Ann Thorac Surg 2008;85:e3-e5. doi:10.1016/j.athoracsur.2007.10.059
© 2008 The Society of Thoracic Surgeons
Case Reports
Multiple Calcifying Fibrous Pseudotumors Disseminated in the Pleura
Kazuo Shibata, MDa,*,
Daisuke Yuki, MDa,
Keita Sakata, MDb
a Department of Pulmonary Surgery, National Center for Geriatrics and Gerontology, Aichi, Japan
b Department of Pathology, National Center for Geriatrics and Gerontology, Aichi, Japan
Accepted for publication October 16, 2007.
* Address correspondence to Dr Shibata, Department of Pulmonary Surgery, National Center for Geriatrics and Gerontology, 36-3 Gengo, Morioka-cho, Obu-City, Aichi, 474-8511, Japan (Email: kshibata{at}ncgg.go.jp).
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Abstract
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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.
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Introduction
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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 [1] first described two cases of CFP in two different girls and named it "childhood fibrous tumor with psammoma bodies." Fetsch and colleagues [2] reported 10 adult cases of this entity in 1993 and named it CFP. In 1996, Pinkard and colleagues [3] 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.
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Fig 1. Computed tomographic scan reveals a subpleural mass with dystrophic calcification (arrow) in the left chest wall.
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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.
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Fig 2. (A) Thoracoscopic view shows the largest tumor developed, shown hanging down into the thoracic cavity from the chest wall. (B) Small nodules (arrowheads) were disseminated on the chest wall and the visceral pleura of mainly the lower lobe.
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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, 
-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.
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Fig 3. Microscopic features of the resected specimen shows hyalinized collagenous tissue with psammomatous calcifications. (Hematoxylin & eosin stain, x200.)
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Comment
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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 [3] 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.
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 [4] 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 [5–8]. In two previously reported cases, CD34 [6, 8] 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.
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References
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- Rosenthal NS, Abdul-Karim FW. Childhood fibrous tumor with psammoma bodiesClinicopathologic features in two cases. Arch Pathol Lab Med 1988;112:798-800.[Medline]
- Fetsch JF, Montgomery EA, Meis JM. Calcifying fibrous pseudotumor Am J Surg Pathol 1993;17:502-508.[Medline]
- Pinkard NB, Wilson RW, Lawless N, et al. Calcifying fibrous pseudotumor of pleuraA report of 3 cases of a newly described entity involving the pleura. Am J Clin Pathol 1996;105:189-194.[Medline]
- Hainaut P, Lesage V, Weynand B, Coche E, Noirhomme P. Calcifying fibrous pseudotumor: a patient presenting with multiple pleural lesions Acta Clin Belg 1999;54:162-164.[Medline]
- Ammar A, Hammami SE, Horchani H, Sellami N, Kilani T. Calcifying fibrous pseudotumor of the pleura: a rare location Ann Thorac Surg 2003;76:2081-2082.[Abstract/Free Full Text]
- Jang KS, Oh YH, Han HX, et al. Calcifying fibrous pseudotumor of the pleura Ann Thorac Surg 2004;78:e87-e88.[Abstract/Free Full Text]
- Kawahara K, Yasukawa M, Nakagawa K, Katsura H, Nagano T, Iwasaki T. Multiple calcifying fibrous tumor of the pleura Virchows Arch 2005;447:1007-1008.[Medline]
- Mito K, Kashima K, Daa T, et al. Multiple calcifying fibrous tumors of the pleura Virchows Arch 2005;446:78-81.[Medline]
- Cavazza A, Gelli MC, Agostini G, Sgarbi G, DeMarco L, Gardini G. Pseudotumor fibroso calcifico della pleura: descrizione di un casoCalcifying fibrous pseudotumour of pleura: a case report. Pathologica 2002;94:201-205.[Medline]
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Abstract
Introduction
