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SAGE Open Med Case Rep. 2017 Dec 4;5:2050313X17745208. doi: 10.1177/2050313X17745208. eCollection 2017.

Embryonal tumor with multilayered rosettes, C19MC-altered: Report of an extremely rare malignant pediatric central nervous system neoplasm.

Author information

1
Section of Histopathology, Department of Pathology and Laboratory Medicine, The Aga Khan University, Karachi, Pakistan.
2
Section of Paediatric Oncology, Department of Oncology, The Aga Khan University, Karachi, Pakistan.
3
Department of Paediatric Laboratory Medicine, The Hospital for Sick Children (Sickkids), Toronto, ON, Canada.

Abstract

The 2016 update of the WHO Classification of Tumours of the Central Nervous System has redefined a number of tumors. Embryonal tumor with multilayered rosettes, C19MC-altered is one such tumor entity which has been newly defined on the basis of a characteristic molecular alteration. We report, to our knowledge, the first case of this rare pediatric brain neoplasm in the Pakistani population. An 8-month-old girl was presented with vomiting and left-sided ptosis, and magnetic resonance imaging scan showed a cerebellar tumor. Histologically, a highly cellular population of primitive cells was seen alternating with hypocellular neuropil-rich regions containing multilayered true rosettes and cells with glial and neuronal differentiation. Amplification of 19q13. 42 chromosome region on fluorescence in situ hybridization analysis confirmed the diagnosis. Post-operative radiological examination revealed widespread central nervous system involvement. Adjuvant treatment was not offered due to complications. Patient expired a week after diagnosis.

KEYWORDS:

C19MC; Central nervous system–primitive neuroectodermal tumor; LIN28A; embryonal tumor with multilayered rosettes; multilayered rosettes

Conflict of interest statement

Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article.

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