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Embryonal tumor with multilayered rosettes: Post-treatment maturation and implications for future therapy.
Francesca M Gualano1,2, Patrice Hassoun3, Claire L Carter1,2
1Department of Pathology, Hackensack Meridian School of Medicine, Nutley, New Jersey, USA.
Cancer Reports (Hoboken, N.J.)
|March 25, 2023
Summary
Embryonal tumor with multilayered rosettes (ETMR), a rare pediatric brain tumor, showed long-term survival after treatment-induced cell maturation. This suggests less toxic therapies targeting differentiation may be effective.
Area of Science:
- Pediatric neuro-oncology
- Cancer biology
- Developmental neurobiology
Background:
- Embryonal tumor with multilayered rosettes (ETMR) is a rare, aggressive pediatric brain tumor with poor prognosis.
- Current treatments including surgery, chemotherapy, and radiotherapy yield limited long-term survival rates below 30% at 5 years.
Observation:
- A case report details a 5-month-old female diagnosed with ETMR presenting a large posterior fossa mass.
- Histopathology revealed highly proliferative, undifferentiated cells with multilayered rosettes pre-treatment.
- Post-treatment, histopathology showed significant differentiation into neuronal and ganglionic phenotypes.
Findings:
- The patient achieved long-term progression-free survival for 5 years following treatment-induced tumor cell maturation.
- This case represents one of the few documented instances of ETMR post-treatment differentiation.
Implications:
- Findings suggest that ETMR cell populations can mature and differentiate in response to therapy.
- This opens avenues for developing less cytotoxic therapeutic strategies focused on promoting tumor cell differentiation rather than solely relying on aggressive treatments.
