Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor

Report of the first case

Bassem El-Nabbout, Rolla Shbarou, Charles M. Glasier, Ali Saad

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics. AT/RT typically involves the posterior fossa of the pediatric population. Supratentorial AT/RT is exceedingly rare. In this report, we describe a very unusual case of a child who presented with signs and symptoms suggestive of leptomeningitis. However, imaging studies and histologic findings showed plaque-like AT/RT involving the leptomeninges of the cerebrum, cerebellum, and spinal cord. The disease proved to be rapidly fatal and resulted in the patient's death within approximately two weeks. To our knowledge, this is the first case of primary leptomeningeal AT/RT involving the supratentorial leptomeninges.

Original languageEnglish (US)
Pages (from-to)431-434
Number of pages4
JournalJournal of Neuro-Oncology
Volume98
Issue number3
DOIs
StatePublished - Jul 1 2010
Externally publishedYes

Fingerprint

Nervous System Neoplasms
Cerebrum
Cerebellum
Signs and Symptoms
Spinal Cord
Central Nervous System
Atypical Teratoid Tumor
Typical Teratoid Rhabdoid Tumor
Pediatrics
Population

All Science Journal Classification (ASJC) codes

  • Oncology
  • Neurology
  • Clinical Neurology
  • Cancer Research

Cite this

Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor : Report of the first case. / El-Nabbout, Bassem; Shbarou, Rolla; Glasier, Charles M.; Saad, Ali.

In: Journal of Neuro-Oncology, Vol. 98, No. 3, 01.07.2010, p. 431-434.

Research output: Contribution to journalArticle

El-Nabbout, Bassem ; Shbarou, Rolla ; Glasier, Charles M. ; Saad, Ali. / Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor : Report of the first case. In: Journal of Neuro-Oncology. 2010 ; Vol. 98, No. 3. pp. 431-434.
@article{2fed465e6631407791d3006e6d881a7f,
title = "Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor: Report of the first case",
abstract = "Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics. AT/RT typically involves the posterior fossa of the pediatric population. Supratentorial AT/RT is exceedingly rare. In this report, we describe a very unusual case of a child who presented with signs and symptoms suggestive of leptomeningitis. However, imaging studies and histologic findings showed plaque-like AT/RT involving the leptomeninges of the cerebrum, cerebellum, and spinal cord. The disease proved to be rapidly fatal and resulted in the patient's death within approximately two weeks. To our knowledge, this is the first case of primary leptomeningeal AT/RT involving the supratentorial leptomeninges.",
author = "Bassem El-Nabbout and Rolla Shbarou and Glasier, {Charles M.} and Ali Saad",
year = "2010",
month = "7",
day = "1",
doi = "10.1007/s11060-009-0094-z",
language = "English (US)",
volume = "98",
pages = "431--434",
journal = "Journal of Neuro-Oncology",
issn = "0167-594X",
publisher = "Kluwer Academic Publishers",
number = "3",

}

TY - JOUR

T1 - Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor

T2 - Report of the first case

AU - El-Nabbout, Bassem

AU - Shbarou, Rolla

AU - Glasier, Charles M.

AU - Saad, Ali

PY - 2010/7/1

Y1 - 2010/7/1

N2 - Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics. AT/RT typically involves the posterior fossa of the pediatric population. Supratentorial AT/RT is exceedingly rare. In this report, we describe a very unusual case of a child who presented with signs and symptoms suggestive of leptomeningitis. However, imaging studies and histologic findings showed plaque-like AT/RT involving the leptomeninges of the cerebrum, cerebellum, and spinal cord. The disease proved to be rapidly fatal and resulted in the patient's death within approximately two weeks. To our knowledge, this is the first case of primary leptomeningeal AT/RT involving the supratentorial leptomeninges.

AB - Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics. AT/RT typically involves the posterior fossa of the pediatric population. Supratentorial AT/RT is exceedingly rare. In this report, we describe a very unusual case of a child who presented with signs and symptoms suggestive of leptomeningitis. However, imaging studies and histologic findings showed plaque-like AT/RT involving the leptomeninges of the cerebrum, cerebellum, and spinal cord. The disease proved to be rapidly fatal and resulted in the patient's death within approximately two weeks. To our knowledge, this is the first case of primary leptomeningeal AT/RT involving the supratentorial leptomeninges.

UR - http://www.scopus.com/inward/record.url?scp=77953621826&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77953621826&partnerID=8YFLogxK

U2 - 10.1007/s11060-009-0094-z

DO - 10.1007/s11060-009-0094-z

M3 - Article

VL - 98

SP - 431

EP - 434

JO - Journal of Neuro-Oncology

JF - Journal of Neuro-Oncology

SN - 0167-594X

IS - 3

ER -