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Chapter 83: Neoplasms of the Lung

Chapter 83 | Part 4: Oncology and Hematology

KEY CLINICAL POINTS

  • Lung cancer is the leading cause of cancer-related mortality globally, with ~1.8 million deaths annually. Smoking remains the primary risk factor, accounting for ~85% of cases.
  • The TNM staging system (8th edition) is critical for determining treatment strategies, with stage I NSCLC having a 5-year survival rate of ~63% with surgical resection.
  • Immunotherapy (e.g., pembrolizumab, atezolizumab) has transformed treatment for PD-L1-positive NSCLC, with significant survival benefits in stage III and IV disease.
  • Targeted therapies (e.g., EGFR TKIs, ALK inhibitors) are effective in specific molecular subtypes, with response rates up to 80% in EGFR-mutant NSCLC.
  • SCLC is highly aggressive with rapid progression, treated with concurrent chemoradiation for limited-stage disease and platinum-based chemotherapy for extensive-stage disease.

1. DEFINITION & OVERVIEW

Lung cancer encompasses malignancies arising from the respiratory epithelium, including small-cell lung cancer (SCLC) and non-small-cell lung cancer (NSCLC). NSCLC includes adenocarcinoma, squamous cell carcinoma, and large-cell carcinoma. SCLC is characterized by rapid growth, early metastasis, and responsiveness to chemotherapy.

Table 83-1: Genetic Alterations with Existing Therapies in NSCLC

GENE ALTERATION FREQUENCY IN NSCLC (%) TYPICAL HISTOLOGY
ALK Rearrangement 3–7% Adenocarcinoma
ALK Mutation 1–3%
EGFR Mutation 10–35% Adenocarcinoma
EGFR Mutation 2–4%
KRAS Mutation 15–25% Adenocarcinoma
KRAS Amplification 2–4%
MET Amplification 2–4% Adenocarcinoma
ROS1 Rearrangement 1–2% Adenocarcinoma
RET Rearrangement 1–2% Adenocarcinoma
NTRK Rearrangement 1–2% Adenocarcinoma

1.1 Histopathologic Classification

NSCLC is classified into four main types: adenocarcinoma (most common in never-smokers), squamous cell carcinoma (linked to tobacco use), large-cell carcinoma, and neuroendocrine tumors (SCLC). Adenocarcinoma is increasingly common in never-smokers and women.

1.2 Molecular Subtypes

Key molecular drivers include EGFR mutations (10–15% of NSCLC), ALK rearrangements (3–7% of NSCLC), KRAS mutations (15–25% of NSCLC), and ROS1 fusions (1–2% of NSCLC).

2. EPIDEMIOLOGY

Lung cancer is the leading cause of cancer mortality globally, with ~1.8 million deaths in 2020. Incidence peaks in individuals aged 65–74 years. Smoking accounts for ~85% of cases, with 10–15% of patients having a family history of lung cancer. Never-smokers account for ~15% of cases, with adenocarcinoma being more common in this group.

Table 83-2: Benefits and Harms of LDCT Screening

LDCT CXR
4 in 1000 fewer died from lung cancer 13 in 1000
5 in 1000 fewer died from all causes 70 in 1000
223 in 1000 had at least 1 false alarm 365 in 1000
18 in 1000 had a false alarm leading to invasive procedure 25 in 1000
2 in 1000 had a major complication from invasive procedure 3 in 1000

2.1 Demographics

Incidence is higher in males (235,000 new cases in 2024) and increases with age, peaking at 70–74 years. African Americans have higher age-adjusted rates than Caucasians. Never-smokers account for ~15% of cases, with adenocarcinoma being more common.

2.2 Screening

LDCT screening reduces lung cancer mortality by 20% in high-risk populations (55–74 years, ≥ 30 pack-years smoking history). The NLST demonstrated a 20% reduction in mortality with LDCT compared to CXR.

3. ETIOLOGY & PATHOPHYSIOLOGY

Tobacco smoke is the primary cause, with ~85% of cases linked to smoking. Environmental carcinogens (asbestos, radon) and genetic factors (TP53, RB1 mutations) also contribute. Molecular mechanisms include driver mutations (EGFR, KRAS, ALK) and epigenetic changes.

