A retrospective study of 53 cases of resectable primary bronchopulmonary cancers associated with Pierre Marie syndrome

: The prognosis of primary lung cancer associated with hypertrophic osteopulmonary arthropathy is not well known. Between July 1973 adn August 1995, we cared for 53 consecutive patients with resectable non-small-cell lung cancer associated with osteoplumonary arthropathy. There were 51 men and 2 women, mean age 56 years. In 83% of the cases the lung cancer was revealed by hypertrophic osteopulmonary arthropathy. The tumor generally involved the right lung (n = 38) and the upper lobe (n = 35). There was no peripheral or central predominance. Complete tumoral resection was performed in 47 patients, incomplete resection in 4 and exploratory thoracotomy in 2. The main histologies were adenocarcinoma (50%) and squamous cell carcinoma (40%). Among the 51 resected tumors, 27 were grade I, 5 grade II, 17 grade III and 2 grave IV. Overall 5-year survival was 39%, reaching 51% for grade I, 40% for grade II, 27% for grade III and 0% for grade IV. The pulmonary manifestations of hypertrophic osteopulmonary arthropathy regressed within the first postoperative hours in all the patients whose tumor was resected and in 1 of the 2 patients who underwent exploratory thoracotomy. AT follow-up, the hypertropic pulmonary arthropathy had disappeared in all resected patients except 1 with a grade I tumor. Tumor recurrence was proven in 18 resected patients, 5 of whom also had recurrent osteopulmonary arthropathy. Our results suggest that primary lung cancer associated with hypertrophic pulmonary arthropathy has characteristic features and that prognosis is comparable with primary lung cancer alone.