The case of a 33-year-old man with a primary leiomyosarcoma arising in
a mature teratoma in the pineal area is presented, The tumor extended
into the posterior part of the third ventricle and caused hydrocephal
us. Its smooth muscle derivation was confirmed by immunohistochemistry
and electron microscopy. The patient has been followed for more than
2 years after surgery and postoperative radiotherapy. He has full work
ing capacity and there are no signs of tumor recurrence. To our knowle
dge this is the first presentation of a leiomyosarcoma derived from a
teratoma in the pineal area.