Table 83-3: Presenting Signs and Symptoms of Lung Cancer

SYMPTOM AND SIGNS RANGE OF FREQUENCY
Cough 8–75%
Weight loss 0–68%
Dyspnea 3–60%
SYMPTOM AND SIGNS RANGE OF FREQUENCY
Chest pain 20–49%
Hemoptysis 6–35%
Bone pain 6–25%
Clubbing 0–20%
Fever 0–20%
Weakness 0–10%
Superior vena cava obstruction 0–4%
Dysphagia 0–2%
Wheezing and stridor 0–2%

3.1 Genetic Mutations

Key mutations include EGFR (exon 19 deletion, L858R), KRAS (G12C), ALK rearrangements, and ROS1 fusions. These drive tumor growth and are targets for therapy.

3.2 Paraneoplastic Syndromes

Common syndromes include hypercalcemia (PTH-related), SIADH, and Cushing’s syndrome. These are more prevalent in SCLC and can mimic metastatic disease.

4. CLINICAL FEATURES

Most patients present with advanced disease (stage III/IV). Common symptoms include cough, hemoptysis, weight loss, and dyspnea. Paraneoplastic syndromes (e.g., SIADH, Cushing’s) are common in SCLC.

Table 83-4: Clinical Findings Suggestive of Metastatic Disease

SYMPTOMS ELICITED IN HISTORY CLINICAL FINDINGS ON PHYSICAL EXAMINATION
Constitutional: weight loss >10 lb Lymphadenopathy (>1 cm)
Musculoskeletal: pain Hoarseness, superior vena cava syndrome
Neurologic: headaches, syncope, seizures, extremity weakness, recent change in mental status Bone tenderness, hepatomegaly (>13 cm span)
Focal neurologic signs, papilledema
Soft-tissue mass

4.1 Localized Disease

Stage I/II NSCLC presents with localized tumors. Stage III involves regional lymph node involvement. Stage IV has distant metastases (bone, brain, liver).

4.2 Metastatic Disease

Metastases to bone, brain, liver, and adrenal glands are common. Brain metastases occur in ~10% of patients, often in SCLC. Skeletal metastases cause pain and pathologic fractures.

5. DIAGNOSTIC APPROACH

Diagnosis requires tissue sampling (biopsy, cytology) and imaging (CT, PET). Biomarker testing (EGFR, ALK, ROS1) guides targeted therapy. PD-L1 expression determines immunotherapy eligibility.

Table 83-5: TNM Staging System for Lung Cancer (8th Edition)

PRIMARY TUMOR (T) NODES (N) METASTASES (M)
T1: £3 cm N0: No regional lymph node metastases M0: No distant metastasis
T1mi: Minimally invasive adenocarcinoma N1: Ipsilateral peribronchial/hilar lymph nodes M1a: Separate tumor nodule(s) in contralateral lobe
T2: >3 cm but £5 cm N2: Mediastinal/subcarinal lymph nodes M1b: Single distant metastasis
T3: >5 cm but £7 cm N3: Contralateral mediastinal/hilar lymph nodes M1c: Multiple distant metastases
T4: >7 cm or invasion of mediastinum, heart, great vessels N3: Contralateral mediastinal/hilar lymph nodes M1: Distant metastasis

5.1 Imaging

LDCT is preferred for screening. PET-CT improves staging accuracy. MRI is essential for brain metastases. Bone scans detect skeletal metastases.

5.2 Biomarker Testing

Next-generation sequencing (NGS) identifies actionable mutations. PD-L1 testing ( ≥ 1% or ≥ 50%) guides immunotherapy selection.

6. MANAGEMENT & TREATMENT

Treatment depends on stage and molecular profile. Early-stage NSCLC is managed with surgery (lobectomy) and adjuvant chemotherapy. Advanced disease uses immunotherapy, targeted therapy, or chemoradiation.

Table 83-6: TNM Stage Groupings, 8th Edition

T/M SUBCATEGOR Y N0 N1 N2 N3
T1a IA1 IIB IIIB IIIA IIIB
T1b IA2 IIB IIIB IIIA IIIB
T1c IA3 IIB IIIB IIIA IIIB
T2a IB IIB IIIB IIIA IIIB
T2b IIA IIB IIIA IIIB IIIC
T3 IIB IIIA IIIB IIIC IIIC
T4 IIIA IIIA IIIB IIIC IVB
M1a IVA IVA IVA IVA IVB
M1b IVA IVA IVA IVA IVB
M1c IVB IVB IVB IVB IVB

6.1 Early-Stage NSCLC

Surgical resection (lobectomy) is the standard for stage I/II. Adjuvant chemotherapy (e.g., platinum-based) improves survival. Radiation may be used for inoperable cases.

