True aqueductal tumors: a unique entity.
BACKGROUND
Pure aqueductal tumors (ATs) differ from pineal region and tectal/tegmental tumors in that they are epicentered within the aqueduct. Nevertheless, these tumors are rarely described as a separate type of tumor, and are often grouped with other lesions located in the same vicinity. The present multicenter study focuses on our experience treating patients with pure ATs.


METHODS
Data from three large tertiary centers was collected retrospectively, including presenting symptoms, treatment paradigm, surgical approaches, pathology, and outcome.


RESULTS
Between 1999 and 2013, 16 patients with AT were diagnosed and treated at the three tertiary centers. Ages at presentation ranged from 5.5 to 57 years. Thirteen patients presented with hydrocephalus-related symptoms, and two were identified incidentally. Thirteen patients underwent an endoscopic third ventriculostomy, and two of these underwent a simultaneous endoscopic biopsy (one grade II ependymoma, one non-specified low-grade glioma). Two others underwent shunt placement. Three patients underwent resection due to tumor progression. Pathologies included glioblastoma multiforme, glioneural tumor, and ependymoma grade II. All non-resected tumors remained stable or grew only minimally.


CONCLUSIONS
ATs are a rare entities that usually present with obstructive hydrocephalus. Treatment includes primarily cerebrospinal fluid drainage (preferably via an endoscopic third ventriculostomy). Simultaneous endoscopic biopsy may be done in selected cases. Tumor resection should be reserved for growing tumors; the trans-fourth ventricular or trans-choroidal approaches are probably safer than other approaches used to reach the tectal region.
Department of Pediatric Neurosurgery, Dana Children's Hospital, Tel-Aviv Medical Center, Tel-Aviv University, 6 Weizman Street, Tel Aviv, 64239, Israel, jonaroth@gmail.com.
Jonathan
J
Roth
Kaisorn L
KL
Chaichana
George
G
Jallo
Giuseppe
G
Mirone
Giuseppe
G
Cinalli
Shlomi
S
Constantini
D000293:Adolescent; D000328:Adult; D002535:Cerebral Aqueduct / Q000473:pathology* / Q000601:surgery; D002551:Cerebral Ventricle Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D004806:Ependymoma / Q000473:pathology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D005909:Glioblastoma / Q000473:pathology / Q000601:surgery; D005910:Glioma / Q000473:pathology* / Q000601:surgery; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology / Q000601:surgery; D008297:Male; D008875:Middle Aged; D044583:Neuroendoscopy; D019635:Neurosurgical Procedures / Q000379:methods*; D012189:Retrospective Studies; D016896:Treatment Outcome; D014696:Ventriculostomy; D055815:Young Adult