Objectives: Pleomorphic carcinoma of the lung, a rare malignant disease with a dual-cell component of spindle and/or giant cells, and of epithelial cells, was defined in the World Health Organization classification updated in 1999. Reported prognoses are heterogeneous, and optimal treatment remains undefined.
Methods: Data were retrospectively examined for 45 consecutive patients (41 men and 4 women) who had undergone surgical resection for pulmonary pleomorphic carcinoma.
Results: Sarcomatous elements were as follows: 23 spindle cell types (51.1%), 11 giant cell types (24.4%), and 11 combined spindle and giant cell types (24.4%). Epithelial components were adenocarcinoma in 25 (55.6%) patients, squamous cell carcinoma in 8 (17.8%) patients, and large call carcinoma in 12 (26.7%) patients. Nodal status was classified as pN0 disease in 28 (62.2%) patients, pN1 disease in 5 (11.1%) patients, and pN2 disease in 12 (26.7%) patients. Even patients with pN0 disease frequently manifested vascular invasion (16/28 [57.1%]). Five-year overall survival and disease-free survival were 39.2% and 47.1%, respectively. Although the subtype of epithelial components, as well as sarcomatous elements, did not affect prognosis, overall survival (P = .02) and disease-free survival (P = .002) in patients with pN1/N2 disease were significantly worse than those in patients with pN0 disease. Most recurrences occurred at distant sites (14/20 [70.0%]), and recurrence within 6 months after resection was found in 10 patients (10/20 [50.0%]). Moreover, median survival time after confirmation of initial relapse was 2.6 months.
Conclusions: Pulmonary pleomorphic carcinoma, which often presented in symptomatic male smokers as a large peripheral lesion, carried a poor prognosis, even when early-stage disease was diagnosed and resected. Distant metastases occurred more frequently and earlier, and the survival after relapse was very short, suggesting that this entity should be considered to have a tremendously aggressive malignant behavior. Further investigation of biologic features of pulmonary pleomorphic carcinoma and therapeutic response is a high-priority issue, so that suitable treatment strategies can be planned.