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Multiple Calcifying Fibrous Pseudotumors Disseminated in the Pleura

2008; Elsevier BV; Volume: 85; Issue: 2 Linguagem: Inglês

10.1016/j.athoracsur.2007.10.059

1552-6259

Kazuo Shibata, Daisuke Yuki, Keita Sakata,

Medical Imaging and Pathology Studies

Calcifying fibrous pseudotumor is an extremely rare benign lesion that develops in the pleura. We describe a case of multiple lesions in the pleura. The patient is a 52-year-old woman who had a subpleural mass on chest roentgenogram. The diagnosis was established by percutaneous needle biopsy. The largest tumor was hanging down from the parietal pleura, and additional small nodules were disseminated throughout the pleural cavity. Only a few tumors arising from the largest one were resected, and the others were left unresected. The resected tumors consisted of collagenous fibrous tissue, calcifications, and spindle cells that were positive only for vimentin immunostaining. Calcifying fibrous pseudotumor is an extremely rare benign lesion that develops in the pleura. We describe a case of multiple lesions in the pleura. The patient is a 52-year-old woman who had a subpleural mass on chest roentgenogram. The diagnosis was established by percutaneous needle biopsy. The largest tumor was hanging down from the parietal pleura, and additional small nodules were disseminated throughout the pleural cavity. Only a few tumors arising from the largest one were resected, and the others were left unresected. The resected tumors consisted of collagenous fibrous tissue, calcifications, and spindle cells that were positive only for vimentin immunostaining. Calcifying fibrous pseudotumor (CFP) is histologically classified as a cicatricose lesion composed of thick-hyalinized collagenous, fibrous tissue; scanty spindle-shaped cells; and lymphoplasmocytic infiltrates with psammomatous or dystrophic calcifications, or both. In 1988, Rosenthal and Abdul-Karim [1Rosenthal N.S. Abdul-Karim F.W. Childhood fibrous tumor with psammoma bodies Clinicopathologic features in two cases.Arch Pathol Lab Med. 1988; 112: 798-800PubMed Google Scholar] first described two cases of CFP in two different girls and named it "childhood fibrous tumor with psammoma bodies." Fetsch and colleagues [2Fetsch J.F. Montgomery E.A. Meis J.M. Calcifying fibrous pseudotumor.Am J Surg Pathol. 1993; 17: 502-508Crossref PubMed Scopus (223) Google Scholar] reported 10 adult cases of this entity in 1993 and named it CFP. In 1996, Pinkard and colleagues [3Pinkard N.B. Wilson R.W. Lawless N. et al.Calcifying fibrous pseudotumor of pleura A report of 3 cases of a newly described entity involving the pleura.Am J Clin Pathol. 1996; 105: 189-194Crossref PubMed Scopus (108) Google Scholar] first described 3 patients with CFP that developed in the pleura. Calcifying fibrous pseudotumor usually occurs in the extremities and trunk of individuals and is extremely rare in the pleura. A 52-year-old woman was admitted to our hospital with a tumor shadow on her chest roentgenogram. She was a smoker and had no subjective symptoms and no history of asbestos exposure. Her chest roentgenogram showed a knobby subpleural mass in the left costophrenic angle that had increased in size compared with a roentgenogram from 2003. A computed tomographic scan revealed a widely based mass (6 cm in maximum diameter) on the chest wall, with a calcification measuring several millimeters in diameter (Fig 1). The specimen obtained by percutaneous needle biopsy was suggestive of being a calcifying fibrous pseudotumor. Video-assisted surgery through a mini-thoracotomy was performed on our patient to exclude diffuse mesothelioma or a solitary fibrous tumor. The largest tumor was hanging down with a long pedicle into the thoracic cavity from the chest wall (Fig 2A). Another tumor (Fig 2B), which was 3.5 cm in diameter was present on the left diaphragm and formed a knobby appearance, together with the main tumor on her chest roentgenogram. Furthermore, many small nodules were disseminated throughout the chest wall, diaphragm, and visceral pleura of the left lower lobe. Therefore, we decided not to resect all of the nodules and only