Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2016.053
Autopsy and Case Reports
Article / Clinical Case Report

Endobronchial solitary fibrous tumor

Cristiano Claudino Oliveira, Marcelo Padovani Toledo de Moraes, Thomas Colby, Gilmar Felisberto Oliveira, Erica Nishida Hasimoto, Daniele Cristina Cataneo, Antônio José Maria Cataneo, Julio De Faveri

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Abstract

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that appears primarily in the pleura and rarely in intrapulmonary or endobronchial topography. The authors report the case of a 47-year-old woman who presented obstructive respiratory symptoms for 4 years. The chest computed tomography and bronchoscopy showed an obstructive polypoid lesion located between the trachea and the left main bronchus associated with distal atelectasis of the left lung. A resection of the lesion was performed and, macroscopically, the mass was oval, encapsulated, and firm, measuring 2.3 × 1.7 × 1.5 cm. Histology revealed low-grade mesenchymal spindle cell neoplasm, with alternating cellularity, myxoid areas, and mature adipose tissue outbreaks, as well as blood vessels with irregular walls. The immunohistochemical study was positive for CD34, CD99, and BCL2. The diagnosis was SFT in an unusual topography. The patient’s symptoms remitted after tumor excision, and no systemic problems were evident. SFTs primarily affect adults and often follow a benign course; however, their behavior is unpredictable. The presence of necrosis and mitotic activity may portend a poor prognosis. Endobronchial SFTs are rare but should be evaluated and monitored similar to SFTs at other sites, with a long-term follow-up.

Keywords

Solitary Fibrous Tumors, Bronchi, Immunohistochemistry, Lung Neoplasms, Pathology

References

1. Goldblum JR, Folpe AL, Weiss SW. Soft tissue tumors of intermediate malignancy of uncertain type. In: Goldblum JR, Folpe AL, Weiss SW, editors. Enzinger and Weiss’s soft tissue tumors. 6th ed. Philadelphia: Mosby Elsevier; 2014. p. 1093-1160.

2. Fletcher CDM, Bridge JA, Lee JC. Extrapleural solitary fibrous tumor. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Martens F, editors. WHO classification of tumors of soft tissue and bone. Lyon: IARC; 2013.

3. Pak PS, Yanagawa J, Abtin F, Wallace WD, Holmes C, Lee JM. Surgical manegement of endobronchial solitary fibrous tumor. Ann Thorac Surg. 2010;90(2):659-61. PMid:20667378. http://dx.doi.org/10.1016/j.athoracsur.2010.02.024.

4. Rao N, Colby TV, Falconieri G, Cohen H, Moran CA, Suster S. Intrapulmonary solitary fibrous tumors: clinicopathological and immunohistochemical study of 24 cases. Am J Surg Pathol. 2013;37(2):155-66. PMid:23108019. http://dx.doi.org/10.1097/PAS.0b013e31826a92f5.

5. Hasegawa T, Matsuno Y, Shimoda T, Hasegawa F, Sano T, Hirohashi S. Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior. Hum Pathol. 1999;30(12):1464-73. PMid:10667425. http://dx.doi.org/10.1016/S0046-8177(99)90169-7.

6. Saint Aubain Somerhausen N, Rubin BP, Fletcher CD. Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis. Mod Pathol. 1999;12(5):463-71. PMid:10349983.

7. Guillou L, Gebhard S, Coindre JM. Lipomatous hemangiopericytoma: a fat-containing variant of solitary fibrous tumor? clinicopathologic, immunohistochemical, and ultrastructural analysis of a series in favor of a unifying concept. Hum Pathol. 2000;31(9):1108-15. PMid:11014579. http://dx.doi.org/10.1053/hupa.2000.9777.

8. Bouvier C, Bertucci F, Métellus P, et al. ALDH1 is an immunohistochemical diagnostic marker for solitary fibrous tumours and haemangiopericytomas of the meninges emerging from gene profiling study. Acta Neuropathol Commun. 2013;1(1):10. PMid:24252471. http://dx.doi.org/10.1186/2051-5960-1-10.

9. Mohajeri A, Tayebwa J, Collin A, et al. Comprehensive genetic analysis identifies a pathognomonic NAB2/STAT6 fusion gene, nonrandom secondary genomic imbalances, and a characteristic gene expression profile in solitary fibrous tumor. Genes Chromosomes Cancer. 2013;52(10):873-86. PMid:23761323. http://dx.doi.org/10.1002/gcc.22083.

10. Schweizer L, Koelsche C, Sahm F, et al. Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. Acta Neuropathol. 2013;125(5):651-8. PMid:23575898. http://dx.doi.org/10.1007/s00401-013-1117-6.

11. Boland JM, Fritchie KJ, Erickson-Johnson MR, Oliveira AM, Colby TV, Folpe AL. Endobronchial lipomatous tumors: clinicopathologic analysis of 12 cases with molecular cytogenetic evidence supporting classification as “lipoma”. Am J Surg Pathol. 2013;37(11):1715-21. PMid:24121172. http://dx.doi.org/10.1097/PAS.0b013e3182a115c9.

12. Magro G, Bisceglia M, Michal M, Eusebi V. Spindle cell lipoma-like tumor, solitary fibrous tumor and myofibroblastoma of the breast: a clinico-pathological analysisof 13 cases in favor of a unifying histogenetic concept. Virchows Arch. 2002;440(3):249-60. PMid:11889594. http://dx.doi.org/10.1007/s00428-001-0572-y.

13. Fritchie KJ, Carver IP, Sun Y, et al. Solitary Fibrous Tumor: Is There a Molecular Relationship With Cellular Angiofibroma, Spindle Cell Lipoma, and Mammary-Type Myofibroblastoma? Am J Clin Pathol. 2012;137(6):963-70. PMid:22586056. http://dx.doi.org/10.1309/AJCPQEG6YNN6CNAL.

14. Demicco EG, Park MS, Araujo DM, et al. Solitary Fibrous Tumors: a clinicalpathological study of 110 cases and proposed risk assessment model. Mod Pathol. 2012;25(9):1298-306. PMid:22575866. http://dx.doi.org/10.1038/modpathol.2012.83.


Publication date:
12/12/2016

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