Snapshot A 32-year-old woman presents to the clinic with complaint of diarrhea for the past three months. She states that her diarrhea has been watery and not greasy, and she has not seen any blood in the toilet bowl. Upon further questioning, she states that she has also experienced wheezing and skin peeling on her hands and feet. On physical examination, the patient's face and neck appears as seen in the image. Introduction Overview carcinoid syndrome is a rare syndrome that is caused by the metastasis of carcinoid tumors that secrete high levels of serotonin (5-HT) treatment is usually with surgical resection or octreotide Epidemiology incidence rare, 1-2 cases per 100,000 individuals demographics most frequently in patients 50-70 years of age Pathophysiology metastasis of carcinoid tumors that produce serotonin carcinoid tumors most commonly located in the ileum and appendix (90%) carcinoid tumors arise from neuroendocrine cells carcinoid syndrome is observed if there is metastasis of the tumor outside of the GI system if there is metastasis to the liver, first-pass metabolism of secreted serotonin is disrupted and hormones reach systemic circulation Associated conditions medical conditions pellagra caused by niacin (vitamin B3) deficiency all tryptophan is consumed in making excess 5-HT Presentation Symptoms common symptoms cutaneous flushing recurrent diarrhea ↑ bowel motility abdominal cramps asthma-like wheezing Physical exam erythema pellagra skin lesions wheezing hepatomegaly from metastases pulmonary systolic and diastolic heart murmur cardiac involvement causes carcinoid heart disease primarily occurs on right side of the heart tricuspid valve most commonly affected Imaging CT indication localize the primary tumor assess extent of tumor spread and metastases In-111 octreotide scan indication localize the primary carcinoid tumor and recurrences Studies Labs measurement of urinary excretion of 5-HIAA best initial test urinary excretion of 5-HIAA ≥ 25 mg/day is diagnostic 5-HT is degraded to 5-HIAA and excreted in the urine Differential VIPoma key distinguishing factor presents with WDHA syndrome Watery Diarrhea Hypokalemia Achlorhydria may present with flushing similar to carcinoid syndrome, but no wheezing or right-sided heart murmurs are present Treatment Surgical surgical resection of tumor indications first-line treatment reduces tumor mass and achieves symptom remission Medical octreotide or other somatostatin analog indications alleviates flushing and diarrhea Complications Carcinoid heart disease incidence up to 50% of patients with carcinoid syndrome risk factors higher urinary levels of 5-HIAA associated with greater risk of progression of carcinoid heart disease treatment somatostatin analogs
QUESTIONS 1 of 3 1 2 3 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.EC.15.75) A 54-year-old male presents to his primary care physician with complaints of severe diarrhea, flushing of the face, and several incidents of trouble breathing over the past week. A CT scan (Figure A) is obtained and shows a primary tumor in the small bowel (blue arrow) as well as metastasis to the liver (yellow arrow). The patient is referred to a surgeon for further management. The surgeon schedules a date to attempt surgical removal of all tumor tissue. The surgeon also decides to start the patient on a medication to control symptoms prior to surgery. Which of the following medications is the best choice for the management of this patient's symptoms? QID: 106744 FIGURES: A Type & Select Correct Answer 1 Propranolol 4% (7/158) 2 Phenoxybenzamine 18% (29/158) 3 Octreotide 70% (110/158) 4 Propylthiouracil 3% (5/158) 5 Sumatriptan 3% (5/158) M 3 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (M1.EC.13.129) A 58-year-old female presents to her primary care physician with a 1-month history of facial and chest flushing, as well as intermittent diarrhea and occasional difficulty breathing. On physical exam, a new-onset systolic ejection murmur is auscultated and is loudest at the left second intercostal space. Subsequent echocardiography reveals leaflet thickening secondary to fibrous plaque deposition on both the pulmonic and tricuspid valves. Which of the following laboratory abnormalities would most likely in this patient? QID: 100645 Type & Select Correct Answer 1 Decreased serum chromogranin A 17% (5/29) 2 Elevated serum bicarbonate 3% (1/29) 3 Elevated urinary vanillylmandelic acid 31% (9/29) 4 Elevated serum potassium 3% (1/29) 5 Elevated urinary 5-hydroxyindoleacetic acid 45% (13/29) M 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic