Uniquely Lethal: Pulmonary Sarcomatoid Carcinoma, a Case Report

Abstract

INTRODUCTION: Malignant neoplasms are the third leading cause of mortality in the Philippines. Lung cancer is the leading cause of cancer-related mortality throughout the world. However, there are variants to this disease that are rarely seen. Presented is a case of a 75 year old male who was diagnosed with pulmonary sarcomatoid carcinoma at the Philippine Heart Center. CASE PRESENTATION: A 75 year old male, heavy smoker, presented 6 months prior with non-productive cough, chest x-ray showed a nodule on the left upper lobe. Persistence of cough, associated with anorexia and weight loss prompted consult. Upon admission, chest x-ray showed a mass on the left upper lobe. Chest CT scan with contrast was done which revealed a left upper lobe mass with surrounding lymphangitic spread and nodules on the left hilar and right lower lobe. Consideration was malignancy. CT guided fine needle aspiration biopsy of the left upper lobe mass was done. Metastatic work-ups were requested. Cranial CT scan showed enhancing nodules in the subcortical and cortical region of the left frontal lobe. Whole abdominal CT scan revealed enhancing nodules in the liver, bilateral adrenal glands, bilateral subcutaneous abdominal wall, and left gluteal region, also indicative of metastasis. Histopathologic findings were cytomorphologic features and immunohistochemical profile consistent with Sarcomatoid Carcinoma, of Lung Primary. Patient was diagnosed as stage IV disease and palliative treatment was recommended. Patient underwent whole brain radiation therapy and was started on chemotherapy with Gemcitabine then discharged. DISCUSSION: Pulmonary sarcomatoid carcinomas are poorly differentiated non-small cell carcinomas that contain at least 10% of sarcoma or sarcoma-like elements. These tumors are believed to arise from epithelial to mesenchymal transition of cells and and represent only 0.1% to 0.4% of all cases of non-small cell lung cancer. There is no record of a previous case of sarcomatoid carcinoma in our center. Average age of diagnosis for these patients is about 60 years of age, with a male preponderance of 4:1. The disease is noted to be more prevalent in patients with history of smoking. It is said to present more commonly as a mass in the upper lobes, as was noted in our patient. Median size is 4.5 cms, but diameters of up to 19 cms have been noted. They can invade adjacent structures by forming large necrotic and hemorrhagic masses and can metastasize to conventional sites such as the brain bone adrenals and liver, but may also include unusual locations such as the skin, as noted in our patient, as well as the esophagus, stomach, pancreas and heart. Treatment of localized disease relies on surgical resection, with adjuvant chemotherapy and radiation for patients with bulky tumors, or nodal involvement. Those with stage III or stage IV sarcomatoid carcinomas are managed non-operatively and given palliative treatment. However, cases show consistent poor response to systemic chemotherapy. These patients are said to have poorer prognosis compared with conventional NSCLCs, with a 5-year survival of only 24.5% versus 46.5% for other NSCLC. Future goals may be to target the epithelial mesenchymal transition pathways of these sarcomatoid carcinomas to eliminate surviving cancer cells in order to prevent recurrence and improve survival. CONCLUSIONS: Pulmonary sarcomatoid carcinoma is a rare variant of non-small cell lung carcinoma, seen to have a sarcomatoid component. It commonly presents as advanced disease, and combined with aggressive metastases, as well as poor response to conventional chemotherapeutic agents, makes it a uniquely lethal disease and thus leads to poorer outcomes in our patients. Our only hope is to better understand the epithelial-mesenchymal transition pathways of these cancer cells and hopefully develop targeted therapies, that will improve the survival for these patients.