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Brain Tumor Pathology

, Volume 36, Issue 1, pp 20–26 | Cite as

A rapidly fatal intracranial anaplastic hemangiopericytoma with de-novo dedifferentiation: emphasis on diagnostic recognition, molecular confirmation and discussion on treatment dilemma

  • N. J. H. Tan
  • I. S. Y. Sun
  • S. W. Low
  • C. H. Kuick
  • K. T. E. Chang
  • C. L. TanEmail author
Case Report

Abstract

Solitary fibrous tumors/ hemangiopericytomas (SFT/HPC) are mesenchymal tumors that share a common genetic aberration and very rarely undergo dedifferentiation. We report a unique case of an intracranial anaplastic SFT/HPC with de-novo dedifferentiation, which pursued a rapidly fatal clinical course in a 41-year-old lady. The dedifferentiated component comprised a focal area of glandular formation with epithelial immunophenotype acquisition. The distinct biphasic pattern of the tumor imparted great diagnostic challenges to the pathologists. An increased awareness of SFT/HPCs with a diverse morphologic spectrum or even a biphasic histologic pattern is essential in working up such cases. We first attempted gamma knife radiosurgery in treating a recurrent dedifferentiated SFT/HPC; unfortunately it was to no avail. Although it is now known that SFT/HPC is characterized by NAB2-STAT6 gene fusion, the unavailability of targeted therapy against this molecular signature still results in a treatment dilemma.

Keywords

Solitary fibrous tumor Hemangiopericytoma STAT6 Intracranial Dedifferentiation Gamma knife 