excised several tumors, including the largest nodule. In our patient, the main tumor, which was 6 cm in diameter, was firm, lobulated in shape, and had a smooth surface. Histologically, the tumor was composed of thick collagenous, fibrous tissue; scanty spindle cells; and small vascular structures with lymphocytic infiltrates. Dystrophic or psammomatous calcifications were present (Fig 3). No atypical features were identified. Immunohistochemically, spindle-shaped cells were positive only for vimentin, negative for CD34, factor XIIIa, β-cell CLL/lymphoma 2, desmin, α-smooth muscle actin, and CD68. The histologic findings were compatible with a diagnosis of CFP. Her postoperative course was uneventful and no additional surgery was performed. It was recommended that the patient undergo computed tomography every 6 months to determine whether or not she should undergo a reoperation if the residual tumors were found to be enlarged. Calcifying fibrous pseudotumor is histologically characterized as a cicatricose lesion composed of thick hyalinized collagenous, fibrous tissue; scanty spindle-shaped cells; and lymphoplasmocytic infiltrates with psammomatous or dystrophic calcifications, or both. This usually originates from the soft tissue of the extremities and trunk, and quite rarely from the pleura. Only 10 cases originating from the pleura have been reported in the literature to date since Pinkard and colleagues [3Pinkard N.B. Wilson R.W. Lawless N. et al.Calcifying fibrous pseudotumor of pleura A report of 3 cases of a newly described entity involving the pleura.Am J Clin Pathol. 1996; 105: 189-194Crossref PubMed Scopus (108) Google Scholar] first described three cases in 1996. In the new classification system by the World Health Organization in 1999, CFP was classified as a soft tissue tumor. The previously reported cases and our case are summarized in Table 1. The median age was 38.7 years (range, 23 to 54 years), and there were 3 men and 8 women. The CFP often occurs in young adults and predominantly in women, as in our case.Table 1Previously Reported 10 Cases of Calcifying Fibrous Pseudotumor of the Pleura in the LiteratureCaseAuthor [Ref No.] (Year)Age/GenderNumber of TumorsOperationImmunostaining PositiveFollow-UpRecurrence1Pinkard and colleagues [3Pinkard N.B. Wilson R.W. Lawless N. et al.Calcifying fibrous pseudotumor of pleura A report of 3 cases of a newly described entity involving the pleura.Am J Clin Pathol. 1996; 105: 189-194Crossref PubMed Scopus (108) Google Scholar] (1996)23/FMultipleLocal resection—9 mosnone2Pinkard and colleagues [3Pinkard N.B. Wilson R.W. Lawless N. et al.Calcifying fibrous pseudotumor of pleura A report of 3 cases of a newly described entity involving the pleura.Am J Clin Pathol. 1996; 105: 189-194Crossref PubMed Scopus (108) Google Scholar] (1996)28/FTwoLocal resection—12 mosnone3Pinkard and colleagues [3Pinkard N.B. Wilson R.W. Lawless N. et al.Calcifying fibrous pseudotumor of pleura A report of 3 cases of a newly described entity involving the pleura.Am J Clin Pathol. 1996; 105: 189-194Crossref PubMed Scopus (108) Google Scholar] (1996)34/MSolitaryLocal resection——none4Hainaut and colleagues [4Hainaut P. Lesage V. Weynand B. Coche E. Noirhomme P. Calcifying fibrous pseudotumor: a patient presenting with multiple pleural lesions.Acta Clin Belg. 1999; 54: 162-164PubMed Google Scholar] (1999)29/FDisseminatedIncomplete resection———5Cavazza and colleagues [9Cavazza A. Gelli M.C. Agostini G. Sgarbi G. DeMarco L. Gardini G. Pseudotumor fibroso calcifico della pleura: descrizione di un caso Calcifying fibrous pseudotumour of pleura: a case report.Pathologica. 