Ann Thorac Surg 2004;78:e87-e88
© 2004 The Society of Thoracic Surgeons
Case report
Calcifying Fibrous Pseudotumor of the Pleura
Ki Seok Jang, MDa,
Young-Ha Oh, MDa,
Hong Xiu Han, MDa,
Soon-Ho Chon, MDb,
Won Sang Chung, MDb,
Chan Kum Park, MDa,
Seung Sam Paik, MD*,a
a Department of Pathology, College of Medicine, Hanyang University, Seoul, South Korea
b Department of Thoracic and Cardiovascular Surgery, College of Medicine, Hanyang University, Seoul, South Korea
Accepted for publication March 25, 2004.
* Address reprint requests to Dr Paik, Department of Pathology, College of Medicine, Hanyang University, 17, Haengdang-dong, Seongdong-ku, Seoul 133-791, South Korea
sspaik{at}hanyang.ac.kr
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Abstract
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Calcifying fibrous pseudotumor is an uncommon benign lesion that has unique histologic features. We report a case of calcifying fibrous pseudotumor of the pleura occurring in a 31-year-old woman. A computed tomographic scan revealed a pleural mass in the right anterior costophrenic angle. The excised mass was well circumscribed, nonencapsulated, solid, and firm. The tumor showed dense hyalinized collagenous tissue interspersed with spindle cells, psammomatous calcifications, and a predominantly lymphoplasmocytic infiltrate. Most spindle cells were diffusely positive for vimentin, focally positive for CD34, and negative for desmins, smooth muscle actin, S-100 protein, and anaplastic lymphoma kinase-1.
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Introduction
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Calcifying fibrous pseudotumor (CFP) is an uncommon benign fibrous lesion that was originally described by Rosenthal and Abdul-Karim [1], and the term itself was introduced by Fetsch and associates [2]. The lesion is relatively common in children and young adults, but its pathogenesis is still unclear. Recently it has been postulated that CFPs may represent a late sclerosing stage of inflammatory myofibroblastic tumor (IMT) [3, 4]. Lesions most commonly arise in the extremities, followed by the trunk, inguinal and scrotal regions, and the head and neck. Rare cases have been reported in the mediastinum, pleura, and visceral peritoneum [4]. We report a case of pleural CFP with immunohistochemical studies.
A chest wall mass was incidentally found in a simple chest roentgenogram of a healthy 31-year-old woman with clavicular fracture due to a traffic accident. The patient was transferred to our hospital for further evaluation of the chest wall mass. Computed tomographic scan revealed a well demarcated calcifying mass, measuring 8 x 7 x 5 cm in the right anterior costophrenic angle. The mass was shown to compress the dome of her liver (Fig 1). This mass was not associated with her ribs or her adjacent lung. Percutaneous needle biopsy was performed and a piece of grayish white tissue was obtained. Histopathologic examination of the needle biopsy revealed cicatricial tissue with diffusely distributed calcifications without evidence of malignancy. Right lateral thoracotomy was performed and the pleural cavity was entered through the sixth intercostal space. The mass, which originated from the parietal pleura, was resected en-bloc with surrounding tissue, including portions of periosteum from the overlying ribs. There was no extension to the visceral pleura and adhesions were absent. The completely excised mass measured 8.5 x 7.5 x 4.3 cm in dimension. The mass was attached to the parietal pleura. The outer surface was smooth and lobulated. The cut surface showed a well circumscribed, nonencapsulated, solid, and firm appearance (Fig 2). Microscopically, the tumor was composed largely of dense interwoven collagen bundles, scattered wavy spindle-shaped cells, and evenly distributed psammomatous calcifications with inflammatory infiltrates including lymphocytes, plasma cells, and macrophages (Fig 3A). Most infiltrating lymphocytes were of T-cell origin. The spindle cells were diffusely positive for vimentin (Fig 3B) and focally positive for CD34 (Fig 3C), but negative for desmins, smooth muscle actin, anaplastic lymphoma kinase-1, and S-100 protein. The patient's postoperative recovery was uneventful, and the patient was well at her 12-month follow-up examination.
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Fig 1. Computed tomographic scan reveals a pleural mass in the right anterior costophrenic angle (arrow).
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Fig 2. The cut surface of the tumor shows a well circumscribed, nonencapsulated, solid, and firm appearance.
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Fig 3. (A) Microscopically, the tumor is composed largely of dense interwoven collagen bundles, scattered spindle-shaped cells, and psammomatous calcifications with inflammatory infiltrate, including lymphocytes and plasma cells. The spindle cells were positive for (B) vimentin and (C) CD34.
