Embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature

作者：Adamek Dariusz^*; Sofowora Kolawole D; Cwiklinska Magdalena; Herman Sucharska Izabela; Kwiatkowski Stanislaw

来源：Child's Nervous System, 2013, 29(5): 849-854.

DOI：10.1007/s00381-013-2037-4

• ETANTR
• Embryonal tumor with abundant neuropil and true rosettes
• Pediatric brain tumor
• Immunohistochemistry
• Ependymoblastoma
• Primitive neuroectodermal tumor

Introduction Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a rare subtype of primitive neuroectodermal tumors first reported in 2000. It is rare among the group of embryonal central nervous system tumors with approximately 50 reported cases. ETANTR has been suggested to be a separate entity among this group of tumors.
Case report Herein, we present only the second autopsy case of ETANTR, which occurred in a 17-month-old boy, and was located in the brainstem. The tumor was inoperable, and despite chemotherapy, the child died 3 months after initial hospitalization. A brain only autopsy was performed.
Discussion Neuropathological and neuroimaging examinations suggest ETANTR grew by expansion rather than invasion distorting anatomical structures of the posterior fossa. We suggest that the characteristic histopathological picture of the tumor is the result of multifocal and dispersed germinative activity surrounded by mature neuropil-like areas.
Conclusion ETANTR is a pediatric tumor occurring in children under 4 with a significantly poor prognosis with more cases and research required to characterize this rare embryonal tumor.

• 出版日期2013-5