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Mesenchymal, Non-meningothelial Tumors

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Neuropathology of Brain Tumors with Radiologic Correlates

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Abstract

Meninges are more commonly affected than the brain parenchyma. Supratentorial location is more commonly involved than the infratentorial. The histological features correspond to the extracranial soft tissue and bone tumors. The cell of origin could be meninges, vasculature, or osseous structures. The WHO classification of CNS tumors, 2016 includes the spectrum of hemangiopericytoma/solitary fibrous tumor, hemangioblastoma, hemangioma, epithelioid hemangioendothelioma, angiosarcoma, Kaposi sarcoma, Ewing sarcoma/PNET, lipoma, liposarcoma, desmoid-type fibromatosis, myofibroblastoma, inflammatory myofibroblastic tumor, fibrosarcoma, undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma, leiomyoma, leiomyosarcoma, rhabdomyoma, rhabdomyosarcoma, chondroma, chondrosarcoma, osteoma, osteochondroma, and osteosarcoma. They range from benign grade I to highly malignant sarcoma, grade IV. Specific antibodies are needed for precise diagnosis. Detection of STAT6 nuclear expression or NAB2-STAT6 fusion is highly recommended to confirm the diagnosis of hemangiopericytoma/solitary fibrous tumor on immunohistochemistry. FLI 1 is a sensitive marker, however not specific, and maybe expressed by other tumors. RT-PCR for the presence of EWSR1-FLI1 or EWSR1-ERG fusion transcript or by FISH for EWSR1-gene rearrangement is confirmatory for Ewing sarcoma. Inhibin is the specific marker in stromal cells of hemangioblastoma and brachyury for chordoma.

The chapter illustrates multiple MR images and findings, intraoperative cytology, histopathology, IHC pictures, and their features along with differential diagnosis for each of the above, genetic profile, prognosis, and case studies.

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References

  1. Schweizer L, Koelsche C, Sahm F, Piro RM, Capper D, 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 May;125(5):651–8.

    Article  CAS  Google Scholar 

  2. Osborn A. Osborn's brain. 2nd ed. Salt Lake City, Utah: Amirsys Pub; 2013. p. 683–6.

    Google Scholar 

  3. Osborn A, Salzman K, Jhaveri M. Diagnostic imaging: Brain. 3rd ed. Philadelphia: Elsevier; 2016. p. 563.

    Google Scholar 

  4. Perry A, Scheithauer BW, Nascimento AG. The Immunophenotypic Spectrum of meningeal Hemangiopericytoma: a comparison with fibrous meningioma and solitary fibrous tumor of meninges. Am J Surg Pathol. 1997 Nov;21(11):1354–60.

    Article  CAS  Google Scholar 

  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 Aug;106(2):217–24.

    Article  CAS  Google Scholar 

  6. Probst-Cousin S, et al. Ki-67 and biological behaviour in meningeal haemangiopericytomas. Histopathology. 1996 Aug;29(1):57–61.

    Article  CAS  Google Scholar 

  7. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. World Health Organization classification of tumors of the central nervous system. Lyon, France: IARC; 2016. Mesenchymal, non-meningothelial tumors, page 249–254

    Google Scholar 

  8. Cherekaev VA, Kushel YUV, Shkarubo AN. Primary and metastatic Ewing’s sarcoma of the Skull Base — case reports and comparative analysis, N.N. Burdenko. J Neurosurg. 2013;1:27–32.

    Google Scholar 

  9. Lin L, Varikatt W, Dexter M. Ng T; diagnostic pitfall in the diagnosis of mesenchymal chondrosarcoma arising in the central nervous system. Neuropathology. 2012 Feb;32(1):82–90. https://doi.org/10.1111/j.1440-1789.2011.01224.x.

    Article  PubMed  Google Scholar 

  10. Bridge RS, Rajaram V, Dehner LP, Pfeifer JD, Perry A. Molecular diagnosis of Ewing sarcoma/primitive neuroectodermal tumor in routinely processed tissue; comparision of two FISH strategies and RT-PCR in malignant round cell tumors. Mod Pathol. 2006;19(1):1–8.

    Article  CAS  Google Scholar 

  11. Fountas KN, Stamatiou S, Barbanis S, Kourtopoulos H. Intracranial falx chondroma: literature review and a case report. Clin Neurol Neurosurg. January 2008;110(1):8–13.

    Article  Google Scholar 

  12. Osborn A. Osborn’s brain. 2nd ed. Salt Lake City, Utah: Amirsys Pub; 2013. p. 674–6.

    Google Scholar 

  13. Scheithauer BW, Rubinstein LJ. Meningeal mesenchymal chondrosarcoma: report of 8 cases with review of the literature; cancer. Dec. 1978;42(6):2744–52.

