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A report of a desmoplastic non-infantile ganglioglioma in a 6-year-old boy with review of the literature

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Abstract

Desmoplastic infantile gangliogliomas (DIG) are uncommon supratentorial brain tumors with a usually good prognosis despite an aggressive radiological appearance that typically occurs in infants below the age of 24 months. DIGs are exclusively supratentorial, generally have a voluminous size, and are partially cystic. Total surgical removal is sufficient for the treatment of these tumors, and no chemotherapy or radiotherapy is indicated if complete resection is achieved. Except for age difference, DIG and desmoplastic non-infantile gangliogliomas are radiologically and histologically similar. Non-infantile variants of this biologically benign intracranial neoplasm are rare; only 15 cases of non-infantile DIGs have been reported in the literature. As far as we know, this case is the 16th in literature. We reported the magnetic resonance imaging and histological findings of desmoplastic ganglioglioma in a 6-year-old boy.

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Authors and Affiliations

  1. Department of Pediatric Neurology, Medical Faculty, Erciyes University, Kayseri, Turkey

    Hüseyin Per & Sefer Kumandaş

  2. Department of Pathology, Medical Faculty, Erciyes University, Kayseri, Turkey

    Olgun Kontaş

  3. Department of Pediatric Neurosurgery, Medical Faculty, Erciyes University, Kayseri, Turkey

    Ali Kurtsoy

Authors
  1. Hüseyin Per
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  2. Olgun Kontaş
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  3. Sefer Kumandaş
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  4. Ali Kurtsoy
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Correspondence to Hüseyin Per.

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Comments

Michel Mittelbronn, Zürich, Switzerland

Per et al. present an interesting neoplasia with both a glial and a neuronal component. The tumor is of low cell density and does not exhibit clear features of malignancy (e.g., low proliferation index, no mitotic activity, and no signs of atypia). Further, a marked desmoplastic reaction is visible. Therefore, it is suitable to name this tumor “desmoplastic ganglioglioma” (DG) as proposed. However, also the classical gangliogliomas tend to possess a dense reticulin fiber network which might be quite similar to the desmoplastic reaction seen in DGs. Therefore, it is necessary to take clinical (e.g., age, epilepsy) and radiological (e.g., localization, cyst formation) information into account to come to the right diagnosis. Usually, the age range of desmoplastic ganglioglioma is 1–24 months leading to the current official name of “Desmoplastic infantile ganglioglioma.” Since several cases with ages ranging from 5 to 25 years have been observed in the meantime, these cases are now called “Desmoplastic non-infantile ganglioglioma.”

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Per, H., Kontaş, O., Kumandaş, S. et al. A report of a desmoplastic non-infantile ganglioglioma in a 6-year-old boy with review of the literature. Neurosurg Rev 32, 369–374 (2009). https://doi.org/10.1007/s10143-009-0195-3

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  • DOI: https://doi.org/10.1007/s10143-009-0195-3

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