Notes

Acknowledgements

Targeted next generation sequencing and RT-PCR of the tumor were funded by the VIVA-KKH Paediatric Brain and Solid Tumour Programme.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Mosquera J-M, Fletcher CDM (2009) Expanding the spectrum of malignant progression in solitary fibrous tumors: a study of 8 cases with a discrete anaplastic component—is this dedifferentiated SFT? Am J Surg Pathol 33:1314–1321CrossRefGoogle Scholar
  2. 2.
    Olson NJ, Linos K (2018) Dedifferentiated solitary fibrous tumor: a concise review. Arch Pathol Lab Med 142:761–766.  https://doi.org/10.5858/arpa.2016-0570-RS CrossRefGoogle Scholar
  3. 3.
    Lu C, Alex D, Benayed R, Rosenblum M, Hameed M (2018) Solitary fibrous tumor with neuroendocrine and squamous dedifferentiation: a potential diagnostic pitfall. Hum Pathol 77:175–180.  https://doi.org/10.1016/j.humpath.2017.12.024 CrossRefGoogle Scholar
  4. 4.
    Maekawa A, Kohashi K, Yamada Y, Nakamizo A, Yoshimoto K, Mizoguchi M et al (2014) A case of intracranial solitary fibrous tumor/hemangiopericytoma with dedifferentiated component. Neuropathology 35:260–265.  https://doi.org/10.1111/neup.12181 CrossRefGoogle Scholar
  5. 5.
    Moritani S, Ichihara S, Hasegawa M, Takada S, Takahashi T, Kato E et al (2010) Dedifferentiation and progression of an intracranial solitary fibrous tumor: autopsy case of a Japanese woman with a history of radiation therapy of the head during infancy. Pathol Int 61:143–149.  https://doi.org/10.1111/j.1440-1827.2010.02627.x CrossRefGoogle Scholar
  6. 6.
    Pekmezci M, Vlodavsky E, Perry A (2014) Previously unrecognized pattern of central nervous system hemangiopericytoma with pseudoglandular spaces. Clin Neuropathol 33:186–189CrossRefGoogle Scholar
  7. 7.
    Fletcher CDM, World Health Organization, International Agency for Research on Cancer (2013) WHO classification of tumours of soft tissue and bone. World Health Organization classification of tumours, 4th edn. IARC Press, LyonGoogle Scholar
  8. 8.
    Marguet F, Proust F, Crahes M, Basset C, Joly-Helas G, Chambon P et al (2014) Le méningiome malin avec métaplasie adénocarcinomateuse: une entité rare à ne pas méconnaître. Ann Pathol 34:223–227.  https://doi.org/10.1016/j.annpat.2014.03.004 CrossRefGoogle Scholar
  9. 9.
    Patil S, Scheithauer BW, Strom RG, Mafra M, Chicoine MR, Perry A (2011) Malignant meningiomas with epithelial (adenocarcinoma-like) metaplasia: a study of 3 cases. Neurosurgery 69:884–892.  https://doi.org/10.1227/NEU.0b013e318222dc6f CrossRefGoogle Scholar
  10. 10.
    Healy V, O’Halloran P, O’Brien S, Beausang A, Caird J (2017) CNS metastasis secondary to malignant-mixed Müllerian tumor: case report and review of therapeutics. CNS Oncol 6:315–323.  https://doi.org/10.2217/cns-2017-0015 CrossRefGoogle Scholar
  11. 11.
    Voorhies J, Hattab EM, Cohen-Gadol AA (2013) Malignant peripheral nerve sheath tumor of the abducens nerve and a review of the literature. World Neurosurg 80:654–658CrossRefGoogle Scholar
  12. 12.
    Mrowczynski O, Greiner R, Kapadia M et al (2018) Intracranial malignant peripheral nerve sheath tumor variant: an unusual neurovascular phenotype sarcoma case invading through the petrous bone. Childs Nerv Syst 34:1605–1608CrossRefGoogle Scholar
  13. 13.
    Lin Y-J, Yang Q-x, Tian X-y, Li B, Li Z (2012) Unusual primary intracranial dural-based poorly differentiated synovial sarcoma with t(X; 18)(p11; q11). Neuropathology 33:75–82.  https://doi.org/10.1111/j.1440-1789.2012.01320.x CrossRefGoogle Scholar
  14. 14.
    Sharma S, Sharma A, Lobo G, Nayak M, Pradhan D, Samriti et al (2017) Primary dura-based synovial sarcoma of the parafalcine region of brain. Pathol Res Pract 213:868–871.  https://doi.org/10.1016/j.prp.2017.03.005 CrossRefGoogle Scholar
  15. 15.
    Xiao G, Pan B, Tian X et al (2014) Synovial sarcoma in cerebellum: a case report and literature review. Brain Tumor Pathol 31:68–75CrossRefGoogle Scholar
  16. 16.
    Nakada S, Minato H, Nojima T (2016) Clinicopathological differences between variants of the NAB2–STAT6 fusion gene in solitary fibrous tumors of the meninges and extra-central nervous system. Brain Tumor Pathol 33Google Scholar
