Embryonal tumor with abundant neuropil and true rosettes (ETANTR): report of a case with prominent neurocytic differentiation

Dunham, Christopher; Sugo, Ella; Tobias, Vivienne; Wills, E. J.; Perry, Arie

doi:10.1007/s11060-007-9346-y

Cited by 47 publications

(52 citation statements)

References 12 publications

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“…13,14 These tumors include glioneuronal tumors, central and extraventricular neurocytomas, as well as other benign tumors with minor neurocytic elements (rosette-forming glioneuronal tumor, papillary glioneuronal tumors, and cerebellar liponeurocytomas). The same is not true of ependymomas and astrocytomas featuring neuropil islands containing neurocytes and occasionally small ganglion cells.…”

Section: Discussionmentioning

confidence: 99%

Atypical Pituitary Adenoma With Neurocytic Transformation

Rotondo

Bernardo

Scheithauer

et al. 2014

Applied Immunohistochemistry &Amp; Molecular Morphology

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Here, we report an example of an atypical prolactin-producing pituitary adenoma showing clear morphologic and immunohistochemical evidence of neurocytic transformation. Its features support the concept that neoplastic neuroendocrine cells, in this case adenohypophyseal cells, are capable of neuronal differentiation and broaden the morphologic spectrum of such rare tumors. Our findings have implications with respect to the nosology of neuronal tumors of the adenohypophysis.

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Section: Discussionmentioning

confidence: 99%

Atypical Pituitary Adenoma With Neurocytic Transformation

Rotondo

Bernardo

Scheithauer

et al. 2014

Applied Immunohistochemistry &Amp; Molecular Morphology

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“…In 2010, Buccoliero and colleagues 3 described a 2-year-old girl with a bihemispheric lesion of the parietooccipital lobes that was completely removed in 2 stages. In 2007, Dunham and colleagues 5 reported on a 2-year-old boy with a partly calcified cystic left temporoparietal hemispheric ETANTR who underwent near-total resection with no adjuvant therapy. He developed a posterior fossa tumor recurrence after 11 months necessitating urgent debulking.…”

Section: Discussionmentioning

confidence: 99%

Embryonal tumors with abundant neuropil and true rosettes: 2 illustrative cases and a review of the literature

Manjila¹,

Ray²,

Hu³

et al. 2011

FOC

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Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently identified variant of primitive neuroectodermal tumor, with fewer than 50 cases reported in the literature to date. Histologically, this tumor has features of ependymoblastoma and neuroblastoma, demonstrating areas of fine fibrillary neuropil intermingled with ependymoblastic rosettes and zones of undifferentiated neuroepithelial cells. However, ETANTR is distinguished pathologically from other embryonal tumors by the striking abundance of neuropil. Clinically, ETANTRs have shown high malignant potential and poor clinical outcome despite aggressive treatment. The authors describe 2 illustrative surgical cases of ETANTR, one involving the longest reported survival in the literature to date. The other had a poor outcome despite high-dose adjuvant chemotherapy with sequential autologous hematopoietic stem cell rescue. The authors review the natural history and treatment strategies available for this unusual malignant pediatric brain tumor.

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“…An increased frequency of chromosome 2 gain was observed in ependymoblastoma and ETANTR [18, 34, 37, 88]. More recently, a highly specific focal amplification at chromosome band 19q13.42 containing a cluster of mi-RNA-coding genes was found in virtually all embryonal brain tumors with true multilayered rosettes [63, 66, 84] (Fig.…”

Section: Cns Primitive Neuroectodermal Tumors (Pnets)mentioning

confidence: 99%

Molecular diagnostics of CNS embryonal tumors

et al. 2010

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Tremendous progress has recently been made in both molecular subgrouping, and the establishment of prognostic biomarkers for embryonal brain tumors, particularly medulloblastoma. Several prognostic biomarkers that were initially identified in retrospective cohorts of medulloblastoma, including MYC and MYCN amplification, nuclear β-catenin accumulation, and chromosome 17 aberrations have now been validated in clinical trials. Moreover, molecular subgroups based on distinct transcriptome profiles have been consistently reported from various groups on different platforms demonstrating that the concept of distinct medulloblastoma subgroups is very robust. Well-described subgroups of medulloblastomas include tumors showing wingless signaling pathway (Wnt) activation, and another characterized by sonic hedgehog pathway activity. Two or more additional subgroups were consistently reported to contain the vast majority of high-risk tumors, including most tumors with metastatic disease at diagnosis and/or large cell/anaplastic histology. Several years ago, atypical teratoid rhabdoid tumor (AT/RT) was recognized as a separate entity based on its distinct biology and particularly aggressive clinical behavior. These tumors may occur supra or infratentorially and are usually found to have genetic alterations of SMARCB1 (INI1/hSNF5), a tumor suppressor gene located on chromosome 22q. Subsequent loss of SMARCB1 protein expression comprises a relatively specific and sensitive diagnostic marker for AT/RT. For CNS primitive neuroectodermal tumors (CNS PNETs), a consistent finding has been that they are molecularly distinct from medulloblastoma. Furthermore, a distinct fraction of CNS PNETs with particularly poor prognosis only occurring in young children was delineated, which was previously labeled ependymoblastoma or embryonal tumor with abundant neuropil and true rosettes (ETANTR) and which is morphologically characterized by the presence of multilayered “ependymoblastic” rosettes. This group of tumors shows a unique cytogenetic abnormality not seen in other brain tumors: focal amplification of a micro-RNA cluster at chromosome 19q13.42, which has never been found to be amplified in other CNS PNETs, medulloblastoma or AT/RT. In summary, these consistent findings have significantly contributed to our ability to sub-classify embryonal brain tumors into clinically and biologically meaningful strata and, for some of the subgroups, have led to the identification of specific targets for future development of molecularly targeted therapies.

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Embryonal tumor with abundant neuropil and true rosettes (ETANTR): report of a case with prominent neurocytic differentiation

Cited by 47 publications

References 12 publications

Atypical Pituitary Adenoma With Neurocytic Transformation

Atypical Pituitary Adenoma With Neurocytic Transformation

Embryonal tumors with abundant neuropil and true rosettes: 2 illustrative cases and a review of the literature

Molecular diagnostics of CNS embryonal tumors

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