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Acta Neuropathologica

, Volume 135, Issue 3, pp 485–488 | Cite as

Multinodular and vacuolating neuronal tumor of the cerebrum is a clonal neoplasm defined by genetic alterations that activate the MAP kinase signaling pathway

  • Melike Pekmezci
  • Meredith Stevers
  • Joanna J. Phillips
  • Jessica Van Ziffle
  • Boris C. Bastian
  • Nadejda M. Tsankova
  • Bette K. Kleinschmidt-DeMasters
  • Marc K. Rosenblum
  • Tarik Tihan
  • Arie Perry
  • David A. Solomon
Correspondence

Initially described in 2013, multinodular and vacuolating neuronal tumor of the cerebrum (MVNT) is a low-grade neuronal neoplasm of the cerebral hemispheres composed of small to medium sized neuronal cells arranged in nodules involving the deep cortex and subcortical white matter, showing prominent intracytoplasmic and stromal vacuolation [8]. The tumor cells are typically immunopositive with some glial and neuronal markers (OLIG2 and synaptophysin), but negative for others (GFAP and NeuN), and are associated with ramified CD34-positive processes in adjacent parenchyma. The radiologic features of MVNT are distinct and include predominantly solid, T2-hyperintense lesions in the deep cortical ribbon and superficial white matter with variable internal nodularity and typically absent contrast enhancement [1, 8, 11]. Patients may be asymptomatic or can present with seizures or headaches, and all pathologically proven cases have reportedly followed a benign clinical course to date [1, 8, 11]...

Notes

Acknowledgements

B.C.B. is supported by an NCI Outstanding Investigator Award (R35 CA220481). D.A.S. is supported by NIH Director’s Early Independence Award (DP5 OD021403) and the UCSF Physician-Scientist Scholar Program. We thank the UCSF Brain Tumor Research Center (supported by NIH SPORE Grant P50 CA097257) for assistance with phospho-ERK immunohistochemistry.

Compliance with ethical standards

This study was approved by the Committee on Human Research of the University of California, San Francisco, with a waiver of patient consent.

Conflict of interest

The authors declare that they have no competing interests related to this study.

Supplementary material

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Supplementary material 1 (XLSX 20 kb)
401_2018_1820_MOESM2_ESM.pdf (4.1 mb)
Supplementary material 2 (PDF 4230 kb)

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Melike Pekmezci
    • 1
  • Meredith Stevers
    • 1
  • Joanna J. Phillips
    • 1
    • 2
  • Jessica Van Ziffle
    • 1
    • 3
  • Boris C. Bastian
    • 1
    • 3
  • Nadejda M. Tsankova
    • 4
  • Bette K. Kleinschmidt-DeMasters
    • 5
  • Marc K. Rosenblum
    • 6
  • Tarik Tihan
    • 1
  • Arie Perry
    • 1
    • 2
  • David A. Solomon
    • 1
    • 3
  1. 1.Department of PathologyUniversity of California, San FranciscoSan FranciscoUSA
  2. 2.Department of Neurological SurgeryUniversity of CaliforniaSan FranciscoUSA
  3. 3.Clinical Cancer Genomics LaboratoryUniversity of CaliforniaSan FranciscoUSA
  4. 4.Departments of Pathology and NeuroscienceMount Sinai Hospital, Icahn School of MedicineNew YorkUSA
  5. 5.Departments of Pathology, Neurology, and NeurosurgeryUniversity of ColoradoAuroraUSA
  6. 6.Department of PathologyMemorial Sloan Kettering Cancer CenterNew YorkUSA

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