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J Thorac Cardiovasc Surg 1995;110:549-551
© 1995 Mosby, Inc.


BRIEF COMMUNICATIONS

INTRAPULMONARY BENIGN FIBROUS TUMOR OF THE PLEURA

Thomas X. Aufiero, MDa, Suzan A. McGary, MDa, David B. Campbell, MDa, Peter P. Phillips, MDb


Hershey, Pa.

From the Department of Surgery, Division of Cardiothoracic Surgery,a and the Department of Pathology,b University Hospital, The Milton S. Hershey Medical Center, The Pennsylvania State University, PO Box 850, Hershey, PA 17033.

Primary pleural tumors are commonly divided into diffuse and localized types.Go 1 The diffuse malignant mesothelioma, associated with asbestos exposure, is the most commonly recognized lesion of this group. The localized variety is less well defined and has been described by a variety of names, including benign mesothelioma, fibrosing mesothelioma, subpleural fibroma, and localized fibrous tumor of the pleura. The clinical behavior and histologic features of these tumors are quite different from those of the diffuse type. Some investigators have suggested that the term mesothelioma is confusing.Go 2 The majority of these benign fibrous tumors appear as polypoid lesions and have rarely been reported as intraparenchymal lesions.Go 3

A 20-year-old white woman had a routine chest roentgenogram after a fall from a horse. Before that time she had been completely free of symptoms. She had no history of cough, shortness of breath, sputum production, or tobacco use. The x-ray film revealed a large mass within the upper lobe of the right lung. A subsequent computed tomographic scan (Fig. 1) showed the proximity of the lesion to the origin of the right upper lobe bronchus. A bronchoscopic study showed an endobronchial lesion consistent with inflammation. Endobronchial biopsies were performed, but a satisfactory histopathologic diagnosis could not be determined from these specimens. At thoracotomy a 3 cm tumor was seen involving the basal segment of the right upper lobe. Associated atelectasis and inflammation involved the superior segment of the right lower lobe. For complete excision of the tumor with satisfactory margins, a sleeve resection of the right upper lobe had to be performed.



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Fig. 1. Computed tomographic scan showing size of tumor and proximitry to hilum.

 
The mass was well circumscribed and measured 25 by 3 cm. A cross section revealed an intrapulmonary well-circumscribed whorled white nodule with central calcification. It encircled the bronchus but did not grossly invade the bronchus. The tumor extended to the pleural surface without involving the pleura. No necrosis was appreciated. The remaining lung tissue had several areas of atelectasis and inflammation that appeared to be associated with the mass.

Histologically, the lesion showed a proliferation of spindle cells in haphazardly arranged fascicles and a storiform pattern. The cells were bland in appearance, and a low mitotic rate of less than 1 mitosis per 10 high-power fields was appreciated. The cytoplasmic borders were indistinct, and collagen separated individual cells and fascicles. An area of metaplastic bone formation was identified in the center of the lesion (Fig. 2). At the periphery a focus of entrapped ciliated bronchial epithelium was identified (Fig. 3). A final diagnosis of intrapulmonary benign fibrous tumor of the pleura was made. The specimen was sent to an outside reviewer at another institution, who confirmed the diagnosis.



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Fig. 2. Proliferation of spindle cells arranged in haphazard fascicles with interspersed collagen and entrapped bronchiolar epithelium. (Hematoxylin and eosin; original magnification x 63; inset x 160.)

 


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Fig. 3. Metaplastic bone formation within tumor. (Hematoxylin and eosin; original magnification x 63; inset x 160.)

