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Optimizing Management of Carcinoid Syndrome in Patients With Neuroendocrine Tumors

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i3 Health is pleased to make the clinician's resource guide available for use as a nonaccredited self-study or teaching resource.

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Optimizing Management of Carcinoid Syndrome in Patients With Neuroendocrine Tumors

  1. 1. www.i3Health.com CARCINOID SYNDROME CLINICIAN’S RESOURCE GUIDE The clinical tools and resources contained herein are provided as educational adjuncts to the CE- certified online activity, Optimizing Management of Carcinoid Syndrome in Patients With Neuroendocrine Tumors. To access the activity and earn CE credit, visit: https://www.i3Health.com/carcinoid-syndrome CONTENTS I: NCI-CTCAE Version 4.03: Grading Scale for Symptoms of Carcinoid Syndrome .......................... 2 II: Clinical Presentation of Carcinoid Syndrome ...................................................................................... 3 III: Carcinoid Syndrome Impact Tool......................................................................................................... 4 IV: Medications and Foods to Avoid Before 5-HIAA Test...................................................................... 5 V: Bristol Stool Form Scale......................................................................................................................... 6 VI: Assessment of Carcinoid Heart Disease.............................................................................................. 7 VII: Tests to Identify Causes of Flushing.................................................................................................... 8 VIII: NCCN Guidelines: Systemic Therapy for Neuroendocrine Tumors................................................ 9
  2. 2. 074CS Clinician’s Resource Guide | Page 2 of 9 www.i3Health.com I: NCI-CTCAE VERSION 4.03: GRADING SCALE FOR SYMPTOMS OF CARCINOID SYNDROME Adverse Event Grade 1 Grade 2 Grade 3 Grade 4 Skin flushing Asymptomatic; clinical or diagnostic observations only; intervention not indicated Moderate symptoms; medical intervention indicated; limiting instrumental ADL Symptomatic, associated with hypotension and/or tachycardia; limiting self care ADL - Diarrhea Increase of <4 stools per day over baseline; mild increase in ostomy output compared to baseline Increase of 4-6 stools per day over baseline; moderate increase in ostomy output compared to baseline Increase of ≥7 stools per day over baseline; incontinence; hospitalization indicated; severe increase in ostomy output compared to baseline; limiting self care ADL Life-threatening consequences; urgent intervention indicated Wheezing Detectable airway noise with minimal symptoms Moderate symptoms; medical intervention indicated; limiting instrumental ADL Severe respiratory symptoms limiting self care ADL; oxygen therapy of hospitalization indicated Life-threatening consequences; urgent intervention indicated Tachycardia Asymptomatic, intervention not indicated Symptomatic, medical intervention indicated Medical intervention indicated; IV medication needed Life-threatening consequences; hemodynamic compromise; urgent intervention indicated NCI-CTCAE = National Cancer Institute Common Terminology Criteria for Adverse Events; ADL = activities of daily living. National Cancer Institute (2010). National Cancer Institute Common Terminology Criteria for Adverse Events. V4.03. Available at: https://evs.nci.nih.gov
  3. 3. 074CS Clinician’s Resource Guide | Page 3 of 9 www.i3Health.com II: CLINICAL PRESENTATION OF CARCINOID SYNDROME Clinical Presentation Syndrome Tumor Type Sites Hormones Flushing Carcinoid Medullary thyroid carcinoma Pheochromocytoma Carcinoid C cell tumor Tumor of chromaffin cells Mid/foregut Adrenal medulla Gastric Thyroid C cells Adrenal and sympathetic nervous system Serotonin CGRP Calcitonin Metanephrine and normetanephrine Diarrhea, abdominal pain, and dyspepsia Carcinoid WDHHA Zollinger-Ellison Pancreatic polypeptide Medullary thyroid carcinoma Carcinoid VIPoma Gastrinoma PPoma Medullary thyroid carcinoma Mastocytoma As above Pancreas Mast cells Thyroid As above VIP Gastrin Pancreatic polypeptide Calcitonin Diarrhea/ steatorrhea Somatostatin Bleeding GI tract Somatostatinoma Neurofibromatosis Pancreas Duodenum Somatostatin Wheezing Carcinoid Carcinoid Gut/pancreas/ lung Substance P, CGRP, serotonin Ulcer/dyspepsia Zollinger-Ellison Gastrinoma Pancreas Duodenum Gastrin Hypoglycemia Whipple’s triad Insulinoma Sarcoma Hepatoma Pancreas Retroperitoneal liver Insulin IGF1 IGF11 Dermatitis Sweet syndrome Pellagra Glucagonoma Carcinoid Pancreas Midgut Glucagon Serotonin Dementia Sweet syndrome Glucagonoma Pancreas Glucagon Diabetes Glucagonoma Somatostatin Glucagonoma Somatostatinoma Pancreas Pancreas Glucagon Somatostatin DVT, steatorrhea, cholelithiasis Neurofibromatosis Somatostatin Somatostatinoma Pancreas Duodenum Somatostatin Silent, liver metastases Silent PPoma Pancreas Pancreatic polypeptide CGRP = calcitonin gene-related peptide; GI = gastrointestinal; IGF = insulin-like growth factor. Adapted from Vinik A, Hughes MS, Feliberti E, et al (2018). Carcinoid tumors. Available at: https://ww.endotext.org