6.2 Advanced NSCLC

First-line treatment includes immunotherapy (pembrolizumab, atezolizumab) for PD-L1 ≥ 50%, or chemotherapy with immunotherapy for PD-L1 ≥ 1%. Targeted therapy for EGFR/ALK mutations.

7. PROGNOSIS & COMPLICATIONS

5-year survival for stage I NSCLC is ~63% with surgery. Prognosis worsens with advanced stage. Complications include treatment-related toxicity (e.g., pneumonitis, myelosuppression), paraneoplastic syndromes, and metastatic disease.

Table 83-7: Assessment of Risk of Cancer in Patients with Solitary Pulmonary Nodules

VARIABLE LOW RISK INTERMEDIATE RISK HIGH RISK
Diameter (cm) <1.5 1.5–2.2 ‡2.3
Age (years) <45 45–60 >60
Smoking status Never smoker Current smoker (<20 cigarettes/d) Current smoker (>20 cigarettes/d)
Smoking cessation Quit ‡7 years ago Quit <7 years ago Never quit
Characteristics of nodule margins Smooth Scalloped Spiculated

7.1 Survival Rates

Stage I: 63% 5-year survival. Stage II: 50–60%. Stage III: 15–30%. Stage IV: <10%.

7.2 Treatment Toxicity

Chemotherapy: myelosuppression, mucositis. Radiation: esophagitis, pneumonitis. Immunotherapy: immune-related adverse events (e.g., colitis, pneumonitis).

8. SPECIAL CONSIDERATIONS

Pregnancy, pediatric, and elderly populations require tailored approaches. Never-smokers and women have distinct clinical profiles. SCLC has a poor prognosis with limited treatment options.

Table 80-1: Phase 3 Trials of EGFR TKIs in EGFR-Positive NSCLC

TRIAL THERAPY NO. OF PATIENTS ORR (%) PFS (MONTHS)
IPASS Cisplatin/paclitaxel 129 47 6.3
IPASS Gefitinib 132 71 9.3
OPTIMAL Cisplatin/gemcitabin e 72 36 4.6
OPTIMAL Erlotinib 82 83 13.1
WJTOG3405 Cisplatin/docetaxel 89 31 6.3
WJTOG3405 Gefitinib 88 62 9.2
LUX-LUNG 3 Cisplatin/paclitaxel 115 23 6.9
TRIAL THERAPY NO. OF PATIENTS ORR (%) PFS (MONTHS)
LUX-LUNG 3 Afatinib 230 56 11.1
LUX-LUNG 6 Cisplatin/gemcitabin e 122 23 5.6
LUX-LUNG 6 Afatinib 242 67 11.0
ARCHER 1050 Gefitinib 225 72 9.2
ARCHER 1050 Dacomitinib 227 75 14.7
FLAURA Erlotinib or gefitinib 277 76 16.7
FLAURA Osimertinib 279 80 17.2
FLAURA2 Osimertinib/chemoth erapy 279 83 25.5
FLAURA2 Osimertinib 278 76 16.7
MARIPOSA Amivantamab/lazerti nib 429 86 23.7
MARIPOSA Osimertinib 429 85 16.6

8.1 Never-Smokers

Adenocarcinoma is more common in never-smokers. Molecular testing is critical for targeted therapy.

8.2 SCLC in Elderly

Elderly patients with SCLC have higher mortality and are often treated with palliative chemotherapy.

9. KEY POINTS & CLINICAL PEARLS

  1. Lung cancer is the leading cause of cancer mortality globally, with smoking as the primary risk factor. 2. Early detection with LDCT reduces mortality by 20% in high-risk populations. 3. Molecular testing is critical for selecting targeted therapies (EGFR, ALK, ROS1). 4. Immunotherapy improves survival in PD-L1-positive NSCLC. 5. SCLC is highly aggressive with poor prognosis, requiring concurrent chemoradiation for limited-stage disease.