Supratentorial Primitive Neuroectodermal Tumors in Children Homepage

 

 

Authors

Ash Singhal, M.D.

Paul Steinbok, M.D.

Section Editor

Paul Steinbok, M.D.

Editor in Chief

Rick Abbott, M.D.

Introduction

Supratentorial PNETs are rare, highly malignant, embryonal tumors, which are similar in histology to pineoblastomas and medulloblastomas. They tend to present early in childhood and the symptoms are rapid in evolution. The expected survival rate after multimodality treatment that includes surgical resection followed by radiation and chemotherapy is 40-45%.

Key Points

  • Different from posterior fossa PNET: While supratentorial PNETs are similar to posterior fossa PNETs (aka medulloblastomas) morphologically, these tumors are different cytogenically.
  • Metastasis predicts treatment failure: Metastasis at the time of initial diagnosis predicts failure of local treatments.
  • Reoperation for local recurrence: If there is recurrence of tumor locally then surgical resection is seriously considered.  Recurrence at a distal site is handled with radiation or chemotherapy.