Response of subependymal giant cell astrocytoma with spinal cord metastasis to everolimus

Dolly Aguilera; Robert Flamini; Claire Mazewski; Matthew Schniederjan; Laura Hayes; William Boydston; Robert C. Castellino; Tobey J. MacDonald

doi:10.1097/MPH.0000000000000005

Response of subependymal giant cell astrocytoma with spinal cord metastasis to everolimus

Dolly Aguilera
, Robert Flamini
, Claire Mazewski
, Matthew Schniederjan
, Laura Hayes
, William Boydston
, Robert C. Castellino
, Tobey J. MacDonald

Nemours Children's Health, Pensacola

Emory University
Department of Neurology
Children's Healthcare of Atlanta

Research output: Contribution to journal › Article › peer-review

11 Scopus citations

Abstract

Background: Brain subependymal giant cell astrocytomas (SEGAs) in patients with tuberous sclerosis have been reported to respond to everolimus.

Methods: A 15-year-old male patient with intractable seizures and multiple SEGAs of the brain developed leptomeningeal enhancement and multiple metastatic, histologically confirmed SEGAs of the spinal cord. He received daily everolimus at a dose of 3 mg/m2 for 6 weeks, which was then increased to 6 mg/m2.

Results: Magnetic resonance image of the brain and spine showed significant reduction in the size of SEGAs after 6 weeks of treatment. The patient has remained free of progression for 24 months. Additional benefits included: excellent seizure control, decrease in the size of cardiac rhabdomyomas, and improved quality of life.

Conclusions: We describe a rare case of metastatic SEGA, which was successfully treated with everolimus.

Original language	English
Pages (from-to)	e448-e451
Journal	Journal of Pediatric Hematology/Oncology
Volume	36
Issue number	7
DOIs	https://doi.org/10.1097/MPH.0000000000000005
State	Published - 8 Oct 2014

Keywords

Everolimus
SEGA
Spinal cord
Tuberous sclerosis

Access to Document

10.1097/MPH.0000000000000005

Cite this

@article{6cd5930e0ed1400a8d8dbbf330afde17,

title = "Response of subependymal giant cell astrocytoma with spinal cord metastasis to everolimus",

abstract = "Background: Brain subependymal giant cell astrocytomas (SEGAs) in patients with tuberous sclerosis have been reported to respond to everolimus.Methods: A 15-year-old male patient with intractable seizures and multiple SEGAs of the brain developed leptomeningeal enhancement and multiple metastatic, histologically confirmed SEGAs of the spinal cord. He received daily everolimus at a dose of 3 mg/m2 for 6 weeks, which was then increased to 6 mg/m2.Results: Magnetic resonance image of the brain and spine showed significant reduction in the size of SEGAs after 6 weeks of treatment. The patient has remained free of progression for 24 months. Additional benefits included: excellent seizure control, decrease in the size of cardiac rhabdomyomas, and improved quality of life.Conclusions: We describe a rare case of metastatic SEGA, which was successfully treated with everolimus.",

keywords = "Everolimus, SEGA, Spinal cord, Tuberous sclerosis",

author = "Dolly Aguilera and Robert Flamini and Claire Mazewski and Matthew Schniederjan and Laura Hayes and William Boydston and Castellino, \{Robert C.\} and MacDonald, \{Tobey J.\}",

note = "Publisher Copyright: Copyright {\textcopyright} 2013 by Lippincott Williams \& Wilkins.",

year = "2014",

month = oct,

day = "8",

doi = "10.1097/MPH.0000000000000005",

language = "English",

volume = "36",

pages = "e448--e451",

journal = "Journal of Pediatric Hematology/Oncology",

issn = "1077-4114",

publisher = "Lippincott Williams and Wilkins",

number = "7",

}

TY - JOUR

T1 - Response of subependymal giant cell astrocytoma with spinal cord metastasis to everolimus

AU - Aguilera, Dolly

AU - Flamini, Robert

AU - Mazewski, Claire

AU - Schniederjan, Matthew

AU - Hayes, Laura

AU - Boydston, William

AU - Castellino, Robert C.

AU - MacDonald, Tobey J.

PY - 2014/10/8

Y1 - 2014/10/8

N2 - Background: Brain subependymal giant cell astrocytomas (SEGAs) in patients with tuberous sclerosis have been reported to respond to everolimus.Methods: A 15-year-old male patient with intractable seizures and multiple SEGAs of the brain developed leptomeningeal enhancement and multiple metastatic, histologically confirmed SEGAs of the spinal cord. He received daily everolimus at a dose of 3 mg/m2 for 6 weeks, which was then increased to 6 mg/m2.Results: Magnetic resonance image of the brain and spine showed significant reduction in the size of SEGAs after 6 weeks of treatment. The patient has remained free of progression for 24 months. Additional benefits included: excellent seizure control, decrease in the size of cardiac rhabdomyomas, and improved quality of life.Conclusions: We describe a rare case of metastatic SEGA, which was successfully treated with everolimus.

AB - Background: Brain subependymal giant cell astrocytomas (SEGAs) in patients with tuberous sclerosis have been reported to respond to everolimus.Methods: A 15-year-old male patient with intractable seizures and multiple SEGAs of the brain developed leptomeningeal enhancement and multiple metastatic, histologically confirmed SEGAs of the spinal cord. He received daily everolimus at a dose of 3 mg/m2 for 6 weeks, which was then increased to 6 mg/m2.Results: Magnetic resonance image of the brain and spine showed significant reduction in the size of SEGAs after 6 weeks of treatment. The patient has remained free of progression for 24 months. Additional benefits included: excellent seizure control, decrease in the size of cardiac rhabdomyomas, and improved quality of life.Conclusions: We describe a rare case of metastatic SEGA, which was successfully treated with everolimus.

KW - Everolimus

KW - SEGA

KW - Spinal cord

KW - Tuberous sclerosis

UR - https://www.scopus.com/pages/publications/84910115574

U2 - 10.1097/MPH.0000000000000005

DO - 10.1097/MPH.0000000000000005

M3 - Article

C2 - 24276039

AN - SCOPUS:84910115574

SN - 1077-4114

VL - 36

SP - e448-e451

JO - Journal of Pediatric Hematology/Oncology

JF - Journal of Pediatric Hematology/Oncology

IS - 7

ER -

Response of subependymal giant cell astrocytoma with spinal cord metastasis to everolimus

Abstract

Keywords

Access to Document

Other files and links

Fingerprint

Cite this