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

Abstract

IntroductionEmbryonal 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 reportHerein, 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.DiscussionNeuropathological 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.ConclusionETANTR 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.

Incidence and survival of children and young people with central nervous system embryonal tumours in the North of England, 1990–2013

“…The main limitation of this study is the lack of information on molecular status of the tumours. Although distinct subgroups of medulloblastoma and CNS PNET have been established (7)(8)(9)(10)(11)(12)(13), these subgroups are not as yet classified as separate entities according to WHO or the ICCC-3.…”

“…The CNS PNET subgroup refers to a heterogeneous group of tumours that show aggressive clinical behaviour. Specific histotypes with distinct molecular features have recently been identified such as embryonal tumour with abundant neuropil and true rosettes (ETANTR) (10,11).…”

Embryonal Tumors with Multilayer Rosettes – Rare Central Nervous System Tumors in Infants

“…Všetky tri entity sa vyskytujú u detí v najnižšej vekovej kategórii (medián pod 3 roky), s miernou prevahou dievčat nad chlapcami [5]. Odpoveď na chemoterapiu a rádiotera-piu je zlá, celkové prežívanie (overall survival -OS) je menej ako 5-10 %, medián prežívania je 6-9 mesiacov [6][7][8].…”

Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas

“…There are but a few case reports or small case series in the literature of embryonal tumors, with the most commonly described example of this group being a primitive neuroectodermal tumor (PNET). 1,4,[12][13][14]38,40,44 We present our experience with these unusual and challenging neoplasms.…”