Intracranial Solitary Fibrous Tumor in a 25-Year-Old Woman
DOI:
https://doi.org/10.19166/med.v13i3.8890Keywords:
Intracranial solitary fibrous tumor, SFT, ISFT, STAT6, CD34Abstract
Introduction: Intracranial solitary fibrous tumors (ISFTs) are extremely rare spindle cell tumors originating from dendritic mesenchymal cells expressing CD34 antigens that are usually benign, although malignant transformation had been reported. The knowledge of natural course and prognostic factors of ISFTs is still limited and the tumor is easily misdiagnosed.
Case Presentation: An intra-cranial extra-axial tumor tissue resection from a 25-year-old woman was evaluated in the Surgical Pathology Laboratory. Histologic findings (cellular spindle cell tumor with ”˜patternless’ pattern, staghorn blood vessels and <5 mitoses per 10 hpf) and immunophenotype (positive for CD34, weakly and focally positive for STAT6) suggested a diagnosis of intracranial solitary fibrous tumor WHO Grade II.
Discussion: ISFTs have very low incidence in the CNS and are difficult to distinguish radiologically from meningiomas, thus post operative pathological examination and immunohistochemistry markers evaluations are the mainstay for diagnosis. ISFT is associated with NAB2-STAT6 gene fusion and may exhibits a wide spectrum of histological features. STAT6 immunohistochemistry is considered as one of the most sensitive diagnostic methods, while the evaluation of CD34 expression can be used as alternative diagnostic method despite having lower sensitivity.
References
1. Suzuki SO, Fukui M, Nishio S, Iwaki T. Clinicopathological features of solitary fibrous tumor of the meninges: An immunohistochemical reappraisal of cases previously diagnosed to be fibrous meningioma or hemangiopericytoma. Pathol Int. 2000; 50(10):808-17. https://doi.org/10.1046/j.1440-1827.2000.01120.x
2. Wang ZY, Qiu K, Ma YH, Wang XT, Bao JJ, Zhang ZF, Liu XZ. Intracranial solitary fibrous tumors: A report of two cases and a review of the literature. Oncol Lett. 2016; 11(2):1057-1060. https://doi.org/10.3892/ol.2015.3985
3. Yang G, Deisch J. Intracranial Solitary Fibrous Tumor with Pseudopapillary Architecture: An Uncommon Tumor with Unusual Histopathology. N A J Med Sci. 2016 ;9(4):181-186. https://doi.org/10.7156/najms.2016.0904181
4. Gubian A, Ganau M, Cebula H, Todeschi J, Scibilia A, Noel G, Spatola G, Chaussemy D, Nannavecchia B, Gallinaro P, Coca A, Pop R, Signorelli F, Proust F, Lhermitte B, Chibbaro S. Intracranial Solitary Fibrous Tumors: A Heterogeneous Entity with an Uncertain Clinical Behavior. World Neurosurg. 2019; 126:e48-e56. https://doi.org/10.1016/j.wneu.2019.01.142
5. Carneiro SS, Scheithauer BW, Nascimento AG, Hirose T, Davis DH. Solitary fibrous tumor of the meninges: a lesion distinct from fibrous meningioma. A clinicopathologic and immunohistochemical study. Am J Clin Pathol. 1996; 106(2):217-24. https://doi.org/10.1093/ajcp/106.2.217
6. Bisceglia M, Galliani C, Giannatempo G, Lauriola W, Bianco M, D'angelo V, Pizzolitto S, Vita G, Pasquinelli G, Magro G, Dor DB. Solitary fibrous tumor of the central nervous system: a 15-year literature survey of 220 cases (August 1996-July 2011). Adv Anat Pathol. 2011; 18(5):356-92. https://doi.org/10.1097/pap.0b013e318229c004
7. Demicco EG, Fritchie KJ, Han A. Solitary fibrous tumor. In: WHO Classification of Tumours Editorial Board, editor. WHO Classification of Tumours 5th edition: Soft Tissue and Bone Tumours. 2020; 104-108.
