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J Thorac Cardiovasc Surg 2008;136:1077-1079
© 2008 The American Association for Thoracic Surgery
Brief Communication |
a Department of Surgery, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
b Department of Pathology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
Received for publication October 10, 2007; revisions received December 18, 2007; accepted for publication December 22, 2007. * Address for reprints: Yung-Chie Lee, MD, PhD, Department of Surgery, National Taiwan University Hospital and National Taiwan University College of Medicine, No. 7, Chung-Shan S Rd, Taipei, Taiwan 100. (Email: yclee{at}ntuh.gov.tw).
Solitary fibrous tumor (SFT) of the pleura is an uncommon intrathoracic neoplasm. A middle-aged woman had progressive exertional dyspnea for 6 months, and eventually respiratory failure developed which necessitated ventilatory support. After examination, a giant tumor was found to occupy the whole left pleural cavity with chest wall distention and severe mediastinal shift to the right side. We completely resected this giant tumor by two separate thoracotomies. The pathologic examination revealed an SFT weighing 5.1 kg. Her respiratory condition improved markedly after the operation and she was discharged 23 days postoperatively.
A 51-year-old woman, a nonsmoker, was referred to our hospital with endotracheal intubation and mechanical ventilation owing to respiratory distress caused by a huge lesion occupying the left chest cavity. A large tumor had been discovered in the left side of the chest 3 years earlier during a physical examination, but she had ignored it because it was asymptomatic. Six months before admission, she had exertional dyspnea that she had treated with oxygen therapy without seeking medical intervention. Seventeen days before referral, the sudden onset of progressive dyspnea, drowsy consciousness, generalized edema, and eventually a faint occurred at home. She was sent to a district general hospital immediately, where emergency intubation was performed.
On physical examination, bilateral decreased breath sounds were noted without finger clubbing. Laboratory examination data were all within normal ranges, including a fasting sugar level of 107 mg/dL. Chest radiography (
Figure 1, A) and computed tomography (CT) (Figure 1, B) both revealed one huge tumor that occupied the whole left chest cavity with chest wall distention and shifting of the mediastinum to the right side. Echo-guided biopsy favored an SFT.
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Figure 2, A). Microscopic examination disclosed a typical patternless pattern with occasional mitosis (4/30 high-power fields) (Figure 2, B). With the positive immunoreactivity of CD-34 and vimentin, it was confirmed as an SFT with malignant potential. On the 23rd day after surgery, she was discharged in excellent condition without sequel. During follow-up, thalidomide was prescribed for prevention of recurrence. Seventeen months after the operation, there was no evidence of local recurrence on chest radiograph (
Figure 3). She was well without any respiratory symptoms.
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SFTs were first described by Klemperer and Rabin1 in 1931. The growths were initially termed localized mesothelioma because the tumor was believed to arise from mesothelial cells.2 However, according to immunohistochemical analysis, SFTs of the pleura express vimentin and CD-34, which are markers of mesenchymal cells, and do not express cytoplasmic keratins, which are found in mesotheliomas, indicating that these fibromas originate from the mesenchyme rather than from mesothelial cells.3
SFTs represent less than 5% of all neoplasms involving the pleura. Two thirds of these tumors arise from the visceral pleura, and one third arises from the parietal pleura.4 The etiology of SFTs is still unknown. The most frequent symptoms are dyspnea, cough, and chest pain. Paraneoplastic manifestations include arthritic pain, clubbed fingers, and hypoglycemia, but about 54% of the patients are asymptomatic. The tumors are noted by chest x-ray screening. By the time the tumors are found, their size can vary from centimeters to occupying the whole lung field. The SFTs on chest radiograph vary from a sharply delineated round or lobulated mass, with or without pleural effusion, to opacification of the complete hemithorax. Computed tomographic scanning and magnetic resonance imaging are important to evaluate the relationship of the tumor to neighboring structures and to evaluate the resectability of the tumor. Displacement rather than invasion of adjacent structures is a characteristic finding of SFTs on computed tomography or magnetic resonance imaging.5
Histologic and immunohistochemical analysis are the most important diagnostic measures. Surgical resection is needed. Fine needle aspiration is mostly inconclusive because of the combination of acellular and hypercellular portions. The microscopic picture is dominated by a "patternless pattern," with fibroblasts, collagen, and reticular fibers haphazardly arranged. Immunohistochemical study shows immunoreactivity for vimentin and CD-34. England, Hochholzer, and McCarthy6 listed criteria of malignant SFTs as followed: 4 mitotic figures in 10 high-power fields, hemorrhage, high cellularity, pleomorphism, and necrosis.
Most of these tumors have a benign clinical course if resected completely. However, according to the literature, even after complete resection, benign SFT still can result in local recurrence, malignant transformation, distant metastasis, and even death. On the contrary, even if the pathologic diagnosis is malignant SFT, cure may be effected by wide local resection. Because SFTs have the potential for adverse biological behavior, such as repeated recurrences or malignant transformation, the principle of treating this tumor is complete resection and followed by long-term follow-up.3
Oral thalidomide was prescribed because of our previous successful experience with thalidomide as an adjuvant therapy for patients with malignant SFT, margin-involved desmoids, and unresectable metastatic osteosarcoma. Thalidomide is postulated to have antiangiogenic activity through its ability to interfere with the vascular endothelial growth factor and basic fibroblast growth factor pathway.7
In summary, our patient had an exceptionally huge SFT weighing 5.1 kg that occupied the whole left thoracic cavity. To our knowledge, it is the biggest one ever reported in the literature. Thalidomide was prescribed for 9 months after the operation. There has been no recurrence or any sequel during 17 months' follow-up.
References
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