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 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. A tumor arising from neuroendocrine cells → leading to excess secretion of serotonin, histamine, 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
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- 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
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| 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 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)
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| Pulmonary nodules |
Patient will present as → a 35-year-old female who was found to have a small (2.5 cm) pulmonary lesion on 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"
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 yr
- Calcification, smooth well-defined edges, suggests benign disease
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Back to PANCE Blueprint Pulmonary (10%)
