Complete resection of pulmonary inflammatory pseudotumors has excellent long-term prognosis

Objective Pulmonary inflammatory pseudotumor is an uncommon disease, often with a benign presentation. However, invasion of adjacent thoracic organs, local recurrence, and distant metastases have been described, and the best management strategy remains unclear. We present a single large institutional experience in patients with pulmonary inflammatory pseudotumor and propose guidelines for treatment of this patient population. Methods A retrospective study was performed to review all patients who underwent resection for pulmonary inflammatory pseudotumor between 1974 and 2007. Results A total of 25 patients were treated with pulmonary inflammatory pseudotumor at the Marie Lannelongue Hospital. The mean age was 33 years. Two patients were referred after an incomplete resection. One patient presented with cerebral metastasis. We performed a complete resection in all patients: wedge resection (n = 7), lobectomy (n = 6), sleeve arterial lobectomy (n = 1), lobectomy with thoracic inlet exenteration (n = 2), bilobectomy (n = 2), pneumonectomy with brain metastasectomy (n = 1), sleeve pneumonectomy (n = 2), sleeve main bronchus or tracheal resection (n = 2), wedge with sleeve main pulmonary artery resections (n = 1), and sleeve pneumonectomy with esophageal, aortic arch, and right pulmonary artery resection (n = 1). No adjuvant therapy was given to any patients. Postoperative 30-day mortality and morbidity rates were 4% and 8%, respectively. With a mean follow-up of 80 months (range 4–369 months, 100% follow-up), actuarial 10-year survival was 89%. One patient died of an extensive sarcomatous recurrence 2 years after surgery. Conclusion Pulmonary inflammatory pseudotumor is a malignant disease affecting young patients with local invasion, distant metastasis, local recurrence, and sarcomatous degeneration. A complete resection should always be performed at initial presentation because of its high likelihood of cure with aggressive management.