Metastatic Group 3 Medulloblastoma in a Patient With Tuberous Sclerosis Complex: Case Description and Molecular Characterization of the Tumor

Pediatr Blood Cancer. 2016 Apr;63(4):719-22. doi: 10.1002/pbc.25851. Epub 2015 Dec 2.

Abstract

Medulloblastoma is the most common pediatric brain tumor. We describe a child with tuberous sclerosis complex that developed a Group 3, myc overexpressed, metastatic medulloblastoma (MB). Considering the high risk of treatment-induced malignancies, a tailored therapy, omitting radiation, was given. Based on the evidence of mammalian target of rapamycin mTORC, mTOR Complex; RAS, Rat sarcoma; RAF, rapidly accelerated fibrosarcoma (mTOR) pathway activation in the tumor, targeted therapy was applied resulting in complete remission of disease. Although the PI3K/AKT/mTOR signaling pathway plays a role in MB, we did not find TSC1/TSC2 (TSC, tuberous sclerosis complex) mutation in our patient. We speculate that a different pathway resulting in mTOR activation is the basis of both TSC and MB in this child; H&E, haematoxilin and eosin; Gd, gadolinium.

Keywords: TSC1/2; mTOR; medulloblastoma.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Blotting, Western
  • Cerebellar Neoplasms / complications
  • Cerebellar Neoplasms / genetics
  • Cerebellar Neoplasms / pathology*
  • DNA Mutational Analysis
  • Female
  • Humans
  • Medulloblastoma / complications
  • Medulloblastoma / genetics
  • Medulloblastoma / pathology*
  • TOR Serine-Threonine Kinases / biosynthesis
  • Tuberous Sclerosis / complications*
  • Tuberous Sclerosis / genetics

Substances

  • MTOR protein, human
  • TOR Serine-Threonine Kinases