Endoscopic aqueductal membrane fenestration was effective for intractable hydrocephalus after removal of a nongerminomatous germ cell tumor exhibiting growing teratoma syndrome: a case report

BMC Pediatr. 2022 Nov 28;22(1):683. doi: 10.1186/s12887-022-03743-y.


Background: Primary central nervous system (CNS) germ cell tumors (GCTs) are rare neoplasms predominantly observed in the pediatric and young adult populations. A mixed GCT including immature teratoma exhibiting growing teratoma syndrome is presented. The pathogenesis of growing teratoma syndrome remains unclear, and its treatment strategy has not been established. GCTs are often located within the ventricles, causing hydrocephalus, which sometimes improves after removal of the tumor due to restoration of cerebrospinal fluid (CSF) flow. On the other hand, even if the flow route of CSF from the third ventricle to arachnoid granulations on the brain surface quadrigeminal cistern is restored after removal of the tumor, hydrocephalus may not improve.

Case presentation: A case whose intractable hydrocephalus improved after penetrating the aqueductal membrane via endoscopy is described. An 11-year-old boy was treated for pineal intracranial growing teratoma syndrome (IGTS). The tumor grew rapidly in a short period, and hydrocephalus progressed despite endoscopic third ventriculostomy (ETV). Although the obstruction was removed by radiation, chemotherapy, and total tumor resection, the hydrocephalus did not improve. Endoscopic membrane perforation was performed because a membrane-like structure was seen at the entrance of the cerebral aqueduct on magnetic resonance imaging. The hydrocephalus improved immediately after the operation, and the patient's consciousness disturbance also improved significantly.

Conclusion: The purpose of this report is to update the current knowledge and standards of management for patients with growing teratoma syndrome, as well as to drive future translational and clinical studies by recognizing the unmet needs concerning hydrocephalus.

Keywords: Aqueductal membrane; Case report; Hydrocephalus; Intracranial growing teratoma syndrome; Nongerminomatous germ cell tumor.

Publication types

  • Case Reports

MeSH terms

  • Cerebral Aqueduct
  • Child
  • Endoscopy
  • Humans
  • Hydrocephalus* / diagnostic imaging
  • Hydrocephalus* / etiology
  • Hydrocephalus* / surgery
  • Male
  • Neoplasms, Germ Cell and Embryonal*
  • Syndrome
  • Teratoma* / complications
  • Teratoma* / diagnostic imaging
  • Teratoma* / surgery
  • Young Adult

Supplementary concepts

  • Nonseminomatous germ cell tumor