Simultaneous subependymomas in monozygotic female twins: further evidence for a common genetic or developmental disorder background

J Neurosurg. 2014 Sep;121(3):570-5. doi: 10.3171/2014.2.JNS122179. Epub 2014 Mar 21.

Abstract

In this paper, a rare case of subependymoma of the fourth ventricle in identical female twins is reported. Magnetic resonance imaging and CT showed nearly identical locations of the tumors in the fourth ventricle and similar growth patterns of the tumors in both sisters. Likewise, postoperative histopathological analysis of both tumors revealed the typical histological appearance of subependymomas. Subependymoma is a rare, low-grade glioma of the CNS, slowly growing and usually asymptomatic. If symptomatic, a subependymoma can in some cases lead to sudden death caused by pressure on the brainstem or decompensated secondary hydrocephalus. This case demonstrates the importance of detecting tumors early and thereby preventing symptoms arising from increasing intracranial pressure, and optimizing therapy options.

Keywords: GFAP = glial fibrillary acidic protein; SNP = single nucleotide polymorphism; familial tumor; fourth ventricle; genetics; monozygotic twins; oncology; subependymoma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cerebral Ventricle Neoplasms / diagnosis*
  • Cerebral Ventricle Neoplasms / genetics*
  • Cerebral Ventricle Neoplasms / surgery
  • DNA Copy Number Variations
  • Diseases in Twins*
  • Female
  • Fourth Ventricle / diagnostic imaging
  • Fourth Ventricle / pathology
  • Fourth Ventricle / surgery
  • Glioma, Subependymal / diagnosis*
  • Glioma, Subependymal / genetics*
  • Glioma, Subependymal / surgery
  • Humans
  • Magnetic Resonance Imaging
  • Neurosurgical Procedures
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Twins, Monozygotic*