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.
References
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
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
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
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
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
Kaplan DR, Miller FD (2000) Neurotrophin signal transduction in the nervous system. Curr Opin Neurobiol 10(3):381–391
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
Berhouma M, Jemel H, Kchir N (2008) Calcified pilocytic astrocytoma of the medulla mimicking a brainstem “stone”. Pathologica 100(5):408–410
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
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):101–103
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):141–144
Funding
No funding was utilized for this study.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
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.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Johnson, D.R., Giannini, C., Jenkins, R.B. et al. Plenty of calcification: imaging characterization of polymorphous low-grade neuroepithelial tumor of the young. Neuroradiology 61, 1327–1332 (2019). https://doi.org/10.1007/s00234-019-02269-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00234-019-02269-y