Early clinical outcomes using proton radiation for children with central nervous system atypical teratoid rhabdoid tumors

Int J Radiat Oncol Biol Phys. 2013 May 1;86(1):114-20. doi: 10.1016/j.ijrobp.2012.12.004. Epub 2013 Mar 13.


Purpose: Atypical teratoid/rhabdoid tumor (AT/RT) is an uncommon and aggressive tumor that often affects infants. Irradiation improves survival but has traditionally been avoided in patients under the age of 3 due to the increasing risk of neurocognitive side effects. We report the first cohort of AT/RT patients treated with proton therapy.

Methods and materials: All patients with AT/RT treated at Massachusetts General Hospital (MGH) Frances H. Burr Proton Beam Therapy Benter between July 2004 and November 2011 were included in this study. All patients were treated with 3-dimensional conformal proton therapy (3D-CPT).

Results: Ten consecutive patients of a median 2.3 years of age and with a median follow-up of 27.3 months (range, 11.3-99.4 months) were identified. Two patients suffered distant relapse; 1 patient was successfully treated with involved field irradiation and chemotherapy, while the second patient died of disease. At last follow-up, 9 patients were alive without evidence of disease. Proton radiation demonstrated increasing sparing of the cerebrum, temporal lobe, cochlea, and hypothalamus.

Conclusions: Initial clinical outcomes with proton therapy are favorable. The advantages of proton therapy are particularly suited to the treatment of AT/RT, a tumor that often requires irradiation treatment at an age when avoiding irradiation to healthy tissues is most desirable.

MeSH terms

  • Adolescent
  • Antineoplastic Agents / adverse effects
  • Central Nervous System Neoplasms / drug therapy
  • Central Nervous System Neoplasms / radiotherapy*
  • Central Nervous System Neoplasms / surgery
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Infratentorial Neoplasms / drug therapy
  • Infratentorial Neoplasms / radiotherapy
  • Infratentorial Neoplasms / surgery
  • Male
  • Organ Sparing Treatments / methods
  • Proton Therapy / methods*
  • Radiotherapy, Conformal / methods*
  • Rhabdoid Tumor / drug therapy
  • Rhabdoid Tumor / radiotherapy*
  • Rhabdoid Tumor / surgery
  • Supratentorial Neoplasms / drug therapy
  • Supratentorial Neoplasms / radiotherapy
  • Supratentorial Neoplasms / surgery
  • Teratoma / drug therapy
  • Teratoma / radiotherapy*
  • Teratoma / surgery
  • Treatment Outcome
  • Young Adult


  • Antineoplastic Agents