PANCE Blueprint Pulmonary (10%)

Pulmonary Neoplasms (PEARLS)

The NCCPA™ PANCE Pulmonary Content Blueprint covers three types of pulmonary neoplastic diseases

Carcinoid tumors
Patient will present with → a 43-year-old man who comes to the emergency department because of a 3-week history of episodic cutaneous flushingdiarrhea, 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.

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) = Cutaneous flushing, diarrhea, wheezing and low blood pressure is actually quite rare and occurs in ~ 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

Treatment is by surgical excision and carries a good prognosis

  • The lesions are resistant to radiation therapy and chemotherapy
  • Octreotide - a somatostatin analog that binds the somatostatin receptors and decreases the secretion of serotonin by the tumor
  • Niacin supplementation
Lung cancer (ReelDx)
Patient will present as → a 65-year-old woman with a 40 pack-year history of smoking presents with a 7 kg weight loss over the last 3 months and recent onset of streaks of blood in the sputum. PE reveals a thin, afebrile woman with clubbing of the fingers, an increased anteroposterior diameter, scattered and coarse rhonchi and wheezes over both lung fields, and distant heart sounds.

ReelDx Virtual Rounds (lung cancer)

Lung cancer is classified into two major categories

  • Small cell lung cancer (SCLC), about 15% of cases (poor prognosis)
  • Non–small cell lung cancer (NSCLC), about 85% of cases, four subtypes include adenocarcinoma, squamous cell carcinoma, large cell carcinoma, and carcinoid tumor

⇒ Small Cell (15% of cases) - 99% are in smokers, does not respond to surgery, and metastases at presentation

  • Location: (central mass), very aggressive
  • Treatment: Combination chemotherapy needed
  • Paraneoplastic syndromes: Cushing's, SIADH

⇒ Non-Small Cell  (85% lung cancer cases)

  • Adenocarcinoma - most common (peripheral mass), 35-40% of cases of lung cancer
    • Most common
    • Associated with smoking and asbestos exposure
    • Location: Periphery
    • Paraneoplastic syndrome: Thrombophlebitis
  • Squamous cell (central mass) with hemoptysis, 25-35% of lung cancer cases
    • Location: Central
    • May cause hemoptysis
    • Paraneoplastic syndrome: hypercalcemia
    • Elevated PTHrp
  • Large cell - fast doubling rates - responds to surgery rare (only 5%)
    • Location: Periphery 60%
    • Paraneoplastic syndrome: Gynecomastia
  • Carcinoid tumor (1-2%): lack glandular and squamous differentiation
    • A tumor arising from neuroendocrine cells leading to excess secretion of serotoninhistamine, and bradykinin
      • GI tract carcinoid tumor may metastasize to the lung (CA of appendix = MC; appendix → liver → lung)
    • Presentation: hemoptysis, cough, focal wheezing, recurrent pneumonia
    • Carcinoid syndrome = cutaneous flushing, diarrhea, wheezing, hypotension (telltale sign)
    • Adenoma = MC type (slow-growing, rare)


Non-Small Cell can be treated with surgery

  • Treatment depends on staging:
    • Stage 1-2 surgery
    • Stage 3 Chemo then surgery
    • Stage 4 palliative
    • Carcinoid tumors are treated with surgery

Small Cell: CAN NOT be treated with surgery will need chemotherapy

Associated manifestations:

  • Superior vena cava syndrome (facial/arm edema and swollen chest wall veins)
  • Pancoast tumor (shoulder pain, Horner’s syndrome, brachial plexus compression)
  • Horner’s syndrome (unilateral miosis, ptosis, and anhidrosis)
  • Carcinoid syndrome (flushing, diarrhea, and telangiectasia)
Pulmonary nodules
Patient will present as → a 35-year-old female who was found to have a small (2.5 cm) pulmonary lesion on a chest radiograph found incidentally after a screening exam for a positive PPD at work. The patient has no significant past medical history and is asymptomatic.

< 3 cm is a nodule (coin lesion) > 3 cm the lesion is considered a "mass"

  • Smooth well-defined edges are more likely to be benign
  • Ill-definedlobular, or spiculated suggests cancer
  • Pulmonary nodules are also known as coin lesions

When managing pulmonary nodules we follow the Fleischner Society pulmonary nodule recommendations

Steps to dealing with a pulmonary nodule:

  • Incidental finding on CXR →
  • Send for CT →
  • If suspicious (depending on radiographic findings below) will need a biopsy
    • Ill-defined lobular or spiculated suggests cancer
  • If not suspicious < 1 cm it should be monitored at 3 mo., 6 mo., and then yearly for 2 yrs.
    • Calcification, smooth, well-defined edges, suggests benign disease
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