THERAPY OF CARCINOMA OF PLEXUS CHOROIDEUS - CASE-REPORT AND REVIEW OF THE LITERATURE

Carcinomas of the plexus choroideus (PCC) represent the rare malignant variety of plexus choroideus papillomas and take a particularly unfavourable course. Tumors of the plexus choroideus account for about 2-4% of all primary brain tumors in children and 0.5% of those in adults. The PCC is more frequent in children than in adults; the authors found reports on 72 cases of PCC in children and on 16 cases in adults. In most cases the PCC is located in the lateral ventricles. The symptoms caused by PCC are non specific and appear as those of increased intracranial pressure (on the basis of hydrocephalus hypersecretorius and/or occlusivus). As the nature of the tumor cannot be identified by means of medical imaging, the diagnosis is usually set up histologically. Prognosis is poor for patients treated only by surgery, which in most cases has been performed as subtotal resection of the tumor. So the necessity for an oncological strategy combining surgery and 'adjuvant' therapy arises. The authors report the application of such a strategy in the case of a three-year-old boy with a PCC of the left lateral ventricle. After nearly four years of remission, the boy died of meningeosis carcinomatosa. This course underlines the malignancy of this tumor; even after years the poor prognosis can still be diminished by the spread of meningeal metastases. In a survey of the case reports published in literature the patients' data, their therapy and the outcome are demonstrated. These observations lead to the conclusion, that an 'adjuvant' oncological therapy following a surgical resection as extensive as possible has to be required as the up-to-date treatment of PCC. As most of the patients are infants chemotherapy should be prefered to immediate postoperative radiotherapy.