1307. Ganglioglioma of the Temporal Lobe in a Patient with Neurofibromatosis Type-1: Case Report and Review of the Literature

Authors: Anthony S. Larson; Albert Tu, MD; Maggie Skrypek, MD (St. Paul, MN)

Introduction:

Neurofibromatosis type-1 (NF1) is an autosomal dominant condition with an estimated world-wide incidence of one per 2,500 to 4,000 individuals. Tumors of the CNS occur in approximately 15-20% of patients with NF1 and are most often astrocytomas in origin. Gangliogliomas in this population of patients are exceedingly rare.

Methods:

We describe a case of a patient with NF1 who was found to have a ganglioglioma of the temporal lobe and review the clinical and pathologic features. A Medline database search was performed. We compare and contrast the cases of ganglioglioma in NF1 patients and in non-NF1 patients.

Results:

We identified 11 prior reported cases of patients with NF1 and a supraspinal ganglioglioma. The average age of patients at presentation was 23.3 years, and 42% were male. In the NF1 population, gangliogliomas were most commonly found in the optic nerve. In contrast, gangliogliomas within the non-NF1 population were most commonly found in the temporal lobe.  All living patients were managed with either a biopsy of the lesion or surgical resection of the tumor. Of patients with documented follow-up, five (83%) had no evidence of recurrence and received no adjuvant therapy. One patient (17%) was treated with adjuvant therapy and died 3.5 years following initial resection.

Conclusion:

This unusual case adds to the few-existing cases of patients with NF1 who have been found to have supraspinal ganglioglioma. Given that lesions of the optic pathway are not typically biopsied or resected, we suggest that GG may be more common in the NF1 population than previously thought. This case emphasizes the potential for alternative diagnoses of CNS tumors in NF1 patients and supports the role of obtaining tissue diagnosis in order to implement the appropriate treatment modality.