Should a patient with pulmonary neuroendocrine carcinoma be referred to both a pulmonologist and an oncologist?

Management of Pulmonary Neuroendocrine Carcinoma: Referral to Both Pulmonologist and Oncologist

Patients with pulmonary neuroendocrine carcinoma should be referred to both a pulmonologist and an oncologist, and should be discussed within a multidisciplinary tumor board with specialists who have expertise in neuroendocrine tumors to establish the most appropriate management. 1

Rationale for Dual Specialty Referral

Pulmonologist Involvement

• Respiratory assessment: Functional respiratory tests should always be carried out to assess surgical risk, evaluate for chronic obstructive airway disease, and screen for bronchostenosis 1
• Bronchoscopic evaluation: Bronchoscopy may be required for staging and assessment of central airway tumors preoperatively 1
• Airway management: For central tumors, pulmonologists can provide critical interventions:
  • Endobronchial treatments (laser resection, cryotherapy) for symptom control or as a bridge to surgery 1
  • Management of respiratory symptoms (cough, hemoptysis, dyspnea, wheezing) which are common in central tumors 1

Oncologist Involvement

• Systemic therapy planning: For advanced or metastatic disease, various treatment options require oncology expertise:
  • Somatostatin analogs for both symptom control and antiproliferative effects 1
  • Targeted therapies (e.g., everolimus) for progressive disease 1, 2
  • Chemotherapy regimens for aggressive metastatic disease 1
  • Peptide receptor radionuclide therapy (PRRT) for tumors with somatostatin receptor expression 1, 2

Diagnostic and Treatment Pathway

1. Initial Diagnosis:

   • Contrast CT is the gold standard imaging modality 1
   • Functional imaging with somatostatin receptor imaging (SRI) or FDG-PET depending on tumor grade 1

2. Staging Workup:

   • CT chest and abdomen for preoperative staging 1
   • Bronchoscopy for central tumors 1
   • Biochemical assessment (chromogranin A, specific hormonal markers if syndromic) 1

3. Treatment Planning:

   • Early-stage disease: Primarily surgical management

     • Pulmonologist: Pre-surgical respiratory assessment, possible bronchoscopic interventions
     • Oncologist: Evaluation for potential adjuvant therapy in high-risk cases

   • Advanced disease: Multimodal approach

     • Pulmonologist: Management of respiratory symptoms, airway interventions
     • Oncologist: Systemic therapy selection and management

4. Follow-up:

   • Long-term follow-up is essential for all patients with typical and atypical carcinoids 1
   • Surveillance schedule varies based on tumor type, with more frequent monitoring for atypical carcinoids 1

Special Considerations

• Carcinoid syndrome: Present in 2-5% of pulmonary carcinoids, requiring specific management with somatostatin analogs 1
• Ectopic hormone production: Some tumors may produce ACTH (Cushing's syndrome) or other hormones requiring specialized management 1
• Lung-preserving approaches: Parenchymal-sparing surgery should be prioritized when possible, requiring pulmonology expertise for assessment 1

Common Pitfalls to Avoid

• Delayed referral: Pulmonary neuroendocrine tumors can have indolent behavior, but early multidisciplinary management improves outcomes
• Inadequate follow-up: These tumors can recur even decades after initial treatment, necessitating long-term surveillance 1
• Misclassification: Accurate histopathologic diagnosis is critical but challenging, requiring specialized pathology expertise 3
• Single-specialty management: The complexity of these tumors requires input from both pulmonary and oncology specialists 1, 4

The management of pulmonary neuroendocrine carcinoma is complex and benefits greatly from the combined expertise of both pulmonology and oncology specialties working together in a coordinated fashion to optimize patient outcomes.