PANCE Blueprint Pulmonary (10%)

• Cancer 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.

Presents with hemoptysis, cough, focal wheezing or recurrent pneumonia, carcinoid syndrome (the hallmark sign) is actually quite rare.

Most common type: Adenoma - Slow growing, rare metastasis

Presentation:

• Carcinoid syndrome = Cutaneous flushing, diarrhea, wheezing and low blood pressure. This is the hallmark sign!

Diagnostics studies:

• Bronchoscopy- pink/purple central lesion, well-vascularized. Pedunculated or sessile
• Elevated 5-HIAA

Treatment: Surgical removal

• 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% 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 serotonin, histamine, and bradykinin
    • GI tract carcinoid tumor may metastasize to 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)

Treatment:

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)

Steps to dealing with a pulmonary nodule:

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