  17. 17.
    Chuang IC, Liao K-C, Huang H-Y, Kao Y-C, Li C-F, Huang S-C et al (2016) NAB2-STAT6 gene fusion and STAT6 immunoexpression in extrathoracic solitary fibrous tumors: the association between fusion variants and locations. Pathol Int 66:288–296.  https://doi.org/10.1111/pin.12408 CrossRefGoogle Scholar
  18. 18.
    Fritchie KJ, Jin L, Rubin BP, Burger PC, Jenkins SM, Barthelmeß S et al (2016) NAB2-STAT6 gene fusion in meningeal hemangiopericytoma and solitary fibrous tumor. J Neuropathol Exp Neurol 75:263–271.  https://doi.org/10.1093/jnen/nlv026 CrossRefGoogle Scholar
  19. 19.
    Huang SC, Li CF, Kao YC, Chuang IC, Tai HC, Tsai JW et al (2016) The clinicopathological significance of NAB2-STAT6 gene fusions in 52 cases of intrathoracic solitary fibrous tumors. Cancer Med 5:159–168.  https://doi.org/10.1002/cam4.572 CrossRefGoogle Scholar
  20. 20.
    Tai H-C, Chuang IC, Chen T-C, Li C-F, Huang S-C, Kao Y-C et al (2015) NAB2–STAT6 fusion types account for clinicopathological variations in solitary fibrous tumors. Mod Pathol 28:1324.  https://doi.org/10.1038/modpathol.2015.90 https://www.nature.com/articles/modpathol201590#supplementary-information. Accessed 16 Sept 2018
  21. 21.
    Barthelmeß S, Geddert H, Boltze C, Moskalev EA, Bieg M, Sirbu H et al. (2014) Solitary fibrous tumors/hemangiopericytomas with different variants of the < em> NAB2</em>- STAT6 gene fusion are characterized by specific histomorphology and distinct clinicopathological features. Am J Pathol 184:1209–1218.  https://doi.org/10.1016/j.ajpath.2013.12.016 CrossRefGoogle Scholar
  22. 22.
    Yoshida A, Tsuta K, Ohno M, Yoshida M, Narita Y, Kawai A et al (2014) STAT6 Immunohistochemistry is helpful in the diagnosis of solitary fibrous tumors. Am J Surg Pathol 38:552–559CrossRefGoogle Scholar
  23. 23.
    Doyle LA, Vivero M, Fletcher CDM, Mertens F, Hornick JL (2013) Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol 27:390.  https://doi.org/10.1038/modpathol.2013.164 CrossRefGoogle Scholar
  24. 24.
    Dagrada GP, Spagnuolo RD, Mauro V, Tamborini E, Cesana L, Gronchi A et al (2015) Solitary fibrous tumors: loss of chimeric protein expression and genomic instability mark dedifferentiation. Mod Pathol 28:1074.  https://doi.org/10.1038/modpathol.2015.70 https://www.nature.com/articles/modpathol201570#supplementary-information. Accessed 25 Sept 2018
  25. 25.
    Schneider N, Hallin M, Thway K (2016) STAT6 loss in dedifferentiated solitary fibrous tumor. Int J Surg Pathol 25:58–60.  https://doi.org/10.1177/1066896916650257 CrossRefGoogle Scholar
  26. 26.
    Collini P, Negri T, Barisella M, Palassini E, Tarantino E, Pastorino U et al (2012) High-grade sarcomatous overgrowth in solitary fibrous tumors: a clinicopathologic study of 10 cases. Am J Surg Pathol 36:1202–1215.  https://doi.org/10.1097/PAS.0b013e31825748f0 CrossRefGoogle Scholar
  27. 27.
    da Ros M, Iorio AL, Lucchesi M, Stival A, de Martino M, Sardi I (2015) The use of anthracyclines for therapy of CNS tumors. Anticancer Agents Med Chem 15:721–727CrossRefGoogle Scholar
  28. 28.
    Mindermann T, Reisch R (2014) Mulitmodality management of rare solitary fibrous tumor can be associated with extended survival. Surg Neurol Int 5:S590–S592.  https://doi.org/10.4103/2152-7806.148058 CrossRefGoogle Scholar
  29. 29.
    Reames DL, Mohila CA, Sheehan JP (2011) Treatment of intracranial solitary fibrous tumors with gamma knife radiosurgery: report of two cases and review of literature. Neurosurgery 69:E1023–E1028.  https://doi.org/10.1227/NEU.0b013e318223b7e6 (discussion E1028)CrossRefGoogle Scholar

Copyright information

© The Japan Society of Brain Tumor Pathology 2019

Authors and Affiliations

  1. 1.Department of PathologyNational University Health SystemSingaporeSingapore
  2. 2.Division of Neurosurgery, Department of SurgeryNg Teng Fong General HospitalSingaporeSingapore
  3. 3.Department of Pathology and Laboratory MedicineKK Women’s and Children’s HospitalSingaporeSingapore

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