Case reportDesmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis
Introduction
Desmoplastic ganglioglioma (DIG) was first described by Vandenberg et al. in 1987 with the closely related desmoplastic infantile astrocytoma (DIA) described by Taratuto in 1984. The only difference between the two is the presence of a neuronal component in DIG. DIG and DIA are listed together in the most recent edition of the World Health Organisation classification of tumours of the nervous system. These are rare intracranial tumours of early childhood that involve superficial cerebral cortex and leptomeninges. It is believed that the cell of origin of these tumours is an immature cell capable of divergent differentiation along astrocytic and neuronal lines and capable of inducing a desmoplastic reaction in the adjacent leptomeninges.[1], [2], [3]
The majority of reported cases of DIG/DIA occur in infants between 1–24 months with a male:female ratio of 1.7:1.0.2 There are several reported cases of non-infantile DIG with the oldest reported patient being 19 years of age.4 The most common presentation is an enlarging head circumference with tense bulging fontanelles. Typically the tumour is large and cystic with a small enhancing solid cortical component. Superficially the tumour is extracerebral, involving leptomeninges and superficial cortex with attachment to the dura. Most of the reported cases are hemispheric. Two tumours have been reported in the suprasellar/ hypothalamic region.[5], [6] In both cases the tumour was described as firm and rubbery.
Despite the large size at presentation and the presence of poorly differentiated cells, these tumours have been reported to behave in a benign fashion and to have an excellent prognosis with disease-free survival of 8–20 years.[1], [3], [6], [7]
We report two patients with DIG/DIA who developed multiple cerebrospinal metastases. To our knowledge only two similar cases have been reported in the literature.
Section snippets
First case
A 4-month-old boy presented with irritability, failure to thrive and increasing head circumference. On examination the anterior fontanelle was tense and there was concern his vision was abnormal.
Brain MRI showed a large lobulated enhancing mass in the suprasellar region compressing the third ventricle and causing hydrocephalus (Fig. 1). The mass contained few cystic areas. There was also a small enhancing lesion in the outlet of the fourth ventricle (Fig. 2). Spine MRI was normal.
Second case
A 3-month-old boy presented with recurrent seizures and bulging fontanelles. Brain MRI scan showed a 6 × 9 × 11 cm multiloculated cystic right hemispheric tumour with a solid enhancing component (Fig. 5).
The diagnoses of DIA or glioblastoma multiforme were entertained and he underwent craniotomy and excision of the peripheral enhancing component of the tumour and drainage of the cyst.
Discussion
The first case was unusual in that the tumour was located in the suprasellar region, was solid and was attached to the dura of the base of the skull, which made safe surgical resection impossible. The patient died 2 months after surgery from respiratory insufficiency and hypothalamic dysfunction. Of the previously reported cases only two were located in the suprasellar region.[5], [6] Although one can argue that the inability to resect the tumour was the cause of the poor outcome, the presence
Acknowledgements
We acknowledge the contribution of Dr Tony Tannenberg and Dr Michael Rodriguez for reviewing the histology on both patients.
References (8)
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Desmoplastic supratentorial neuroepithelial tumours of infancy with divergent differentiation potential (desmoplastic ganglioglioma). Report on 11 cases of a distinctive embryonal tumour with favourable prognosis
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Desmoplastic non-infantile ganglioglioma. Case report
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