2002; 94: 201-205Crossref PubMed Scopus (17) Google Scholar] (2002)46/FSolitaryLocal resectionVimentin7 yrnone6Ammar and colleagues [5Ammar A. Hammami S.E. Horchani H. Sellami N. Kilani T. Calcifying fibrous pseudotumor of the pleura: a rare location.Ann Thorac Surg. 2003; 76: 2081-2082Abstract Full Text Full Text PDF PubMed Scopus (31) Google Scholar] (2003)38/FSolitaryLocal resectionCytokeratin, vimentin9 mosnone7Jang and colleagues [6Jang K.S. Oh Y.H. Han H.X. et al.Calcifying fibrous pseudotumor of the pleura.Ann Thorac Surg. 2004; 78: e87-e88Abstract Full Text Full Text PDF PubMed Scopus (32) Google Scholar] (2004)31/FSolitaryLocal resectionVimentin, CD3412 mosnone8Kondo N (2004)54/MBilateral thoraxRight side resection———9Kawahara and colleagues [7Kawahara K. Yasukawa M. Nakagawa K. Katsura H. Nagano T. Iwasaki T. Multiple calcifying fibrous tumor of the pleura.Virchows Arch. 2005; 447: 1007-1008Crossref PubMed Scopus (23) Google Scholar] (2005)35/FMultipleLocal extirpationVimentin, factor XIIIa——10Mito and colleagues [8Mito K. Kashima K. Daa T. et al.Multiple calcifying fibrous tumors of the pleura.Virchows Arch. 2005; 446: 78-81Crossref PubMed Scopus (38) Google Scholar] (2005)54/MTwoLocal resectionVimentin, CD34, factor XIIIa——11Present case54/FDisseminatedIncomplete resectionVimentin4 mos—F = female; M = male. Open table in a new tab F = female; M = male. With respect to the number of nodules, solitary nodules were present in 4 patients and multiple nodules were present in 7. In 9 of 11 cases, including those with multiple nodules, all nodules were excised entirely, and no recurrence has been reported. In CFP involving the back, which was incompletely excised, local recurrence at 7.5 years after the first operation has been reported. Therefore, the principle treatment for CFP is complete resection of the lesions. In both of the cases described by Hainaut and colleagues [4Hainaut P. Lesage V. Weynand B. Coche E. Noirhomme P. Calcifying fibrous pseudotumor: a patient presenting with multiple pleural lesions.Acta Clin Belg. 1999; 54: 162-164PubMed Google Scholar] and in our case, not all of the nodules could be excised. It is believed that the residual tumors will continue to grow and a reoperation may be required in the near future. The median follow-up period of the reported cases was 19.4 months (range, 4 to 84 months). The lengths of observation, except that in case 5, were too short because the CFP was believed to be benign and slow growing. Therefore, long-term observation seems to be required. Although the confirmation of CFP is obtained by histologic examination, this might aid in the diagnosis of CFP with a preoperative computed tomographic scan that reveals a subpleural mass with calcifications. Percutaneous needle biopsy was performed in three cases, but this was only useful for diagnosis in our case. Immunochemical staining of the specimen obtained by percutaneous needle biopsy is helpful to distinguish CFP from calcified granulomas, calcified pleural plaques, and solitary fibrous tumors. Solitary fibrous tumors tend to be positive for CD34 and bcl-2. On the other hand, CFP is generally negative for those markers. Although CFP is ordinarily positive for vimentin, factor XIIIa, and CD68, the last two markers were negative in the present case [5Ammar A. Hammami S.E. Horchani H. Sellami N. Kilani T. Calcifying fibrous pseudotumor of the pleura: a rare location.Ann Thorac Surg. 2003; 76: 2081-2082Abstract Full Text Full Text PDF PubMed Scopus (31) Google Scholar, 6Jang K.S. Oh Y.H. Han H.X. et al.Calcifying fibrous pseudotumor of the pleura.Ann Thorac Surg. 2004; 78: e87-e88Abstract Full Text Full Text PDF PubMed Scopus (32) Google Scholar, 7Kawahara K. Yasukawa M. Nakagawa K. Katsura H. Nagano T. Iwasaki T. Multiple calcifying fibrous tumor of the pleura.Virchows Arch. 2005; 447: 1007-1008Crossref PubMed Scopus (23) Google Scholar, 8Mito K. Kashima K. Daa T. et al.Multiple calcifying fibrous tumors of the pleura.Virchows Arch. 2005; 446: 78-81Crossref PubMed Scopus (38) Google Scholar]. In two previously reported cases, CD34 [6Jang K.S. Oh Y.H. Han H.X. et al.Calcifying fibrous pseudotumor of the pleura.Ann Thorac Surg. 2004; 78: e87-e88Abstract Full Text Full Text PDF PubMed Scopus (32) Google Scholar, 8Mito K. Kashima K. Daa T. et al.Multiple calcifying fibrous tumors of the pleura.Virchows Arch. 2005; 446: 78-81Crossref PubMed Scopus (38) Google Scholar] was positive. These findings are believed to represent the heterogeneity of CFP. The outcome of patients of incompletely excised lesions is still uncertain. In the setting of multiple nodules, there is the possibility that the nodules represent pleural dissemination from a primary tumor instead of multicentric development.