What are the guidelines for managing pulmonary tumors in children?

Management of Pulmonary Tumors in Children

The provided evidence does not contain specific guidelines for managing pulmonary tumors in children—the available guidelines address pulmonary hypertension, bronchiectasis, and pulmonary embolism, which are distinct from pulmonary tumor management. However, based on the available research evidence on pediatric pulmonary metastases and primary lung tumors, I can provide evidence-based recommendations.

Surgical Management: The Cornerstone of Treatment

Complete surgical resection is the primary curative treatment for pulmonary tumors in children and should be pursued aggressively in carefully selected patients within a multidisciplinary oncology team. 1, 2

Patient Selection Criteria for Surgery

The following factors predict improved survival and should guide surgical candidacy:

• Number of metastases: Two or fewer pulmonary nodules is an independent predictor of survival (p=0.0004) 2
• Laterality: Unilateral disease confers significantly better outcomes than bilateral disease (p=0.001) 2
• Disease-free interval: DFI >2 years from primary tumor diagnosis predicts longer survival (p=0.003) 2
• Complete resectability: Ability to achieve complete resection is critical (p=0.004) 2
• Response to chemotherapy: Tumor regression with decreased size/number of metastases after induction chemotherapy is mandatory before considering surgery 3

Surgical Approaches and Techniques

Wedge resection should be the preferred surgical technique as it preserves maximum lung parenchyma while achieving oncologic control—75% of procedures in one series utilized wedge resection over anatomic resections 2, 4

Surgical access options include:

• Unilateral posterolateral thoracotomy for unilateral disease 4
• Bilateral thoracotomy (can be performed in single operation without increased complications in children) 3
• Thoracoscopic approach when technically feasible (utilized in 14% of cases) 1
• Sternotomy for bilateral anterior lesions 4

Perioperative Outcomes

Surgery for pediatric pulmonary tumors demonstrates excellent safety profile:

• Zero perioperative mortality in multiple series 2, 3, 4
• Mean hospital stay: 5 days 2
• Mean chest tube duration: 2.7 days 2
• Complications are minimal: pneumothorax requiring drainage (uncommon), prolonged air leak, fever 1, 4

Multidisciplinary Treatment Algorithm

Step 1: Initial Assessment

• Confirm histologic diagnosis of primary tumor 1
• Obtain high-resolution chest CT to characterize number, size, and location of pulmonary lesions 2
• Assess disease-free interval from primary tumor diagnosis 2

Step 2: Neoadjuvant Chemotherapy

• Administer tumor-specific induction chemotherapy 3, 4
• Reassess with imaging to document response (tumor regression, decreased size/number) 3
• Surgery should only proceed if chemotherapy response is demonstrated 3

Step 3: Surgical Resection

• Perform complete resection of all visible and palpable metastases (range 1-45 nodules can be resected) 3
• Prioritize wedge resection to preserve lung tissue 4
• Use anatomic resection (segmentectomy, lobectomy) only when necessary for complete resection 2, 4
• Pneumonectomy is rarely indicated (only 1 case in 43 patients in one series) 4

Step 4: Adjuvant Therapy

• All patients receive postoperative chemotherapy 4
• Radiotherapy administered selectively based on histology (37.2% of patients): indicated for Wilms' tumor, Ewing's sarcoma/PNET, and rhabdomyosarcoma 4

Step 5: Surveillance and Re-resection

• Systematic pulmonary follow-up is recommended to optimize postoperative rehabilitation and detect recurrence 1
• Pulmonary function testing should be performed systematically (currently underutilized—only 22/62 patients in one series) 1
• Re-thoracotomy for recurrent metastases is appropriate in selected patients—up to 5 repeat thoracotomies have been performed successfully 3

Tumor-Specific Considerations

Common primary tumors requiring pulmonary metastasectomy in children:

• Osteosarcoma (most common) 2, 3, 4
• Wilms' tumor 3, 4
• Ewing's sarcoma 2, 3, 4
• Hepatoblastoma 3, 4
• Rhabdomyosarcoma 3, 4
• Pleuropulmonary blastoma (most common primary lung tumor) 1

Long-Term Outcomes

Five-year survival after pulmonary metastasectomy is 25% overall, but reaches 74-80% in carefully selected patients with favorable prognostic factors 2, 4, 3

• Complete remission achieved in 80% of patients with bilateral/multiple metastases who underwent aggressive surgical approach after chemotherapy response 3
• Respiratory symptoms persist in 13% of children during follow-up (likely underestimated) 1
• Most survivors have no clinically relevant respiratory restrictions at mean follow-up of 49 months 3

Critical Pitfalls to Avoid

• Do not operate without documented chemotherapy response—tumor regression is mandatory before surgery 3
• Do not assume bilateral disease is inoperable—bilateral thoracotomy in single operation is safe in children and can achieve 80% complete remission 3
• Do not neglect pulmonary function monitoring—systematic respiratory follow-up and pulmonary function testing are underutilized but essential 1
• Do not perform incomplete resections—incomplete resection significantly worsens survival (p=0.004) 2