Clinical characteristics of pleomorphic carcinoma of the lung
Introduction
According to the World Health Organization classification of lung tumors, pleomorphic carcinoma of the lung is one of five subgroups of sarcomatoid carcinoma [1], which itself is defined as a group of poorly differentiated tumors characterized pathologically by a combination of epithelial and mesenchymal elements. Pleomorphic carcinoma is histologically defined as either non-small cell carcinoma combined with neoplastic spindle and/or giant cell or a carcinoma that consists of only spindle cell and giant cell. At least 10% of the neoplasm should be pleomorphic.
If the tumor consists of only spindle cells or only giant cells, it is defined as a spindle cell or giant cell carcinoma, respectively, which are other subgroups of sarcomatoid carcinoma. Carcinosarcoma, which contains carcinoma and sarcoma, is another subgroup. Sarcoma components differentiate to osteosarcoma, chondrosarcoma, and rhabdomyosarcoma. Bone, cartilage, muscle, fat, and neuron are sometimes detected pathologically in tumors. Pulmonary blastoma is a very rare type of sarcomatoid carcinoma characterized as biphasic tumor containing a primitive epithelial component resembling well-differentiated fetal-type adenocarcinoma and a primary mesenchymal stroma.
Since its diagnostic criteria were confirmed, pulmonary pleomorphic carcinoma has been diagnosed more frequently. It is essential to understand its clinical behavior for effective management of patients with this disease. However, as pulmonary pleomorphic carcinoma is rare (only 0.1–0.4% of all malignant tumors of the lung) [2], [3], [4], its clinical and pathological characteristics are not well known. We have retrospectively investigated 22 patients with pulmonary pleomorphic carcinoma diagnosed by surgical resection, autopsy, or transbronchial biopsy (TBB). Radiological findings, results of positron emission tomography with 18-fluorodeoxy-glucose (FDG-PET), treatment, and clinical course are described in this report.
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Patients
We retrospectively analyzed 22 pulmonary pleomorphic carcinomas from a total of 2447 primary lung cancers (including 1022 cases of resected lung cancer) that we experienced between January 2005 and December 2008 at seven institutes (Hokkaido University Hospital, National Hospital Organization Hokkaido Cancer Center, Fukushima Medical University School of Medicine, National Hospital Organization Dohoku National Hospital, Hokkaido Social Insurance Hospital, Hokkaido Chuo Rosai Hospital, and KKR
Patient characteristics
Patient characteristics are summarized in Table 1. The male:female ratio was 19:3. The age at diagnosis was 68.3 ± 10.1 years (range 51–94). Symptoms were seen in 16 patients (72.7%): common symptoms were hemoptysis (7 cases) and cough (6 cases). Fever, pain, dyspnea and body weight loss were also seen. Six patients (27.3%) had no symptoms but were referred to hospital with lung nodules detected on chest X-rays. Eighteen patients (81.8%) were current- or ex-smokers, and the remaining 4 (18.2%)
Discussion
Lung tumors have been reclassified by the World Health Organization. In 1999, a group named “carcinoma with pleomorphic, sarcomatoid, or sarcomatous elements” was defined. In 2004, this group, which contained pleomorphic carcinoma, was renamed “sarcomatoid carcinoma” [1]. Travis et al. had previously reported pleomorphic carcinoma of the lung to be very rare (approximately 0.1–0.4% of all lung malignancies) [2], [3], [4]. Since its pathological definition became widely recognized, pleomorphic
Conclusion
In conclusion, we retrospectively analyzed 22 cases of pulmonary pleomorphic carcinoma in a total of 2447 cases of primary lung cancer. Pulmonary pleomorphic carcinoma had strong malignant potential with frequent distant metastases. We demonstrated that surgical treatment and appropriate follow-up therapy including the use of a molecular targeting drug (e.g., gefitinib for patients with adenocarcinoma as carcinomatous component) might improve outcomes. Some other important findings regarding
Conflict of interest
The authors have no conflict of interest to declare.
Acknowledgements
We are grateful to Dr. Koichi Yamazaki, former associate professor of the First Department of Medicine, Hokkaido University School of Medicine, for his outstanding support. We also thank Dr. Yoshihiro Matsuno (Hokkaido University Hospital), Dr. Katsushige Yamashiro (Hokkaido Cancer Center), Dr. Kazuo Watanabe (Fukushima Medical University Hospital), Dr. Yuichiro Fukasawa (KKR Sapporo Medical Center), and Dr. Kenzo Okamoto (Hokkaido Chuo Rosai Hospital), for valuable assistance in pathological
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