A report of a desmoplastic ganglioglioma in a 12-year-old girl with review of the literature

Desmoplastic infantile ganglioglioma is a supratentorial tumor that typically occurs in infants below the age of 24 months. Rare tumors with the same radiological and histological characteristics have been described in older subjects. We report a case of desmoplastic ganglioglioma in a 12-year-old g...

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Bibliographic Details
Published in:Journal of neuro-oncology 2006-02, Vol.76 (3), p.271-275
Main Authors: POMMEPUY, I, DELAGE-CORRE, M, MOREAU, J. J, LABROUSSE, F
Format: Article
Language:English
Subjects:
Age Factors
Biological and medical sciences
Child
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Ganglioglioma - pathology
Ganglioglioma - surgery
Humans
Medical sciences
Microscopy, Electron, Transmission
Neurology
Neurosurgical Procedures
Supratentorial Neoplasms - pathology
Supratentorial Neoplasms - surgery
Tomography, X-Ray Computed
Tumors of the nervous system. Phacomatoses
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Summary:Desmoplastic infantile ganglioglioma is a supratentorial tumor that typically occurs in infants below the age of 24 months. Rare tumors with the same radiological and histological characteristics have been described in older subjects. We report a case of desmoplastic ganglioglioma in a 12-year-old girl with a 13 years follow-up. The patient presented with an inaugural generalized seizure. CT scan demonstrated a large superficial parieto-occipital mass, attached to the dura with solid and cystic components. Surgical resection was macroscopically complete. No adjuvant treatment was given. Thirteen years after surgery, the patient is symptom free. Histological examination revealed a pleomorphic tumor involving the meningeal space and the cortex. Meningeal portion was made of neoplastic astrocytes enmeshed in a dense network of connective tissue. The cortical component showed abnormal neurons, tumoral astrocytes and small foci of poorly differentiated cells with rare mitoses. Our observation and the seven others found in the literature indicate that desmoplastic gangliogliomas can occur in children and even in young adults. Despite some worrisome radiological and histological features, these tumors should not be misdiagnosed as malignant glioma. Like infantile cases, non-infantile desmoplastic gangliogliomas seem to have a favorable prognosis without additional therapy, if a total surgical resection can be performed.
ISSN:0167-594X
1573-7373
DOI:10.1007/s11060-005-6500-2