8. Chenhui Z, He G, Wu Z, Rong J, Ma F, Wang Z, Fang J, Gao W, Song H, Zhang F, Di G, Jiang X. Intracranial solitary fibrous tumor/hemangiopericytomas: a clinical analysis of a series of 17 patients. Br J Neurosurg. 2021:1-8. https://doi.org/10.1080/02688697.2021.1944980
9. Cheng L, Ni H, Dai Y. Intracranial solitary fibrous tumor mimicking meningioma: A case report. Medicine (Baltimore). 2020; 99(50):e23504. https://doi.org/10.1097%2FMD.0000000000023504
10. Liu J, Wu S, Zhao K, Wang J, Shu K, Lei T. Clinical Features, Management, and Prognostic Factors of Intracranial Solitary Fibrous Tumor. Front Oncol. 2022;12:915273. https://doi.org/10.3389%2Ffonc.2022.915273
11. Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 2016;131(6):803-20. https://doi.org/10.1007/s00401-016-1545-1
12. Gopakumar S, Srinivasan VM, Hadley CC, Anand A, Daou M, Karas PJ, Mandel J, Gopinath SP, Patel AJ. Intracranial Solitary Fibrous Tumor of the Skull Base: 2 Cases and Systematic Review of the Literature. World Neurosurg. 2021;149:e345-e359. https://doi.org/10.1016/j.wneu.2021.02.026
13. Tariq MU, Din NU, Abdul-Ghafar J, Park YK. The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior. Diagn Pathol. 2021;16(1):32. https://doi.org/10.1186/s13000-021-01095-2
14. Kasper E, Boruchow S, Lam FC, Zinn PO, Anderson M, Mahadevan A. "Hitting all the right markers to save a life" Solitary fibrous tumors of the central nervous system: Case series and review of the literature. Surg Neurol Int. 2012;3:83. https://doi.org/10.4103%2F2152-7806.99173
15. Rekhi B, Shetty O, Tripathi P, et al. Molecular characterization of a series of solitary fibrous tumors, including immunohistochemical expression of STAT6 and NATB2-STAT6 fusion transcripts, using reverse transcriptase (RT)-polymerase chain reaction (PCR) technique: an Indian experience. Pathol Res Pract 2017; 213(11):1404-11. https://doi.org/10.1016/j.prp.2017.08.011
16. Robinson DR, Wu YM, Kalyana-Sundaram S, et al. Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nat Genet. 2013; 45(2):180-5. https://doi.org/10.1038/ng.2509
17. Yamashita D, Suehiro S, Kohno S, Ohue S, Nakamura Y, Kouno D, Ohtsuka Y, Nishikawa M, Matsumoto S, Bernstock JD, Harada S, Mizuno Y, Kitazawa R, Ohnishi T, Kunieda T. Intracranial anaplastic solitary fibrous tumor/hemangiopericytoma: immunohistochemical markers for definitive diagnosis. Neurosurg Rev. 2021; 44(3):1591-1600. https://doi.org/10.1007/s10143-020-01348-6
18. Macagno N, Figarella-Branger D, Mokthari K, Metellus P, Jouvet A, Vasiljevic A, Loundou A, Bouvier C. Differential Diagnosis of Meningeal SFT-HPC and Meningioma: Which Immunohistochemical Markers Should Be Used? Am J Surg Pathol. 2016; 40(2):270-8. https://doi.org/10.1111/j.1750-3639.2011.00552.x
19. Sidney LE, Branch MJ, Dunphy SE, Dua HS, Hopkinson A. Concise review: evidence for CD34 as a common marker for diverse progenitors. Stem Cells. 2014 Jun;32(6):1380-9. https://doi.org/10.1002%2Fstem.1661
20. Cummings TJ, Burchette JL,McLendon RE. CD34 and dural fibroblasts: the relationship to solitary fibrous tumor and meningioma. Acta Neuropathol 2001; 102(4):349-54. https://doi.org/10.1007/s004010100389
21. Dermawan JK, Rubin BP, Kilpatrick SE, Gjorgova Gjeorgjievski S, Fritchie KJ, Goldblum JR, McKenney JK, Billings SD. CD34-negative Solitary Fibrous Tumor: A Clinicopathologic Study of 25 Cases and Comparison with Their CD34-positive Counterparts. Am J Surg Pathol. 2021; 45(12):1616-1625. https://doi.org/10.1097/pas.0000000000001717
Downloads
Published
Issue
Section
License
Authors who publish with this journal agree to the following terms:
1) Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License (CC-BY-SA 4.0) that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
2) Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
3) Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website). The final published PDF should be used and bibliographic details that credit the publication in this journal should be included.
