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Infantile
myofibromatosis of the central nervous system
Tamburrini G, Gessi M, Colosimo C Jr, Lauriola L, Giangaspero F, Di
Rocco C
Pediatric
Neurosurgical Unit, Catholic University Medical School, Largo A. Gemelli, 8,
00168 Rome, Italy. gtamburrini@hotmail.com
Background.
Infantile myofibromatosis is the most common fibrous disorder of infancy and
childhood.
It may occur in two distinct forms: multicentric and solitary.
In both cases involvement of the central nervous system (CNS) is unusual: brain
myofibromas are usually intracranial in proximity of the dura mater, with
infiltration of the calvarial bones and secondary brain compression.
Spine myofibromas are exceptional and most of the cases reported in the
literature represent secondary locations of visceral lesions.
The natural history of myofibromas of the CNS in infantile myofibromatosis is
characterized by a period of rapid growth, subsequent stabilization, and
spontaneous regression in many cases.
On these grounds surgical treatment is reserved for lesions that compress the
brain or spinal cord and show an obvious progression.
Small lesions or tumor residuals in asymptomatic children without visceral
involvement should be closely observed as first choice.
Case
Report.
We report the case of a 10-month-old child with a solitary intramedullary
myofibroma, extended from C7 to the conus.
The patient underwent partial surgical removal of this lesion.
Control MRI has shown the spontaneous slow reduction of the tumor residual and a
progressive improvement of preoperative neurological conditions has been
observed.
To our knowledge this is the first case in which a solitary myofibroma was
localized within the spinal cord.
PMID: 12720031 [PubMed - indexed for MEDLINE]
Source: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12720031&dopt=Abstract
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