Advertisement

Neuroradiology

, Volume 61, Issue 11, pp 1327–1332 | Cite as

Plenty of calcification: imaging characterization of polymorphous low-grade neuroepithelial tumor of the young

  • Derek R. JohnsonEmail author
  • Caterina Giannini
  • Robert B. Jenkins
  • Dong Kun Kim
  • Timothy J. Kaufmann
Short Report

Abstract

Purpose

Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a recently described epileptogenic neoplasm. As the name implies, PLNTYs are indolent tumors most often encountered in the pediatric or young adult population. The imaging features of PLNTY are not well characterized in the existing literature.

Methods

We performed a retrospective review, identifying nine patients with pathologically proven PLNTY and available preoperative imaging in order to identify common features which may facilitate confident imaging diagnosis of this entity.

Results

Patients were ages 5 to 34 years (median 16 years), and seven (78%) were female. Most tumors had a highly characteristic appearance, with temporal lobe location (6/9; 67%), calcification (8/9; 89%), cortical/subcortical origin (8/9; 89%), cystic components (8/9; 89%), and relatively infrequent contrast enhancement (3/9; 33%).

Conclusion

PLNTYs demonstrate characteristic calcification, subcortical location, and frequent temporal lobe localization, features that may allow radiologists to prospectively suggest the diagnosis in the proper clinical setting.

Keywords

Polymorphous low-grade neuroepithelial tumor of the young PLNTY Pediatric Brain tumor Calcification CT 

Notes

Funding

No funding was utilized for this study.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study formal consent is not required.

Informed consent

For this type of study formal consent is not required.

References

  1. 1.
    Huse JT, Rosenblum MK (2015) The emerging molecular foundations of pediatric brain tumors. J Child Neurol 30(13):1838–1850.  https://doi.org/10.1177/0883073815579709 CrossRefPubMedGoogle Scholar
  2. 2.
    Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131(6):803–820.  https://doi.org/10.1007/s00401-016-1545-1 CrossRefPubMedGoogle Scholar
  3. 3.
    Bitar M, Danish SF, Rosenblum MK (2018) A newly diagnosed case of polymorphous low-grade neuroepithelial tumor of the young. Clin Neuropathol 37(4):178–181.  https://doi.org/10.5414/np301081 CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Huse JT, Snuderl M, Jones DT, Brathwaite CD, Altman N, Lavi E, Saffery R, Sexton-Oates A, Blumcke I, Capper D, Karajannis MA, Benayed R, Chavez L, Thomas C, Serrano J, Borsu L, Ladanyi M, Rosenblum MK (2017) Polymorphous low-grade neuroepithelial tumor of the young (PLNTY): an epileptogenic neoplasm with oligodendroglioma-like components, aberrant CD34 expression, and genetic alterations involving the MAP kinase pathway. Acta Neuropathol 133(3):417–429.  https://doi.org/10.1007/s00401-016-1639-9 CrossRefPubMedGoogle Scholar
  5. 5.
    Jones DT, Hutter B, Jager N, Korshunov A, Kool M, Warnatz HJ, Zichner T, Lambert SR, Ryzhova M, Quang DA, Fontebasso AM, Stutz AM, Hutter S, Zuckermann M, Sturm D, Gronych J, Lasitschka B, Schmidt S, Seker-Cin H, Witt H, Sultan M, Ralser M, Northcott PA, Hovestadt V, Bender S, Pfaff E, Stark S, Faury D, Schwartzentruber J, Majewski J, Weber UD, Zapatka M, Raeder B, Schlesner M, Worth CL, Bartholomae CC, von Kalle C, Imbusch CD, Radomski S, Lawerenz C, van Sluis P, Koster J, Volckmann R, Versteeg R, Lehrach H, Monoranu C, Winkler B, Unterberg A, Herold-Mende C, Milde T, Kulozik AE, Ebinger M, Schuhmann MU, Cho YJ, Pomeroy SL, von Deimling A, Witt O, Taylor MD, Wolf S, Karajannis MA, Eberhart CG, Scheurlen W, Hasselblatt M, Ligon KL, Kieran MW, Korbel JO, Yaspo ML, Brors B, Felsberg J, Reifenberger G, Collins VP, Jabado N, Eils R, Lichter P, Pfister SM (2013) International Cancer genome consortium PedBrain tumor P (2013) recurrent somatic alterations of FGFR1 and NTRK2 in pilocytic astrocytoma. Nat Genet 45(8):927–932.  https://doi.org/10.1038/ng.2682 CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Kaplan DR, Miller FD (2000) Neurotrophin signal transduction in the nervous system. Curr Opin Neurobiol 10(3):381–391CrossRefGoogle Scholar
  7. 7.
    Gupta K, Harreld JH, Sabin ND, Qaddoumi I, Kurian K, Ellison DW (2014) Massively calcified low-grade glioma - a rare and distinctive entity. Neuropathol Appl Neurobiol 40(2):221–224.  https://doi.org/10.1111/nan.12080 CrossRefPubMedGoogle Scholar
  8. 8.
    Berhouma M, Jemel H, Kchir N (2008) Calcified pilocytic astrocytoma of the medulla mimicking a brainstem “stone”. Pathologica 100(5):408–410PubMedGoogle Scholar
  9. 9.
    Kim YE, Shin HJ, Suh YL (2012) Pilocytic astrocytoma with extensive psammomatous calcification in the lateral ventricle: a case report. Child’s Nerv Syst 28(4):649–652.  https://doi.org/10.1007/s00381-011-1650-3 CrossRefGoogle Scholar
  10. 10.
    Niimi M, Yoshida K, Mayanagi K, Kawase T (2002) Extensive and dense calcification in the core of a ventrally exophytic brainstem glioma. Brain Tumor Pathol 19(2):101–103CrossRefGoogle Scholar
  11. 11.
    Tamura M, Kohga H, Ono N, Zama A, Shibasaki T, Horikoshi S, Kurihara H, Ohye C (1995) Calcified astrocytoma of the amygdalo-hippocampal region in children. Child's Nerv Syst 11(3):141–144CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of RadiologyMayo ClinicRochesterUSA
  2. 2.Department of Laboratory Medicine and PathologyMayo ClinicRochesterUSA

Personalised recommendations