Article

Desmoplastic infantile ganglioglioma: a potentially malignant tumor?

Department of Pathology, University of Leuven, Belgium.
American Journal of Surgical Pathology (Impact Factor: 4.59). 12/2002; 26(11):1515-22.
Source: PubMed

ABSTRACT 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.

Download full-text

Full-text

Available from: Raf Sciot, Apr 28, 2014
0 Followers
 · 
237 Views
  • Source
    [Show abstract] [Hide abstract]
    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.
    Epilepsia 05/2007; 48(4):646-53. DOI:10.1111/j.1528-1167.2007.00925.x · 4.58 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    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.
    The Turkish journal of pediatrics 50(5):495-9. · 0.56 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Desmoplastic infantile gangliogliomas (DIG) are rare cerebral glioneural tumors usually occurring in early childhood. DIGs are generally benign although rare cases with poor outcome are known. Total resection, if possible, is the treatment of choice, without further adjuvant therapy. After incomplete resection, adjuvant chemo-and/or radiotherapy is generally applied, despite the potential negative side effects in such young patients. We describe two girls with DIG, one who twice underwent subtotal resection at 3 and 5 months, the other who underwent total resection at 2 years. Neither had adjuvant therapy and there was no tumor recurrence. Our own experience and a review of the literature suggest that in most DIGs adjuvant therapy is not justified even after incomplete resection. After tumor recurrence a second surgical intervention should be considered instead of adjuvant therapy. An exception may be made for rare, deep-seated DIGs, which are more aggressive and have a poorer outcome.
    Child s Nervous System 07/2003; 19(5-6):359-66. DOI:10.1007/s00381-003-0754-9 · 1.16 Impact Factor