    CAS  Google Scholar 

  14. Malik SN, Farmer PM, Hajdu SI, Rosenthal A. Mesenchymal chondrosarcoma of the cerebellum. Ann Clin Lab Sci. 1996 Nov-Dec;26(6):496–500.

    PubMed  CAS  Google Scholar 

  15. Harnsberger HR. Diagnostic imaging. Head and neck. Salt Lake City, Ut: Amirsys; 2004. p. I-358–61.

    Google Scholar 

  16. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. World Health Organization classification of tumors of the central nervous system. Lyon, France: IARC; 2016.Chondrosarcoma,page 264

    Google Scholar 

  17. Harnsberger HR. Diagnostic imaging. Head and neck. Salt Lake City, Ut: Amirsys; 2004. p. I-3 10–3.

    Google Scholar 

  18. Oakley GJ, Fuhrer K, Seethala RR. Brachyury, SOX-9, and podoplanin, new markers in the skull base chordoma vs chondrosarcoma differential: a tissue microarray-based comparative analysis. Mod Pathol. 2008;21(12):1461–9.

    Article  CAS  Google Scholar 

  19. Osborn A. Osborn's brain. 2nd ed. Salt Lake City, Utah: Amirsys Pub; 2013. p. 688–91.

    Google Scholar 

  20. Osborn A, Salzman K, Jhaveri M. Diagnostic imaging: brain. 3rd ed. Philadelphia: Elsevier; 2016. p. 559.

    Google Scholar 

  21. Wanebo JE, Lonser RR, Glenn GM, Oldfield EH. WHO. 2683, the natural history of Heamangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg. 2003 Jan;98(1):82–94.

    Article  Google Scholar 

  22. Intraoperative diagnosis of CNS tumors, Heamangioblastoma, 140.

    Google Scholar 

  23. Shuji Hamazaki, Hiroyuki Nakashima, Kengo Matsumoto, Kohji Taguch, Shigeru Okada; Metastasis of renal cell carcinoma to central nervous system Heamangioblastoma in two patients with von Hippel–Lindau disease,

    Google Scholar 

  24. Inhibin Alpha Distinguishes Heamangioblastoma From Clear Cell Renal Cell Carcinoma, Article in American Journal of Surgical Pathology 2003 Sep;27(8):1152–6.

    Google Scholar 

  25. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, et al. World Health Organization classification of tumors of the central nervous system. Lyon, France: IARC; 2016. Heamangioblastoma, page no. 256–257

    Google Scholar 

  26. Kelleher T, Aquilina K, Keohane C, O’Sullivan MG. Intramedullary capillary haemangioma. Br J Neurosurg. 2005 Aug;19(4):345–8.

    Article  CAS  Google Scholar 

  27. Simon SL, Moonis G, Judkins AR, Scobie J, et al. Intracranial capillary hemangioma: case report and review of the literature. Surg Neurol. 2005 Aug;64(2):154–9.

    Article  Google Scholar 

  28. Osborn A, Salzman K, Jhaveri M. Diagnostic imaging: brain 3rd ed. Philadelphia: Elsevier; 2016. p. 1185–7.

    Google Scholar 

  29. Chang MW. Updated classification of hemangiomas and other vascular anomalies. Lymphat Res Biol. 2003;1(4):259–65.

    Article  Google Scholar 

  30. Loddenkemper T, Morris HH, Diehl B, et al. Intracranial lipomas and epilepsy. J Neurol. 2006;253:590–3. https://doi.org/10.1007/s00415-006-0065-7.

    Article  PubMed  Google Scholar 

  31. Jabot G, Elsankari S, Saliou G, Toussaint P, Deramond H, Lehmann P. Intracranial lipomas: Clinical appearances on neuroimaging and clinical significance. J Neurol. 2009;256:851–5. https://doi.org/10.1007/s00415-009-5087-5.

    Article  PubMed  Google Scholar 

  32. Osborn A. Osborn’s brain. 2nd ed. Salt Lake City, Utah: Amirsys Pub; 2013. p. 1311–4.

    Google Scholar 

  33. Osborn A, Salzman K, Jhaveri M. Diagnostic imaging brain. 3rd ed. Philadelphia: Elsevier; 2016. p. 22–5.

    Google Scholar 

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Authors and Affiliations

  1. Shanti Pathology Laboratory, Kolhapur, Maharashtra, India

    Meghana Chougule

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  1. Meghana Chougule
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Chougule, M. (2020). Mesenchymal, Non-meningothelial Tumors. In: Neuropathology of Brain Tumors with Radiologic Correlates. Springer, Singapore. https://doi.org/10.1007/978-981-15-7126-8_14

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  • DOI: https://doi.org/10.1007/978-981-15-7126-8_14

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  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-15-7125-1

  • Online ISBN: 978-981-15-7126-8

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