Atypical Teratoid Rhabdoid Tumor in a Teenager with Unusual Infiltration Into the Jugular Foramen

World Neurosurg. 2015 Dec;84(6):2075.e13-6. doi: 10.1016/j.wneu.2015.07.015. Epub 2015 Jul 14.

Abstract

Background: Atypical teratoid rhabdoid tumor is a rare malignant neoplasm that represents 1%-2% of all pediatric central nervous system tumors. Immunohistochemistry plays an important role in establishing the diagnosis with a loss of INI-1 staining in tumor cells. In this case report, we describe a teenager with an unusual presentation and pattern of infiltration of the tumor.

Case description: A 13-year-old boy presented with a history over several months of progressive nausea, weight loss, and hoarseness of voice associated with multiple lower cranial nerve palsies on neurologic examination. Magnetic resonance imaging revealed a large heterogeneously enhancing extra-axial neoplasm with extension and bony expansion of the jugular foramen. After near total resection, neuropathology demonstrated the absence of INI-1 expression consistent with a diagnosis of atypical teratoid rhabdoid tumor.

Conclusions: This case highlights the diverse clinical presentation and infiltrative potential of atypical teratoid rhabdoid tumors, thus expanding the differential diagnosis of extra-axial tumors invading the jugular foramen.

Keywords: Atypical teratoid rhabdoid tumor; Jugular foramen; Neuroimaging.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Chemoradiotherapy
  • Combined Modality Therapy
  • Cranial Nerve Diseases / etiology
  • Hoarseness / etiology
  • Humans
  • Intraoperative Neurophysiological Monitoring
  • Male
  • Nausea / etiology
  • Neoplasm Invasiveness
  • Neuroimaging
  • Neurologic Examination
  • Neurosurgical Procedures / methods*
  • Rhabdoid Tumor / pathology*
  • Rhabdoid Tumor / surgery*
  • Skull Base Neoplasms / pathology*
  • Skull Base Neoplasms / surgery*
  • Treatment Outcome