Updated: 3/24/2020

Carcinoid Syndrome

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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
 

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Questions (3)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(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? Review Topic | Tested Concept

QID: 106744
FIGURES:
1

Propranolol

4%

(6/148)

2

Phenoxybenzamine

16%

(24/148)

3

Octreotide

72%

(106/148)

4

Propylthiouracil

3%

(5/148)

5

Sumatriptan

3%

(5/148)

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(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? Review Topic | Tested Concept

QID: 100645
1

Decreased serum chromogranin A

22%

(4/18)

2

Elevated serum bicarbonate

0%

(0/18)

3

Elevated urinary vanillylmandelic acid

33%

(6/18)

4

Elevated serum potassium

0%

(0/18)

5

Elevated urinary 5-hydroxyindoleacetic acid

44%

(8/18)

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