  4. 4. 074CS Clinician’s Resource Guide | Page 4 of 9 www.i3Health.com III: CARCINOID SYNDROME IMPACT TOOL Available at: https://www.aboutcarcinoid.com/carcinoid-syndrome-impact-tool
  5. 5. 074CS Clinician’s Resource Guide | Page 5 of 9 www.i3Health.com IV: MEDICATIONS AND FOODS TO AVOID BEFORE 5-HIAA TEST Medications Foods (all ≥24 h prior) • Cough and antihistamine preparations (≥48 h prior) • Thorazine and compazine • Nasal drops and sprays, especially if the individual normally develops palpitations and sweats when these medications are used • Hypertension medications, acetaminophen, and muscle relaxants, especially prescribed for lower back pain (eg, methocarbamol) • Diazepam and cyclobenzaprine (≥48 h prior) • MAO inhibitors (eg, phenelzine) • Avocado • Bananas • Cantaloupe • Eggplant • Grapefruit • Hickory nuts/pecans • Honeydew • Kiwi • Pineapple and its juice • Plantains • Plums • Tomatoes and all tomato products • Walnuts Adapted from The Carcinoid Cancer Foundation (2018). Available at: https://www.carcinoid.org/for-doctors/diagnosis-and- surveillance/preparing-for-the-24-hour-urine-5hiaa-test and National Comprehensive Cancer Network (2017). NCCN Clinical Practice Guidelines in Oncology: neuroendocrine tumors. Version 3.2017. Available at: https://www.nccn.org
  6. 6. 074CS Clinician’s Resource Guide | Page 6 of 9 www.i3Health.com V: BRISTOL STOOL FORM SCALE Heaton KW & Lewis SJ (1997). Stool form scale as a useful guide to intestinal transit time. Scand J Gastroenterol, 32(9):920-924.
  7. 7. 074CS Clinician’s Resource Guide | Page 7 of 9 www.i3Health.com VI: ASSESSMENT OF CARCINOID HEART DISEASE Typical Characteristics of Carcinoid Heart Disease Significant tricuspid regurgitation Mixed pulmonary regurgitation and stenosis Concomitant left-sided involvement (<10%), primarily in patients with persistent foramen ovale, bronchial carcinoid, or severe carcinoid syndrome Pathognomonic fibrous plaques on echocardiography involving the endocardium of valve leaflets and cardiac chambers Intramyocardial metastases Adapted from Hart et al, 2017. Hart EA, Mejis RCA, Meijer KM, et al (2017). Carcinoid heart disease: a guide for screening and timing of surgical intervention. Netherlands Heart J, 25(9):471-478. Test(s) Purpose Frequency • Cardiology consultation • Echocardiogram During treatment for carcinoid syndrome to assess whether the patient has carcinoid heart disease Every 2 to 3 years • Surveillance imaging of the abdomen and pelvis using multiphasic CT or MRI • Chest CT scans with or without contrast To monitor disease control and/or progression Every 3 to 12 months Adapted from National Comprehensive Cancer Network (2017). NCCN Clinical Practice Guidelines in Oncology: neuroendocrine tumors. Version 3.2017. Available at: https://www.nccn.org
  8. 8. 074CS Clinician’s Resource Guide | Page 8 of 9 www.i3Health.com VII: TESTS TO IDENTIFY CAUSES OF FLUSHING Clinical Condition Tests Carcinoid • Urine 5-HIAA • 5-hydroxytryptophan • Substance P • CGRP • Chromogranin A Medullary thyroid carcinoma • Calcitonin • Calcium infusion • Ret proto-oncogene Pheochromocytoma • Plasma-free metanephrines • Urine metanephrines • Vanillyl mandelic acid • Epinephrine • Norepinephrine • Glucagon stimulation • MIBG Diabetic autonomic neuropathy • Heart rate variability • 2-hr postprandial glucose Menopause • Follicle stimulating hormone Epilepsy • Electroencephalogram Panic • Pentagastrin/Adrenocorticotropic hormone Mastocytosis • Plasma histamine • Urine tryptase Hypomastia, mitral prolapse • Cardiac echo Adapted from Vinik A, Hughes MS, Feliberti E, et al (2018). Carcinoid tumors. Available at: http://www.endotext.org
  9. 9. 074CS Clinician’s Resource Guide | Page 9 of 9 www.i3Health.com VIII: NCCN GUIDELINES: SYSTEMIC THERAPY FOR NEUROENDOCRINE TUMORS Indication Agents Unresectable and/or Metastatic NET of the Gastrointestinal Tract • Octreotide LAR 20-30 mg intramuscular injection, monthly • Lanreotide 120 mg deep subcutaneous injection, monthly • Consider (listed in alphabetical order): o Anticancer agents such as 5-fluorouracil, capecitabine, dacarbazine, oxaliplatin, streptozocin, and temozolomide can be used in patients with progressive metasatases for whom there are no other treatment options (all category 3) o Everolimus o Interferon alfa-2b (category 3) Unresectable and/or Metastatic NET of the Lung/Thymus • Depending on tumor burden, options may include: o Octreotide LAR 20-30 mg intramuscular injection, monthly o Lanreotide 120 mg deep subcutaneous injection, monthly o Everolimus ± octreotide or lanreotide o Temozolomide ± octreotide or lanreotide o Cisplatin + etoposide ± octreotide or lanreotide o Carboplatin + etoposide ± octreotide or lanreotide Options for Carcinoid Syndrome • Octreotide or lanreotide ± therapy for poorly controlled carcinoid syndrome, including: o Telotristat 250 mg orally, three times daily (for persistent diarrhea) and/or o Additional therapy for disease control (for any persistent systems [eg, flushing, diarrhea]) Adapted from National Comprehensive Cancer Network (2017). NCCN Clinical Practice Guidelines in Oncology: neuroendocrine tumors. Version 3.2017. Available at: https://www.nccn.org

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