CT and Multimodal MR Imaging Features of Embryonal Tumors with Multilayered Rosettes in Children.


Journal

AJNR. American journal of neuroradiology
ISSN: 1936-959X
Titre abrégé: AJNR Am J Neuroradiol
Pays: United States
ID NLM: 8003708

Informations de publication

Date de publication:
04 2019
Historique:
received: 24 10 2018
accepted: 28 01 2019
pubmed: 9 3 2019
medline: 17 3 2020
entrez: 9 3 2019
Statut: ppublish

Résumé

Embryonal tumors with multilayered rosettes, C19MC-altered, are brain tumors occurring in young children, which were clearly defined in the 2016 World Health Organization classification of central nervous system neoplasms. Our objective was to describe the multimodal imaging characteristics of this new entity. We performed a retrospective monocentric review of embryonal brain tumors and looked for embryonal tumors with multilayered rosettes with confirmed C19MC alteration. We gathered morphologic imaging data, as well as DWI and PWI data (using arterial spin-labeling and DSC). We included 16 patients with a median age of 2 years 8 months. Tumors were both supratentorial (56%, 9/16) and infratentorial (44%, 7/16). Tumors were large (median diameter, 59 mm; interquartile range, 48-71 mm), with absent (75%, 12/16) or minimal (25%, 4/16) peritumoral edema. Enhancement was absent (20%, 3/15) or weak (73%, 11/15), whereas intratumoral macrovessels were frequently seen (94%, 15/16) and calcifications were present in 67% (10/15). Diffusion was always restricted, with a minimal ADC of 520 mm Embryonal tumors with multilayered rosettes, C19MC-altered, have characteristic imaging features that could help in the diagnosis of this rare tumor in young children.

Sections du résumé

BACKGROUND AND PURPOSE
Embryonal tumors with multilayered rosettes, C19MC-altered, are brain tumors occurring in young children, which were clearly defined in the 2016 World Health Organization classification of central nervous system neoplasms. Our objective was to describe the multimodal imaging characteristics of this new entity.
MATERIALS AND METHODS
We performed a retrospective monocentric review of embryonal brain tumors and looked for embryonal tumors with multilayered rosettes with confirmed C19MC alteration. We gathered morphologic imaging data, as well as DWI and PWI data (using arterial spin-labeling and DSC).
RESULTS
We included 16 patients with a median age of 2 years 8 months. Tumors were both supratentorial (56%, 9/16) and infratentorial (44%, 7/16). Tumors were large (median diameter, 59 mm; interquartile range, 48-71 mm), with absent (75%, 12/16) or minimal (25%, 4/16) peritumoral edema. Enhancement was absent (20%, 3/15) or weak (73%, 11/15), whereas intratumoral macrovessels were frequently seen (94%, 15/16) and calcifications were present in 67% (10/15). Diffusion was always restricted, with a minimal ADC of 520 mm
CONCLUSIONS
Embryonal tumors with multilayered rosettes, C19MC-altered, have characteristic imaging features that could help in the diagnosis of this rare tumor in young children.

Identifiants

pubmed: 30846437
pii: ajnr.A6001
doi: 10.3174/ajnr.A6001
pmc: PMC7048518
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

732-736

Informations de copyright

© 2019 by American Journal of Neuroradiology.

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Auteurs

V Dangouloff-Ros (V)

From the Departments of Pediatric Radiology (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.) volodia.dangouloff-ros@aphp.fr.
University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.
French National Institute of Health and Medical Research U1000 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.
Institut Imagine 1163 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.

A Tauziède-Espariat (A)

University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.
Department of Neuropathology (A.T.-E., A.G., P.V.), Centre Hospitalier Sainte Anne, Paris, France.

C-J Roux (CJ)

From the Departments of Pediatric Radiology (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.).
University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.
French National Institute of Health and Medical Research U1000 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.
Institut Imagine 1163 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.

R Levy (R)

From the Departments of Pediatric Radiology (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.).
University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.
French National Institute of Health and Medical Research U1000 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.
Institut Imagine 1163 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.

D Grévent (D)

From the Departments of Pediatric Radiology (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.).
University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.
French National Institute of Health and Medical Research U1000 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.
Institut Imagine 1163 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.

F Brunelle (F)

From the Departments of Pediatric Radiology (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.).
University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.
French National Institute of Health and Medical Research U1000 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.
Institut Imagine 1163 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.

A Gareton (A)

Department of Neuropathology (A.T.-E., A.G., P.V.), Centre Hospitalier Sainte Anne, Paris, France.

S Puget (S)

Pediatric Neurosurgery (S.P., K.B., T.B.), Hôpital Necker Enfants Malades, Paris, France.
University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.

K Beccaria (K)

Pediatric Neurosurgery (S.P., K.B., T.B.), Hôpital Necker Enfants Malades, Paris, France.
University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.

T Blauwblomme (T)

Pediatric Neurosurgery (S.P., K.B., T.B.), Hôpital Necker Enfants Malades, Paris, France.
University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.

J Grill (J)

Department of Pediatric and Adolescent Oncology (J.G., C.D.), Gustave Roussy Institute, Villejuif, France.

C Dufour (C)

Department of Pediatric and Adolescent Oncology (J.G., C.D.), Gustave Roussy Institute, Villejuif, France.

P Varlet (P)

University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.
Department of Neuropathology (A.T.-E., A.G., P.V.), Centre Hospitalier Sainte Anne, Paris, France.

N Boddaert (N)

From the Departments of Pediatric Radiology (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.).
University René Descartes (V.D.-R., A.T.-E., C.-J.R., R.L., D.G., F.B., S.P., K.B., T.B. P.V., N.B.), Pôle de Recherche et d'Enseignement Supérieur Sorbonne Paris Cité, Paris, France.
French National Institute of Health and Medical Research U1000 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.
Institut Imagine 1163 (V.D.-R., C.-J.R., R.L., D.G., F.B., N.B.), Paris, France.

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