ABSTRACT
Sarcomas of the skull base occurring in the field of prior radiotherapy are rare;
only a few have been reported in the English literature. We report a fatal osteosarcoma
of the skull base arising in a patient with McCune-Albright syndrome and severe fibrous
dysplasia of the skull base 22 years after radiation therapy for a pituitary adenoma.
This malignancy fulfills Cahan's criteria for a radiation-induced tumor in that it
arose in the radiation field of a pituitary adenoma with a latency of 22 years. The
literature on radiation-induced sarcomas of the skull base is reviewed, and the predisposition
of patients with isolated fibrous dysplasia or McCune-Albright syndrome to develop
radiation-induced tumors is discussed. Although the risk of radiation-induced malignancy
is low in the general population, special consideration should be given when contemplating
the use of radiotherapy for benign disease in patients with McCune-Albright syndrome
or isolated fibrous dysplasia.
KEYWORDS
Radiation therapy - sarcoma - fibrous dysplasia - McCune-Albright syndrome
