PANCE Blueprint Pulmonary (10%)

Carcinoid tumors

Patient will present with → a 43-year-old man comes to the emergency department because of a 3-week history of episodic cutaneous flushing, diarrhea, and wheezing. He has a past medical history of hypertension and type 2 diabetes mellitus. His temperature is 36.6°C (97.9°F), pulse is 125/min, respirations are 30/min, and blood pressure is 90/60 mm Hg. Pulmonary examination shows diffuse wheezes in both lung fields. Cardiac examination shows a prominent "v" wave of the jugular vein and a 1/6 holosystolic murmur best heard on the left lower sternal border. Abdominal examination shows hyperactive bowel sounds.

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A tumor arising from neuroendocrine cells leading to excess secretion of serotoninhistamine, and bradykinin

  • Common primary sites include GI (small and large intestines, stomach, pancreas, liver), lungs, ovaries, and thymus
    • The most common site of a neuroendocrine (carcinoid) tumor to metastasize to is the liver
    • Carcinoid tumor of the appendix is the most common cause. The appendiceal cancer travels from the appendix then to the liver where it metastasizes to the lungs
  • Usually asymptomatic until liver metastasis; symptoms develop occasionally
    • GI tract tumor→ hormone secretion → enter into enterohepatic circulation → liver inactivates hormones → no symptoms
    • Liver tumor → hormone secretion → released into circulation + liver dysfunction → symptoms
  • Carcinoid syndrome (the hallmark sign) is actually quite rare and occurs in approximately 5% of carcinoid tumors and becomes manifest when vasoactive substances from the tumors enter the systemic circulation escaping hepatic degradation.
  • The syndrome includes flushing, ↑ intestinal motility (diarrhea), itching and less frequently, heart failure, vomiting, bronchoconstriction, asthma, and wheezing
    • ↑ Serotonin leads to collagen fiber thickening, fibrosis = heart valve dysfunction → tricuspid regurgitation, pulmonary stenosis/bronchoconstriction and wheezing
    • ↑ Histamine and bradykinin = vasodilation and flushing
    • ↑ serotonin synthesis → ↓ tryptophan → ↓ niacin/B3 synthesis = pellagra

CT-Scan to locate the tumors

  • Octreoscan → radiolabeled somatostatin analog (octreotide) binds to somatostatin receptors on tumor cells
  • Urinalysis → elevated 5-hydroxyindoleacetic acid (5-HIAA) → is the main metabolite of serotonin and is used to determine serotonin levels in the body
  • Pellagra (niacin/B3 deficiency) - ↑ serotonin synthesis → ↓ tryptophan → ↓ niacin/B3 synthesis
  • Chest X-Ray shows low-grade CA seen as pedunculated sessile growth in the central bronchi
  • Bronchoscopy- pink/purple central lesion, well-vascularized
Endobronchial carcinoid tumor in a Crohn disease patient

Endobronchial carcinoid tumor in a Crohn disease patient (centrally located sessile growth is common)

Treatment is by surgical excision and carries a good prognosis

  • The lesions are resistant to radiation therapy and chemotherapy
  • Octreotide - a somatostatin analog which binds the somatostatin receptors and decreases the secretion of serotonin by the tumor
  • Niacin supplementation

osmosis Osmosis
IM_MED_BronchialCarcinoidTumor_v1.1_ Lung carcinoma is typically divided into two categories, small cell carcinoma and non-small cell carcinoma. Non-small cell carcinoma subtypes include adenocarcinoma, squamous cell carcinoma, large cell carcinoma and carcinoid tumor. Bronchial carcinoid tumor is a neuroendocrine tumor that carries an excellent prognosis. It may be asymptomatic or may present with symptoms of bronchial obstruction such as coughing or hemoptysis. Occasionally, bronchial carcinoid tumors may cause carcinoid syndrome which is characterized by flushing, diarrhea, and wheezing. These tumors are chromogranin A positive and histology shows nests of neuroendocrine cells. Additionally, patients will have elevated urine 5-HIAA when carcinoid syndrome is present. Treatment includes surgical resection and octreotide for symptomatic relief.

Bronchial Carcinoid Tumor Picmonic

Question 1
A 39-year-old man comes to the emergency department because of a 3-week history of episodic cutaneous flushing, diarrhea, and wheezing. He has a past medical history of hypertension and type 2 diabetes mellitus. His temperature is 36.6°C (97.9°F), pulse is 125/min, respirations are 30/min, and blood pressure is 90/60 mm Hg. Pulmonary examination shows diffuse wheezes in both lung fields. Cardiac examination shows a prominent "v" wave of the jugular vein and a 1/6 holosystolic murmur best heard on the left lower sternal border. Abdominal examination shows hyperactive bowel sounds. Which other clinical findings is likely to be present?
A
Cheilosis and stomatitis
Hint:
Riboflavin is an essential vitamin used in the production of flavin adenine dinucleotide and flavin mononucleotide. Symptoms of riboflavin deficiency include stomatitis, cheilosis, anemia, and itchy eyes. Riboflavin deficiency is common in developing countries but rare in developed countries with riboflavin fortified grain and rice products.
B
Confusion, ophthalmoplegia, and ataxia
Hint:
Wernicke encephalopathy is caused by a deficiency in thiamine and is particularly common in alcoholics. Wernicke is defined by the classic triad of confusion, ophthalmoplegia, and ataxia. In patients suspected of Wernicke encephalopathy, it is crucial to administer thiamine before glucose is given.
C
Swollen gums, bruising, and hemarthrosis
Hint:
Scurvy is a disease caused by a deficiency in vitamin C. Symptoms include swollen and bleeding gums, easy bruising, hemarthrosis, and neuropathy. Failure to treat scurvy will eventually lead to death. Vitamin C is found in vegetables and fruits such as kale, oranges, and lemons.
D
Dermatitis and mental status change
E
Ptosis, anhydrosis, and miosis
Hint:
Horner syndrome is a constellation of symptoms as a result of a lesion to the sympathetic chain. Symptoms include ipsilateral ptosis, anhydrosis, and miosis. Etiologies include multiple sclerosis, central nervous system tumors, and Pancoast tumors.
Question 1 Explanation: 
Carcinoid syndrome is associated with serotonin secreting neuroendocrine cells. An overproduction of serotonin can lead to niacin deficiency and cause pellagra (diarrhea, dermatitis, and mental status changes).
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