Desmoplastic infantile ganglioglioma: a potentially malignant tumor?

Department of Pathology, University of Leuven, Belgium.
American Journal of Surgical Pathology (Impact Factor: 5.15). 12/2002; 26(11):1515-22.
Source: PubMed


Desmoplastic infantile ganglioglioma is a rare intracranial tumor of early childhood with a usually excellent prognosis despite malignant features both radiologically and histologically. We present the case of a desmoplastic infantile ganglioglioma with histologically highly anaplastic features and both intracerebral and pial metastases. After partial resection the tumor was rapidly progressive and new metastases appeared. A combination of vincristine and carboplatinum was used according to the Low Grade Glioma Protocol of the International Society of Pediatric Oncology, with a temporary good response. When histologically characterized by highly anaplastic features, it seems the biologic behavior of this tumor remains uncertain. The aggressive behavior and the responsiveness to chemotherapy in this case may challenge the belief in the benign nature of these rare tumors.

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Available from: Raf Sciot, Apr 28, 2014
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    • "ing the benign nature of these tumors lurks the potential for malignant transformation (Hayashi et al., 2001; De Munnynck et al., 2002; Whittle et al., 2002) especially within the glial component (Jay and Becker, 1994; David et al., 2000). The neoplastic potential differentiates GGs from malformations of cortical development such as cortical dysplasia with balloon cells (CDBC), which have also been implicated in epilepsy and represent the most common non-neoplastic finding at surgical pathology for resection of pediatric seizure foci (Andermann, 2000). "
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    ABSTRACT: Gangliogliomas (GGs) are neuronal-glial tumors highly associated with epilepsy. We hypothesized that the expression of select gene families including neurotransmitter receptor subunits and growth factors would be distinct in neurons and astrocytes within GG compared with adjacent cortex and that these changes would yield insights into seizure onset and lesion formation. Candidate gene expression was defined in single immunohistochemically labeled neurons and astrocytes microdissected from GG specimens compared with neurons and astrocytes microdissected from morphologically intact cortex adjacent to the GG or normal control cortex. Differential expression of 16 genes including glutamate transporter (EAAC1) and receptor (NMDA2C, mGluR5), growth factor (hepatocyte growth factor), and receptor (platelet derived growth factor receptor beta, fibroblast growth factor receptor 3) mRNAs was detected in GG neurons compared with control neurons. In astrocytes, altered expression of p75NGF, mGluR3, TGFbeta3 and Glt-1 mRNAs was detected. Nestin mRNA, a gene that exhibits enhanced expression in balloon cell cortical dysplasia, was increased in GG neurons. Because of the morphological similarities between GG and cortical dysplasia, we show that there is activation of the mTOR cascade in GG as evidenced by enhanced expression of phospho-p70S6kinase and phosphoribosomal S6 proteins. We find differential candidate gene expression in neurons and astrocytes in GG compared with adjacent cortex and show that there is activation of the mTOR pathway. These changes highlight pathways that may be pivotal for epileptogenesis and lesion growth.
    Full-text · Article · May 2007 · Epilepsia
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    • "DIGs are typically large supratentorial tumors that, at least as observed radiographically in one patient, are initially solid then become cystic [5]. Although this tumor is considered a grade 1 tumor based on the histopathologic features of cases described prior to the publication of the WHO monograph in 2000, at least one report has since documented anaplastic features in a case of DIG, which was ultimately fatal [6]. However, follow-up has generally been favorable following complete resection in the reported cases of DIG, with a median post-surgical interval of 8.7 years without metastases or recurrence in one series of 14 patients [2]. "
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    ABSTRACT: Background Desmoplastic infantile ganglioglioma (DIG) is a rare WHO Grade I tumor of infancy that is characterized by large volume, superficial location, invariable supratentoriality, fronto-parietal lobe predilection and morphologically, by an admixture of astroglial and neuroepithelial elements in a desmoplastic milieu. With over 50 cases described, the histologic and radiographic spectrum of DIG has been well-characterized. The superficial location of DIGs may render them greatly amenable to preoperative assessment utilizing aspiration cytology; however, the cytologic features of this rare tumor have only been reported once previously. Case Presentation We present herein cytomorphologic findings from the intraoperative aspiration of a typical case of DIG diagnosed in a 1-year-old male. As evaluated on a single liquid-based preparation, the specimen showed low cellularity and was comprised predominantly of a population of dispersed (occasionally clustered) large neuronal cells with eccentrically located hyperchromatic nuclei (which were occasionally binucleated) and abundant unipolar cytoplasm. Rare smaller astroglial cells were intermixed. Despite the tumor's characteristic desmoplastic histologic appearance, no stromal fragments were identified on the aspiration material. Conclusions A differential diagnosis is presented and analyzed in detail and it is concluded that when these large neuronal cells are encountered in an aspirate of a brain mass in a child, a combination of clinical, radiologic and immunohistochemical parameters can eliminate most of the differential possibilities.
    Full-text · Article · Feb 2005 · CytoJournal
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    ABSTRACT: Desmoplastic infantile gangliogliomas are very rarely encountered, large supratentorial masses, derived from neuroepithelial origin, which have cystic and solid components and contain cells with astrocytic and ganglionic differentiation. These tumors are benign tumors of childhood that become symptomatic when they reach giant sizes. Sixty cases of desmoplastic ganglioglioma have been reported to date. In the present study, a case of giant desmoplastic infantile ganglioglioma in a 22-month-old patient is presented, which had an aggressive radiological appearance in the midline and presented with atypical symptoms.
    Preview · Article · Jan 2008 · The Turkish journal of pediatrics
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