Carcinoid syndrome

Background

• Carcinoid tumors are neuroendocrine malignancies
• Primary tumors can be located in:
  • Gastrointestinal tract (55%)
  • Bronchopulmonary system (30%)
  • Other sites rarer or may actually be metastases (ovary, liver, gallbladder, thymus, middle ear)
• Syndrome results from tumor production of serotonin, histamine, bradykinin, and/or prostaglandin

Clinical Features

• Vasodilation
  • Due to increased histamine and kinin production
  • Cutaneous flushing
  • Hypotension, vasodilatory shock
• Diarrhea
• Bronchospasm
• Cardiac valvular lesions
  • Due to serotonin-stimulated fibroblast growth/fibrogenesis
  • Can cause tricuspid or pulmonary regurgitation or stenosis, right heart failure

Differential Diagnosis

Acute allergic reaction

• Allergic reaction/urticaria
• Anaphylaxis
• Angioedema
• Asthma exacerbation
• Anxiety attack
• Scombroid
• Cold urticaria
• Contrast induced allergic reaction
• Shock
• Transfusion reaction
• Carcinoid syndrome

Oncologic Emergencies

Related to Local Tumor Effects

• Malignant airway obstruction
• Bone metastases and pathologic fractures
• Malignant spinal cord compression
• Malignant Pericardial Effusion and Tamponade
• Superior vena cava syndrome

Related to Biochemical Derangement

• Hypercalcemia of malignancy
• Hyponatremia due to SIADH
• Adrenal insufficiency
• Tumor lysis syndrome
• Carcinoid syndrome

Related to Hematologic Derangement

• Neutropenic fever
  • Neutropenic enterocolitis (typhlitis)
• Leukostasis and hyperleukocytosis
• Hyperviscosity syndrome
• Thromboembolism

Related to Therapy

• Chemotherapy-induced nausea and vomiting
• Chemotherapeutic drug extravasation
• Differentiation syndrome (retinoic acid syndrome) in APML
• Stem cell transplant complications

Evaluation

• Evaluation to rule out other causes of shock, diarrhea, etc.
• 24 hour urinary HIAA (5-hydroxyindoleacetic acid)
• Echocardiography
• Imaging to locate the tumor, if not known

Management

• Treat shock and right heart failure
• Octreotide
• Antihistamines

See Also

• Oncologic emergencies
• Octreotide

References

Authors

Claire, Ross Donaldson