Case studyDiagnosis and treatment for pure aqueductal tumor

•We examined two aggressive cases of pure aqueductal tumor (PAT).•Genetic analysis were first performed for PAT.•It is often difficult to distinguish between tectal glioma and PAT by T1WI, T2WI and Gd enhanced image.•CISS was helpful to make an accurate diagnosis of PAT.•Close observation is needed if PAT is diagnosed as low-grade tumor.

Pure aqueductal tumor (PAT) typically originates from pure aqueductal region and is extremely rare. It is radiographically similar to tectal glioma. We examined two patients with PATs who were diagnosed with pilocytic astrocytoma and rosette-forming glioneuronal tumor. Both cases showed a progressive clinical course. It is important to distinguish between PAT and tectal glioma by radiographic imaging because the treatment strategy is different. While observation is common for tectal gliomas, a biopsy is recommended at the same time of endoscopic third ventriculostomy for PAT with hydrocephalus. Low-grade PATs show an aggressive clinical course in some cases. Our two cases also showed aggressive course in spite of no genetic aggressive mutations. Sagittal view by constructive interference in steady state (CISS) imaging was helpful to make an accurate diagnosis of PAT. Close observation is needed if PAT is diagnosed as low-grade tumor.