Abstract. Pulmonary pleomorphic carcinoma (PPC) is a rare pulmonary malignant tumor that has a more aggressive clinical course and a poorer prognosis compared with non-small cell lung cancer (NSCLC) due to its resistance to chemotherapy and radiotherapy. In patients with advanced or relapsed PPC, it has been reported that the response rate to chemotherapy regimens known to be effective in patients with NSCLC is only 0-17%. The present study reports the cases of two consecutive patients with advanced PPC who exhibited marked responses to chemotherapy with carboplatin plus paclitaxel chemotherapy and long-term survival without tumor progression. This suggests that carboplatin plus paclitaxel chemotherapy is a good option for the treatment of advanced PPC.
IntroductionPulmonary pleomorphic carcinoma (PPC) is a rare primary malignant tumor in which carcinomatous and sarcomatous elements occur. The occurrence of the disease is extremely rare, accounting for only 0.1-0.4% of all reported malignant lung tumors (1-4). Patients with PPC are reported to be predominantly male smokers with a mean age of 66 years (5-8). PPC generally exhibits an aggressive clinical course, and has a tendency to grow rapidly and invade adjacent structures in the early stage. The recurrence after surgical treatment is common, and systemic metastases are frequently observed in patients with PPC (8). The prognosis of PPC is significantly worse than the majority of other subsets of non-small cell lung cancer (NSCLC) due to its poorer response to chemotherapy and radiotherapy. The response rate to chemotherapy regimens commonly used for NSCLC is in the range of 0-17% (2,9) and the median survival reported for patients with PPC was 5-10 months (1,2,9). The present study reports two cases of advanced PPC that were successfully treated with carboplatin plus paclitaxel chemotherapy and/or radiotherapy.
Case reportCase 1. A 66-year-old male was admitted to the Otsu Red Cross Hospital (Otsu, Shiga, Japan) in May 2013, due to a persistent cough and dyspnea that had persisted for 10 days. The patient had previously undergone a surgical resection of the colon due to a gastrointestinal stromal tumor. The patient had also smoked two packs of cigarettes per day for 37 years. A performance status score of 1, according to the Eastern Cooperative Oncology Group (ECOG) scale (10), was recorded. Computed tomography (CT) of the chest showed a large tumor, measuring 100x80 mm, in the right lower lobe of the lung, with pleural effusion. An ultrasonography-guided percutaneous lung biopsy was performed, and the tumor specimen consisted of epithelioid and sarcomatous cells (Fig. 1A). On immunohistochemical staining, pankeratin was diffusely positive in the epithelioid and sarcomatous components (Fig. 1B). The patient was diagnosed with PPC based on the morphological findings of the tumor cells admixed with sarcomatoid and epithelioid cells. There was no evidence of metastasis to the other organs, corresponding to a diagnosis of clinical stage Ⅳ (cT4N0M1a) in the TNM cla...