Embryonal Tumor with Multilayered Rosettes in a 3-Year-Old Girl: Case Report.

Area of Science:

• Neuro-oncology
• Pediatric Pathology
• Cytogenetics

Background:

• Embryonal tumor with multilayered rosettes (ETMR) is a newly classified central nervous system (CNS) tumor.
• It exhibits features of both neuroblastoma and ependymoblastoma, primarily affecting children under five.
• A characteristic cytogenetic abnormality at 19q13.42 is associated with ETMR.

Observation:

• A case report details an ETMR in a 3-year-old girl with parietooccipital location.
• The patient presented with symptoms of increased intracranial pressure.
• Diagnosis was confirmed by the presence of true rosettes under low-power microscopy.

Findings:

• ETMR presents unique histopathological features distinct from other primitive neuroectodermal tumors (PNETs).
• The presence of true rosettes is a key diagnostic marker.
• A specific chromosomal abnormality at 19q13.42 is a hallmark of ETMR.

Implications:

• Further case studies are needed to differentiate ETMR from conventional PNETs.
• Reporting individual cases contributes valuable data on ETMR pathology, diagnosis, and treatment strategies.
• Enhanced understanding of ETMR can improve diagnostic accuracy and patient management in pediatric neuro-oncology.