 
Mesotheliomas of the pleura have historically been classified as diffuse or localized. The frequency of the diffuse malignant mesothelioma associated with asbestos exposure has made this tumor easy to identify. In 1931 Klemperer and RabinGo 4 divided primary pleural neoplasm into two categories: diffuse and localized. It was believed that the diffuse lesion represented a true mesothelioma and that localized lesions originated from subpleural alveolar cells. Some confusion still exists regarding localized mesothelioma. The histopathologic features of this lesion are consistent with a benign localized fibrous tumor. These lesions are characterized microscopically by a proliferation of fibroblast-like cells and connective tissue arranged in a disorderly and random fashion. Classically, benign fibrous mesotheliomas are described as pedunculated masses attached to the pleura by a narrow pedicle. Yousem and FlynnGo 3 proposed that the histologic and immunochemical presentation of these tumors should be used to include the less frequently observed intraparenchymal lesion. Several explanations for the intrapulmonary location of these tumors have been proposed.Go 3 The first is that the subpleural mesenchyma is in direct continuity with the connective tissues of the intralobular septa. Presumably, intrapulmonary fibrosis might arise from this mesenchyma. A more likely hypothesis, for this patient, is that the tumor originated from the fibroblasts of the pulmonary parenchyma itself. Although alveolar pneumocytes and small bronchioles may become entrapped within the fibrous cells of this tumor, they retain their benign appearance.

Two other tumors must be considered in the differential diagnosis of these lesions: malignant fibrous histiocytomas and pulmonary fibrosarcomas. Malignant fibrous histiocytomas tend to be large tumors with more extensive necrosis and vascular invasion than seen in benign fibrous tumors of the pleura. They can also be discriminated histologically by a storiform growth pattern, cytologic atypia, and a much greater number of mitoses, in addition to pleomorphic malignant giant cells. Guccion and RosenGo 5 have described fibrosarcomas in the lung as having a prominent herring-bone growth pattern, as well as frequent necrosis and more than 5 mitoses per 10 high-power fields. The differential diagnosis is completed if an ambiguous group of tumors plasma cell granulomas, also known as fibrous histiocytomas, is detected. These tumors have a chronic inflammatory infiltrate with numerous plasma cells, as well as histiocytes.Go 3

Intraparenchymal presentation of localized mesotheliomas has been associated with a higher incidence of aggressive or malignant behavior.Go 1 A number of symptoms have been associated with these tumors, including digital clubbing, osteoarthropathy,Go 6 and hypoglycemia.Go 7 Most symptoms, however, have been related to the size of the tumor, rather than to its malignant potential.Go 6 The varied nature of the histologic features of these tumors makes diagnosis by needle biopsy or transbronchial biopsy difficult. Excisional biopsy is indicated in most cases. In a review of 52 cases of localized pleural mesothelioma, Okike, Bernatz, and WoolnerGo 6 reported on eight patients with a malignant variant. All of these patients had a recurrence or distant metastasis between 6 months and 8 years after the initial resection. One half had local recurrences or recurrences within the thoracotomy incision. On the basis of this information, we recommend complete resection of the tumor, as well as close follow-up for years after resection.

Footnotes

J THORAC CARDIOVASC SURG 1995; 110:549-51 Back

References

  1. England DM, Hochholzer L, McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura--a clinico-pathologic review of 223 cases. Am J Surg Pathol 1989;13:640-58.[Medline]
  2. Brisseli M, Mark EJ, Dickersin GR. Solitary fibrous tumors of the pleura--eight new cases and review of 360 cases in the literature. Cancer 1981;47:2678-89.[Medline]
  3. Yousem SA, Flynn SD. Intrapulmonary localized fibrous tumor--intraparenchymal so-called localized fibrous mesothelioma. Am J Clin Pathol 1988;89:365-9.[Medline]
  4. Klemperer P, Rabin LB. Primary neoplasms of the pleura. Arch Pathol 1931;11:385-412.
  5. Guccion JG, Rosen SH. Broncho pulmonary leiomyosarcoma and fibrosarcoma. Cancer 1972;30:836-47.[Medline]
  6. Okiki N, Bernatz PE, Woolner LB. Localized mesothelioma of the pleura: benign and malignant variants. J THORAC CARDIOVASC SURG 1978;75:363-72.[Abstract]
  7. Mandal AK, Roger MA, Salem FA, Oparah SJ. Localized benign mesothelioma of the pleura associated with a hypoglycemic episode. Arch Intern Med 1983;143:1608-10.[Abstract/Free Full Text]



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