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True aqueductal tumors: a unique entity.

Jonathan Roth1, Kaisorn L Chaichana, George Jallo

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Pure aqueductal tumors (ATs) are rare and often cause obstructive hydrocephalus. Treatment prioritizes cerebrospinal fluid drainage, with resection reserved for growing tumors.

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Area of Science:

  • Neuro-oncology
  • Neurosurgery
  • Neuropathology

Background:

  • Pure aqueductal tumors (ATs) are distinct from pineal or tectal tumors, being centered within the cerebral aqueduct.
  • These rare tumors are often misclassified with adjacent lesions, necessitating focused study.
  • This multicenter study details the clinical experience with pure ATs.

Purpose of the Study:

  • To characterize the clinical presentation, treatment strategies, and outcomes for patients with pure aqueductal tumors.
  • To evaluate the efficacy of different management approaches for ATs, including conservative and surgical interventions.

Main Methods:

  • Retrospective data collection from three tertiary centers (1999-2013).
  • Analysis included presenting symptoms, treatment paradigms, surgical approaches, pathology, and patient outcomes.
  • Patient demographics and tumor characteristics were reviewed.

Main Results:

  • Sixteen patients with ATs were treated, aged 5.5 to 57 years.
  • Hydrocephalus was the primary presentation in 13 patients; two were incidental findings.
  • Endoscopic third ventriculostomy (ETV) was performed in 13 patients, with two undergoing simultaneous biopsy. Three patients underwent resection for tumor progression. Pathologies included glioblastoma, glioneural tumor, and ependymoma. Non-resected tumors showed stability or minimal growth.

Conclusions:

  • Aqueductal tumors (ATs) are rare and typically present with obstructive hydrocephalus.
  • Cerebrospinal fluid (CSF) diversion, preferably ETV, is the primary treatment. Endoscopic biopsy is an option in select cases.
  • Tumor resection is reserved for progressive lesions, with trans-fourth ventricular or trans-choroidal approaches suggested for safety.