A 76-year-old Caucasian female never smoker with no previous pulmonary history was referred to our institution for evaluation of a biopsy proven 17 cm fibrous tumor involving the left hemithorax. The patient related of a two-month history of dyspnea, lack of energy, poor appetite, and 10-pound involuntary weight loss. She denied any fevers, chills, sweats, or hemoptysis. As part of her evaluation, she had a chest X-ray and a computed tomography (CT) scan of the chest that revealed a bulky 16.7 × 12.8 cm × 10.1 cm heterogeneous mass occupying the left hemithorax with a mass effect involving the left side of the anterior mediastinum (Fig. 1). The mass abuts the proximal aortic arch, main pulmonary artery, left pulmonary artery, and left pulmonary vein with mass effect on the pulmonary vasculature. There was no evidence of osseous erosion or abnormal calcification. Also present was a moderate sized left pleural effusion. CT of the chest with intravenous contrast revealed abutment of the tumor to the mediastinum and chest wall without frank invasion (Fig. 2A). Positron emission tomography (PET) scan revealed patchy moderate hypermetabolism with a maximum standardized uptake value (SUV) of 6.7 (Fig. 2B). There were hypoattenuating areas within the mass with central photopenic defect compatible with central necrosis and/or hemorrhage. There was also uptake at the region of her left vocal cord with an SUV of 8.6. She underwent a CT guided core biopsy of the mass with pathology showing a SFT with proliferation of spindle cells in hypo and hyper-cellular areas with a collagenous stroma and foci. By immunochemistry, the spindled cells were positive for CD35, BCL-2, CD99, and negative for S-100, AE1/3, and CAM 5.2. Diagnostic thoracentesis was negative for neoplasia. Her past medical history was significant for craniotomy with resection of a hypoglossal neuroma with resultant left cranial nerve palsy involving cranial nerves 8–12 and tracheotomy. She was evaluated by otolaryngology, and endoscopic examination revealed left vocal cord paralysis which was described as chronic and previous Teflon injection. No tumor was identified.
She was taken to the operative suite. Flexible bronchoscopy revealed no evidence of endobronchial tumor. There was extrinsic compression involving the lingular bronchus and left lower lobe bronchus. She underwent a left muscle sparing lateral thoracotomy. Operative findings revealed a large, bulky, firm, well-circumscribed, vascularized tumor involving the left hemithorax with compressive atelectasis of the left lung (Figs. 3, 4, 5). This was associated with a bloody pleural effusion totaling 1 L. The tumor occupied the anterior and middle mediastinum and was firmly adherent to the left upper lobe lung parenchyma and to the pericardium anterior to the left phrenic nerve. There was no evidence of invasion of the mediastinum. The tumor’s blood supply originated from a branch of the left internal thoracic artery. The patient underwent complete resection of the mass en bloc with portions of the left upper lobe, lingula, and pericardium with ligation of the branch of the left internal thoracic artery (feeding vessel), and thoracic lymphadenectomy. Post resection, the left lung expanded fully.
Cytology of the pleural fluid revealed benign mesothelial cells. Tumor pathology revealed a SFT with atypical features with a mitotic rate of 12/10 high-power field. There was no metastasis to the mediastinal lymph nodes.
There was no lymph or vascular invasion. The tumor was ungraded. 1% of the tumor showed necrosis. All resected margins were free of tumor. No gene mutations were detected in the following genes: KRAS, NRAS. Immunostains of the tumor cells were positive for CD34, CD99, and BCL-2, and were negative for AE1/3, CAM 5.2, S-100 protein and desmin. Her postoperative course was uneventful. She was discharged home on postoperative day five with marked relief of her dyspnea. The case was presented at the multidisciplinary thoracic tumor board. It was recommended that no adjuvant therapy was warranted. The consensus of the board was that the patient should be monitored with a chest CT scan at six-month intervals. 18 months post procedure, she developed a 3.3 cm × 1.7 cm soft tissue tumor along the left internal thoracic artery lymph node chain, a 1.1 cm pre-carinal lymph node, a 1 cm subcarinal lymph node, and a sub-centimeter right and left hilar lymph node. PET scan revealed hypermetabolic uptake in the tumor and lymph nodes with SUV’s ranging between 3.6 and 4.2. CT guided biopsy of the left internal thoracic artery lymph node revealed metastatic fibrous tumor. Tumor markers were identical to that of the resected specimen. She is currently being treated with bevacizumab and temozolomide.