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Comment
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Calcifying fibrous pseudotumor is a rare benign soft tissue entity with unique histologic features originally reported by Rosenthal and Abdul-Karim [1] as a childhood fibrous tumor with psammoma bodies in 1988. In 1993, Fetsch and associates [2] introduced the term calcifying fibrous pseudotumor with an analysis of 10 cases. This tumor most commonly appears as a solitary lesion and primarily affects children and young adults with a slight female predilection [3]. This tumor most commonly arises in the extremities [4]. However, CFP of the pleura is extremely rare. We believe that only 5 cases have been reported to have CFP of the pleura [5–7]. Most patients present a slow growing, painless mass in the subcutaneous or deep soft tissues. Most have been treated by simple local excision, and there was a report of only a single recurrence that occurred 7 years after the initial excision. There have been no reports of metastasis.
The pathologic features of a CFP are well established. The features include a nonencapsulated, densely hyalinized, fibrous proliferation with variable degrees of infiltration of chronic inflammatory cells. Two types of calcification have been described, including psammomatous and dystrophic [1–4]. The present case showed typical histologic features of a CFP with many psammomatous calcifications and areas of inflammatory infiltrates, including lymphocytes, plasma cells, and macrophages in densely hyalinized fibrous stroma.
The main differential diagnoses include IMTs and localized fibrous tumors of the pleura. Inflammatory myofibroblastic tumors are usually more cellular and less hyalinized. However, to distinguish them from IMTs may be difficult, because spindle cells, inflammatory infiltrate, and calcification sometimes characterize both CFPs and IMTs. There is a histologic overlap of these lesions, thus it has been proposed that CFPs represent a late sclerosing phase of IMTs [3, 4]. Recently, a subset of IMTs has been shown to have clonal cytogenetic abnormalities involving the anaplastic lymphoma kinase-1 gene on chromosome 2p [4]. Unlike IMTs, CFPs rarely express the anaplastic lymphoma kinase-1 gene. Our presenting case was negative for anaplastic lymphoma kinase-1 immunostaining. This result partly supports the hypothesis that CFPs have a different clinicopathologic entity when comparing them with IMTs. This hypothesis opposes the idea that CFPs represents a late sclerosing stage of IMTs. Localized fibrous tumors of the pleura also show benign fibrous proliferation with hyalinized collagenous stroma, but psammomatous or dystrophic calcifications and inflammatory infiltrates are not combined. Characteristically, the fibrous tumor cells of localized fibrous tumors of the pleura are positive for CD34 immunostaining. Most reported CFPs are negative for CD34, except for rare cases [8]. Our case was positive for CD34 immunostaining. Our CD34 positive finding deviates from the other greater part of CD34 negative immunostaining found in most CFPs. CD34 positive cases in the literature have been found to develop in the neck, peritoneum, mediastinum, and paratesticular sites. Our case had developed in the parietal pleura and was shown to be positive for CD34 immunostaining.
Calcifying fibrous pseudotumor of the pleura is extremely rare, and only 5 cases of CFT of the pleura have been described in the literature. We report an additional case of CFP of the pleura with immunohistochemical studies.
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References
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- Rosenthal NS, Abdul-Karim FW. Childhood fibrous tumor with psammoma bodies. Clinicopathologic 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]
- Van Dorpe J, Ectors N, Geboes K, D'Hoore A, Scoit R. Is calcifying fibrous pseudotumor a late sclerosing stage of inflammatory myofibroblastic tumor? Am J Surg Pathol. 1999;23(3):329–335[Medline]
- Sigel JE, Smith TA, Reith JD, Goldblum JR. Immunohistochemical analysis of anaplastic lymphoma kinase expression in deep soft tissue calcifying fibrous pseudotumor: evidence of a late sclerosing stage of inflammatory myofibroblastic tumor? Ann Diagn Pathol. 2001;5:10–14[Medline]
- Heinaut 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]
- Pinkard NB, Wilson RW, Lawless N, et al. Calcifying fibrous pseudotumor of pleura: a report of three cases of a newly described entity involving the pleura. Am J Clin Pathol. 1996;105(2):189–194[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]
- Hill KA, Gonzalez-Crussi F, Chou PM. Calcifying fibrous pseudotumor versus inflammatory myofibroblastic tumor: a histological and immunohistochemical comparison. Mod Pathol. 2001;14:784–790[Medline]
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Abstract
Introduction
