Standard Treatment Options for Superior Sulcus Tumors (T3, N0 or N1, M0)
Standard treatment options for superior sulcus tumors include the following:
- Radiation therapy alone.
- Radiation therapy and surgery.
- Concurrent chemotherapy with radiation therapy and surgery.
- Surgery alone (for selected patients).
NSCLC of the superior sulcus, frequently termed Pancoast tumors, occurs in less than 5% of patients.[37,38] Superior sulcus tumors usually arise from the apex of the lung and are challenging to treat because of their proximity to structures at the thoracic inlet. At this location, tumors may invade the parietal pleura, chest wall, brachial plexus, subclavian vessels, stellate ganglion, and adjacent vertebral bodies. However, Pancoast tumors are amenable to curative treatment, especially in patients with T3, N0 disease.
Adverse prognostic factors include the presence of mediastinal nodal metastases (N2 disease), spine or subclavian-vessel involvement (T4 disease), and limited resection (R1 or R2).
Radiation therapy alone
While radiation therapy is an integral part of the treatment of Pancoast tumors, variations in dose, treatment technique, and staging that were used in various published series make it difficult to determine its effectiveness.[37,38]
Small, retrospective series of radiation therapy in patients who were only clinically staged have reported 5-year survival rates of 0% to 40%, depending on T stage, total radiation dose, and other prognostic factors. Induction radiation therapy and en-bloc resection was shown to be potentially curative.
Evidence (radiation therapy):
- In the preoperative setting, a dose of 45 Gy over 5 weeks is generally recommended, while a dose of approximately 61 Gy is required when using definitive radiation therapy as the primary modality.[37,38]
- Retrospective case series have reported complete resection was achieved in only 64% of T3, N0 tumors and 39% of T4, N0 tumors.
Evidence (chemoradiation therapy):
- Two large, prospective, multicenter phase II trials have evaluated induction chemoradiation therapy followed by resection.[40,41]
- In the first trial (NCT00002642), 110 eligible patients were enrolled with mediastinoscopy negative, clinical T3-4, N0-1 tumors of the superior sulcus. Induction treatment was two cycles of etoposide and cisplatin with 45 Gy of concurrent radiation therapy.
- The induction regimen was well tolerated, and only five participants had grade 3 or higher toxic effects.
- Induction chemoradiation therapy could sterilize the primary lesion. Induction therapy was completed by 104 patients (95%). Of the 95 patients eligible for surgery, 88 (80%) underwent thoracotomy, two (1.8%) died postoperatively, and 83 (76%) had complete resections.
- Pathologic complete response or minimal microscopic disease was seen in 61 (56%) resection specimens. Pathologic complete response led to better survival than when any residual disease was present (P = .02).
- Five-year survival was 44% for all patients and 54% after complete resection, with no difference between T3 and T4 tumors. Disease progression occurred mainly in distant sites.
- In the second trial, 75 patients were enrolled and treated with induction therapy with mitomycin C, vindesine, and cisplatin combined with 45 Gy of radiation therapy. Fifty-seven patients (76%) underwent surgical resection, and complete resection was achieved in 51 patients (68%).
- There were 12 patients with pathologic complete response.
- Major postoperative morbidity, including chylothorax, empyema, pneumonitis, adult respiratory distress syndrome, and bleeding, was observed in eight patients. There were three treatment-related deaths.
- The disease-free and OS rates at 3 years were 49% and 61%, respectively; at 5 years, they were 45% and 56%, respectively.[Level of evidence: 3iiiDi]