Elsevier

Journal of Neuroradiology

Volume 35, Issue 5, December 2008, Pages 286-291
Journal of Neuroradiology

Case report
Multifocal desmoplastic noninfantile astrocytomaAstrocytome non infantile desmoplastique multifocal

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Summary

This is a report of a case of multifocal desmoplastic astrocytoma in an 11-year-old child in which we describe the MRI findings and discuss the possible mechanism of its development. The MRI appearances in our case support the view that the tumor is primarily of leptomeningeal or superficial cortical origin, with cystic formation secondary to entrapment of cerebrospinal fluid. The question of whether or not the lesions are metastases or metachronous lesions is also discussed. Desmoplastic astrocytoma at a noninfantile age is extremely rare: only four cases have been reported in the literature so far. Even more unusual is the presence of this lesion in multiple locations at the initial presentation.

Résumé

Nous rapportons un cas d’astrocytome desmoplastique multifocal chez un enfant de 11 ans, décrivons le résultat des IRM répétées et discutons le mécanisme possible de son développement. Les IRM réalisées chez notre patient sont en faveur d’une tumeur dont l’origine est initialement leptoméningée ou corticale avec formation secondaire de kystes liés au cloisonnement du liquide cérébrospinal. Le problème de l’origine métastatique ou métachronique des lésions est un sujet largement débattu. L’astrocytome desmoplastique survenant à un âge non infantile est très rare avec seulement quatre cas rapportés dans la littérature. La présence de localisations multiples, lors de la présentation initiale, est encore plus rare.

Introduction

Desmoplastic infantile astrocytoma (DIA), a tumor originally described in infants, can occur in older children and young adults and is then referred to as the noninfantile variant [1] Desmoplastic infantile ganglioglioma (DIG) and DIA are similar-looking rare tumors that involve the superficial cerebral cortex and leptomeninges, with variable attachment to the dura and are considered grade I in the World Health Organization (WHO) classification system [2] In spite of this grading, craniospinal metastases have been reported with DIG/DIA in the literature [3] This is a report of the findings in a series of MRI scans of a rare case of desmoplastic noninfantile astrocytoma presenting with multiple craniospinal lesions.

Section snippets

Case report

A 11-year-old boy was admitted to our hospital in January 2007 complaining of gradually progressing stiffness in both legs, neck pain and visual blackouts, lasting one month. Neurological examination was unremarkable except for wasting of the thenar and hypothenar muscles. MRI showed dilated ventricles, a prominent right middle frontal sulcus and a well-defined cystic lesion in the left anterior frontal cortex (Fig. 1). Cystic areas were hyperintense in comparison to cerebrospinal fluid (CSF)

Discussion

Desmoplastic infantile astrocytoma is a rare brain neoplasm that was first described by Taratuto et al. [1] DIG/DIA is generally seen in infants aged under 18 months, with a mean age at diagnosis of five to six months [2], [3] However, the noninfantile variant of desmoplastic ganglioglioma is increasingly being reported in the literature [4], [5], with these patients ranging from 5 to 25 years of age. The imaging findings and the prognosis for both infantile and noninfantile tumors are often

Conclusion

This was the first reported case of a noninfantile variant of DIA with multiple craniospinal lesions at presentation. Serial MRI features of this tumor indicate a leptomeningeal origin of this lesion. Multiple new lesions appearing over time together with the relatively static older lesions could be indicative of the metachronous nature of this neoplasm. DIA/DIG has a propensity to be multifocal and, hence, these patients should be regularly followed up. Also, the prognosis in these cases

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