Case reportMultifocal desmoplastic noninfantile astrocytomaAstrocytome non infantile desmoplastique multifocal
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
References (17)
- et al.
Desmoplastic low-grade astrocytoma: a case report and review of literature
Clin Neurol Neurosurg
(1995) - et al.
Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis
J Clin Neurosci
(2007) - et al.
Desmoplastic cerebral astrocytomas of infancy: a histopathologic, immunohistochemical, ultrastructural, and molecular genetic study
Hum Pathol
(1992) - et al.
Superficial cerebral astrocytoma attached to dura. Report of six cases in infants
Cancer
(1984) - et al.
Desmoplastic infantile astrocytoma and ganglioglioma
- et al.
MR imaging in the diagnosis of desmoplastic infantile tumor: retrospective study of six cases
AJNR
(2004) - et al.
Non-infantile variant of desmoplastic ganglioglioma: a report of 2 cases
Pediatr Radiol
(2006) - et al.
A report of a desmoplastic ganglioglioma in a 12-year-old girl with review of the literature
J Neurooncol
(2006)
Cited by (10)
Desmoplastic infantile astrocytoma with atypical phenotype, PTEN homozygous deletion and BRAF V600E mutation
2022, Acta Neuropathologica CommunicationsPurely Intraventricular Desmoplastic Infantile Astrocytoma
2021, Journal of Pediatric NeurologyDesmoplastic infantile astrocytoma and ganglioglioma: a series of 12 patients treated at a single institution
2021, Child's Nervous SystemDesmoplastic astrocytoma: new insights into its clinical profile, diagnosis, and treatment
2016, Child's Nervous SystemMultiple desmoplastic astrocytoma: a benign neoplasm?
2015, Child's Nervous System