Case of the Month - Solitary Fibrous Tumor of the Pleura(SFTP)

 

Case History-

A 70 year old lady presented with worsening dyspnea over last 3 months. For the last 2 weeks she was unable to perform her daily chores.

She was evaluated by her GP and a chest x-ray and CT Chest were done (image below).

 

 

The chest X ray showed opacification of the left hemithorax with shifting of the mediastinal structures to the right.
The CT chest showed a large mass (17cm X 8cm X 7cm) occupying the anterior portion of the left hemithorax. It was of homogenous density with areas of calcification. The upper lobe of the lung was completely obliterated. The mass was abutting the left hilar vascualr structures without any evidence of invasion. The mediastinal structures were shifted to the right due to the mass effect.
A FNAC of the mass was performed at another hospital was neagative for malignant cells.

The patient was taken up for surgery after necessary work up. In view of the close proximity to the hilar structures, I decided to open the chest with the clamshell approach(B/L thoracotomy- figure below). The hilar structures (left pulmonary artery and vein) were controlled intrapericardially with vessel loops. The tumor was then dissected out. It was non adherent to the vascular structures and could be delivered out of the chest cavity after lysing flimsy adhesions to the chest wall and the lung. There was a vascular pedicle from the lingula region which was ligated and divided. The left uppel lobe of the lung which was compressed by the tumor was spared and expanded fully once ventilation was restored.
The tumor weighed 1.75kg (figure below).
The gross findings were suggestive of a solitary fibrous tumor of the pleura (SFTP) and the histopathology report was confirmatory. The tumor showed uniform spindle cells with connective tissue in between. There was no evidence of malignancy.

Clamshell Incision



Comment- Solitary fibrous tumor of the pleura is a mesenchymal tumor that has been increasingly recognised over the past few years. Majority of these tumors have a benign course cured by simple excision. About 12% have malignant course and may recur. The case above is a classical presentation of this tumor. The main differential diagnosis include - pleural mesothelioma, neurogenic sarcoma, synovial sarcoma, hemangiopericytoma, fibrosarcoma and malignant fibrous histiocytoma. Immunohistochemical staining can be used to confirm the diagnosis.