 cells

Neuroendocrine ( Carcinoid ) Tumors (NET) = Primary (% occurrence) = Metastasis Liver>Lung>Bone (modified) - Skin / Merkel - Thymus

How many people get a new diagnosis of any cancer per year? ~1.35 million new cancer Modified from Dasari, A. JAMA Oncol. 2017;3:1335

How many people get a new diagnosis of NET per year? ~22,000 new NET 1.6% of all new cancers Modified from Dasari, A. JAMA Oncol. 2017;3:1335

Prevalence, % How many people are living with NET? 0.05 ~150,000 living with NET 0.04 0.03 0.02 0.01 0 1997 2002 Year 2007 2012 Modified from Dasari, A. JAMA Oncol. 2017;3:1335

How many people are living with NET compared to other cancers? Yao, JC. J Clin Oncol 2008

Classification of NETs Important information about your tumor: -Site of origin -Size -Stage (extent of spread; Radiologist) -Grade (how quickly the tumor is growing; Pathologist) -Differentiation (how abnormal looking are tumor cells; Pathologist) -Hormone production (functional vs nonfunctional; blood / urine tests) -Genetic changes* (blood test or tumor biopsy)

Staging of NETs: CT Scan Liver Metastasis Mesenteric nodes

Staging of NETs: Gallium-68 DOTATATE PET (NETSPOT ) scan Pancreas NET Liver Metastases

Classification of NETs Important information about your tumor: -Site of origin -Size -Stage (extent of spread; Radiologist) -Grade (how quickly the tumor is growing; Pathologist) -Differentiation (how abnormal looking are tumor cells; Pathologist) -Hormone production (functional vs nonfunctional; blood / urine tests) -Genetic changes* (blood test or tumor biopsy)

Grading of NETs Ki67 staining Ki-67 stains cells that are dividing <3% Grade 1 3-20% Grade 2 >20% Grade 3

Classification of NETs Important information about your tumor: -Site of origin -Size -Stage (extent of spread; Radiologist) -Grade (how quickly the tumor is growing; Pathologist) -Differentiation (how abnormal looking are tumor cells; Pathologist) -Hormone production (functional vs nonfunctional; blood / urine tests) -Genetic changes* (blood test or tumor biopsy)

What are the symptoms of a NET? No Symptoms Tumor mass: Pain, Bowel obstruction, Bleeding Tumor secretions: Carcinoid Syndrome (~20%): (Diarrhea, Flushing, Wheezing, Palpitations) Other: Changes in BP, blood sugar

How do we diagnose NET? Symptoms: Imaging: Diarrhea, Flushing, Wheeze, Palpitations Local Symptoms: e.g., Abdominal Pain Fluid Retention / Heart Failure CT, MRI, Ga68 DOTA-PET (Diagnosis & Staging) Lab Tests*: Serotonin, CGA, pancreastatin, etc Biopsy: Diagnosis & Grading *(Tumor markers, Biomarkers)

Treatment Protocol for patients with NET Patient Pathology (GRADE): Ki67 / differentiation Somatostatin Receptor Imaging (STAGE) CT, MRI, Gallium- DOTATATE PET (Netspot ) Surgery? Primary tumor, lymph nodes, liver resection / ablation Somatostatin Analog Sandostatin,Somatuline (Serotonin Inhibitor Xermelo ) Hepatic Artery Embolization Bland, Chemo-, Radio- Peptide Receptor Radionuclide Therapy (P.R.R.T.) (LUTATHERA, Clinical Trials) Drug Therapy Targeted : Affinitor, Sutent Conventional : CAP-TEM, other Clinical Trials: Immune Inhibitor

Hepatic artery embolization for NET For localized metastatic liver disease Bland-, Chemo-, or Radio-Embolization Rapidly reverses carcinoid syndrome Can precipitate acute carcinoid syndrome

Not all diarrhea is carcinoid syndrome Cause of Diarrhea Surgical Small Bowel removal Cholecystectomy Small Intestine Bacterial Overgrowth Carcinoid Tumor Treatment Cholestyramine, Colestipol Cholestyramine, Colestipol Antibiotics Octreotide*, Xermelo, Other NET therapy Sandostatin /Lanreotide side effect Pancreas enzymes

University of Iowa Neuroendocrine Tumor Group Thomas M O Dorisio, MD, Director Internal Medicine Thomas O Dorisio, MD Joseph Dillon, MD C. Chandrasekharan, MD Daniel Berg, MD Muhammad Furqan, MD Nuclear Medicine David Bushnell, MD Yusuf Menda, MD Michael Schultz, PhD Michael Graham, MD Nursing/Research & Clinic Staff Kimberly Miller, RN (Clinic) Mary Schall, RN (Clin Res) Kristen Varner, RN (Clin Res) Kellie Bodeker (Clin Res) Melissa Thompson (Clinic) Molly Becker (Clinic) Megan Peterson (Res) Veronica Howsare(Res) Surgery James Howe, MD Kal Parehk, MD Joel Shilyansky, MD Interventional Radiology Schilang Sun, MD Sandeep Laroia, MD Pediatrics Sue O Dorisio, MD, PhD PI Neuroendocrine SPORE

Neuroendocrine Tumor Center of Excellence (ENETS) Neuroendocrine Cancer SPORE (Specialized Program of Research Excellence, NIH)