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Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview

Bächli, Heidi ; Avoledo, Pierino ; Gratzl, Otmar ; Tolnay, Marcus

In: Child's Nervous System, 2003, vol. 19, no. 5-6, p. 359-366

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    Titre
    • Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview
    Auteur
    • Bächli, Heidi. Department of Neurosurgery, University Hospital Basel, Spitalstrasse 21, 4031, Basel, Switzerland
    • Avoledo, Pierino. Division of Oncology/Hematology, University Children's Hospital, Basel, Switzerland
    • Gratzl, Otmar. Department of Neurosurgery, University Hospital Basel, Spitalstrasse 21, 4031, Basel, Switzerland
    • Tolnay, Marcus. Department of Neuropathology, University Basel, Basel, Switzerland
    Type de document
    • Postprint
    Identifiant OAI-PMH
    • oai:doc.rero.ch:321071
    Summary
    Introduction: 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. Case reports: We describe two girls with DIG, one who twice underwent subtotal resection at 3 and 5months, the other who underwent total resection at 2years. Neither had adjuvant therapy and there was no tumor recurrence. Conclusions: 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