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

DX:

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
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.

Lung cancer is classified into 2 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% 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 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)
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:

  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
Tuberculosis (ReelDx + Lecture) (Prev Lesson)
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