INTRODUCTION: Pleomorphic carcinoma of the lung is a rare malignant epithelial tumor that makes up only 0.3% to 1% of all malignant lung tumors and contains carcinomatous and sarcomatoid components. Due to its rarity few studies have been reported and its clinical and pathological features remain unclear. It is believed to spread at an early stage and to have an aggressive clinical course. We describe a case of this unique aggressive malignant tumor. The case will include chest imaging and the pathology slides (from pneumonectomy).
CASE PRESENTATION: A 60 year old cachectic male presented with cough, right side chest pain and weight loss. Physical examination revealed decreased breath sounds on the right side on auscultation. Initial laboratory analysis was essentially unremarkable. Chest x-ray revealed a 13x10 cm mass like density with smooth borders in the right side of the chest. Computed tomography (CT) of the chest showed a large heterogeneous mass with smooth margins in the right mid lung zone with areas of decreased attenuation consistent with necrosis. Internal intra lesional vessels were also identified. PET scan showed an area of malignant range FDG uptake in the right lower lung zone measuring up to 5.3SUV. No distant neoplastic range focal abnormalities were identified. Core biopsy obtained under CT guidance was inconclusive. Subsequently patient underwent right pneumonectomy with lymph node resection. Histopathology revealed a pleomorphic carcinoma with adenocarcinomatous, spindle cell, and clear cell features with extensive necrosis. Immunostaining showed the spindle cells were positive for both keratin and vimentin. No metastasis was found in the hilar or mediastinal lymph node. Pathologic stage was T2N0M0.
DISCUSSIONS: The recent WHO classification defines pleomorphic carcinoma as a poorly differentiated nonsmall cell lung cancer, namely squamous cell carcinoma, adenocarcinoma, or large cell carcinoma, containing spindle cells and/or giant cells, or carcinoma consisting only of spindle and giant cells. At least 10% of spindle cells, giant or both, should be present to classify a carcinoma as pleomorphic carcinoma. The actual nature of pleomorphic carcinoma has been debated extensively because of its rarity. It often presents in male smokers as large peripheral neoplasms with central low attenuation area and frequently invades the pleura and chest wall. Coughing and hemoptysis are the most common complaints although chest pain, dyspnea, fever have also been reported. Preoperative pathologic diagnosis in the small tissue samples is limited and diagnosis generally requires surgically resected samples. Biphasic appearance of the tumor also makes the diagnosis difficult and requires immunohistochemical staining. PC follows an aggressive clinical course with high local disease relapse rate after surgical resection and a high frequency of disseminated disease on initial diagnosis. There have been few reports regarding the use of chemotherapy and evidence of benefit of chemotherapy is also lacking. Advanced stage lymph node metastasis and histologically diagnosed massive coagulation necrosis (>25%) of the tumor predict poor disease free survival.
CONCLUSION: In conclusion, this rare disease should be considered to have a tremendously aggressive malignant behavior and a strict follow up observation must be continued in these patients. Further investigation of biologic features of pulmonary pleomorphic carcinoma and therapeutic operation is a high priority issue so that suitable treatment strategies can be planned. Unfortunately our patient developed brain metastasis and opted for hospice care.
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