|Year : 2013 | Volume
| Issue : 2 | Page : 260-262
Giant fibrous tumours of the pleura with mucus secretion
L Huang, Y Chai
Department of Thoracic Surgery, 2nd Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
|Date of Acceptance||11-Jan-2012|
|Date of Web Publication||6-Apr-2013|
Department of Thoracic Surgery, 2nd Affiliated Hospital, School of Medicine, Zhejiang University, 88 Jiefang Rd, Hangzhou
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Solitary fibrous tumors of the pleura (SFTP) are rare. We report a case of giant SFTP treated by surgical resection. We found that many soft tumors were separated by fibrous cords and creamy white mucus in our case. Among them, one great mass measured 23 cm × 18 cm × 10 cm. These tumors weighed 3750 g. The patient had no postoperative complications and received a good prognosis.
Keywords: Pleural disease, surgery, tumor
|How to cite this article:|
Huang L, Chai Y. Giant fibrous tumours of the pleura with mucus secretion. Niger J Clin Pract 2013;16:260-2
| Introduction|| |
Solitary (localized) fibrous tumors of the pleura (SFTP) are rare neoplasms. They are frequently an incidental finding by chest x-ray. The diagnosis of SFTP relies on pathological immunohistochemistry although computed tomography (CT) got successful preoperative diagnosis. Surgery is the gold standard of treatment,  and long-term follow-up is very important.  In this article, we report a patient with giant SFTP who underwent operation.
| Case Report|| |
A 65-year-old, 56 kg weight, farmer with a history of left intrathoracic tumor resection in 1999 was transferred to this hospital in 2007 with cough and shortness of breath for a month. Chest radiography revealed a large tumor mass in the left hemithorax [Figure 1].
|Figure 1 : Chest X-ray showing a large tumor mass occupying the almost all of left hemithorax|
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CT revealed several tumors in the left hemithorax. Among them, one great mass measured 23 cm × 18 cm × 10 cm. The tumors grew down along the left side of the abdominal cavity [Figure 2]. Lung function showed the forced expiratory volume in 1 second (FEV1) was 0.95 L only.
On 3 April 3 2007, we did the huge tumors resection. When operation, we saw the left pleural cavity was occupied mostly by the huge tumors. The lungs and visceral pleura were not involved by the tumors while the parietal pleura were involved partly. The lobes, especially the lower lobe, were pressed seriously. And we found that these tumors were separated by fibrous cords and white creamy mucus, and they were soft, white and meat-like with white mucus in them [Figure 3]. Our surgical procedure was as follows: After a median sternotomy, we opened the left pleura. While sucking mucus, we separated the adhesion and enucleated a small part of tumors. Then we had some space to separate adhesion towards behind and below, and resected a big part of tumors. However, there were still a lot of tumors in the back part of left pleural cavity. Then left chest wall incision was performed, and we did complete resection. The tumors weighed 3750 g.
|Figure 2 : CT revealing the heterogeneous tumors occupied the left pleural cavity mostly|
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|Figure 3 : Tumor Specimens. The total length of the blade and handle in figure is 16.5 cm|
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Postoperative pathology confirmed that the tumors are SFTP, which are borderline [Figure 4]. Immunohistochemistry (IHC) revealed a diffuse positivity for CD34, vimentin, CD99, and Bcl-2 [Figure 5], while cytokeratin (CK), epithelial membrane antigen (EMA), calretinin, S100 protein, and desmin were negative.
|Figure 4 : Histopathological findings. (a) The tumors have clear boundary, and the tumor cells are sparse. H and E ×100. (b) Multiple cysts can be seen in the part of area. H and E ×100. (c) The tumor cells are spindly and ovoid with ill-defined, and the nuclei are deeply stained with no significant atypia and no mitosis. H and E ×400. (d) The cells on the surface of the cysts and the interstitial cells are in the same. H and E ×400|
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|Figure 5 : Immuohistochemical findings (IHC). Tumor cells exhibiting (a) CD34, (b) vimentin, (c) CD99, (d) Bcl-2 positivity. DAB ×200|
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| Discussion|| |
SFTP are rare, slow-growing neoplasms, and account for <5% of all pleural neoplasms.  The huge tumors mentioned in our case are much rare. To our knowledge, SFTP always have clear boundary with rigid texture. These soft tumors with white mucus in our case have not been seen before, and no similar reports have been found too.
Most of SFTP are frequently an incidental finding on a standard chest X-ray film. However, the patient usually did not take chest X-ray. That leaded to the formation of large tumors.
The diagnosis of SFTP relies on pathological immunohistochemistry. Almost all of SFTP are CD34-positive. Surgery is the gold standard of treatment, , even in cases of poor lung function.  A surgical approach depends on tumor size and location, and larger tumors need open surgery, usually thoracic incision or median sternotomy incision.
Typically, although SFTP may be very huge, they are often not too difficult to remove, for they usually have a certain boundary. However, these tumors in our case almost had no certain boundary. And in our experience, the difficulty also lies in the comprehensive treatment of peri-operation period. Try to discuss it by following aspects:
Firstly, surgeons should on standby during anesthesia. We should wash our hands in advance of intubation, be ready for thoracotomy at any time. During anesthesia, particularly after the use of muscle relaxants, the tissue tension around the tumor decreases, and the strength of support weakens, that may lead a more serious oppression of blood vessels and bronchus, and then may lead to blood pressure decreasing, suffocation and other serious complications. Secondly, do best about "Bleeding Control." The bleeding of the large intrathoracic tumor resection is often appalling. Having prepared adequate venous accesses and enough blood in our cases, the anesthesiologist controlled the blood pressure at a relatively low level. If suturing and oppression while resection, it can reduce bleeding. The bleeding of the patient was up to 8000 ml. Note that review of renal function after operation, and prevent renal failure. Thirdly, we discuss about the prevention of postoperative pulmonary edema. The huge intrathoracic tumors cause the long-term pressure of lungs. After tumors resection, we should pay attention to the speed and intensity of lung expansion, to prevent re-expansion pulmonary edema. In addition, the lungs will produce much sputum after re-expansion, and patients are more susceptible for infection. So we should encourage patients to cough and increase efforts to fight infection.
Although the risk of such surgery is high, the good prognosis encourages us to operate more actively. The patient has received a good prognosis. We follow up him till now for more than 4 years, and find that he is still alive without tumors. What deserves to be mentioned the most is, although the tumors are borderline tumors, they may still be recrudescent tumors from the left intrathoracic tumor resected in 1999 (Unfortunately, the information of first operation in other hospital cannot be provided). Therefore, regardless of benign or malignant, a long-term follow-up is very important. ,,
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]