Abstract
BACKGROUND: Desmoplastic infantile astrocytomas/ganglioglioma (DIA/DIG) arising in the suprasellar region are extremely rare, and their clinical features, optimal management, and outcomes remain unclear. We aimed to summarize the clinical manifestations, treatment strategies, and prognosis of this rare tumor entity. PATIENTS AND METHODS: This retrospective case series included 5 institutional cases and 13 literature cases of suprasellar DIA/DIGs. The clinical and radiological characteristics, therapies, and outcomes of this rare tumor were examined. RESULTS: Our case series comprised 2 males and 3 females, with a median age of 6 months. Three patients underwent partial resection, and 2 had subtotal resection. During follow-up, the residual tumors in 2 patients showed spontaneous regression, 1 tumor progressed, and 2 remained stable. The literature review, including our cases, yielded 18 suprasellar DIA/DIG. Of these, 10 were male and 8 were female, with a median age of 4 months (range, 1-85 months). Ten patients had solitary suprasellar tumors and 8 had multifocal lesions. Over a median follow-up of 25 months, tumor progression of the suprasellar lesions was seen in 9 patients. Notably, spontaneous regression of the suprasellar tumors occurred in 2 patients. CONCLUSIONS: Despite being WHO grade I, suprasellar DIA/DIG can demonstrate multifocal CNS disease and high risk of progression after subtotal resection. DIA/DIG should be considered in the differential diagnosis of suprasellar lesions in infants and long-term close monitoring is warranted.