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

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.