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

Carcinomas of the plexus choroideus (PCC) represent the rare malignant variety of plexus choroideus papillomas and take a particularly unfav ourable 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 intrac ranial 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. P rognosis is poor for patients treated only by surgery, which in most c ases has been performed as subtotal resection of the tumor. So the nec essity for an oncological strategy combining surgery and 'adjuvant' th erapy 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 ventr icle. After nearly four years of remission, the boy died of meningeosi s carcinomatosa. This course underlines the malignancy of this tumor; even after years the poor prognosis can still be diminished by the spr ead of meningeal metastases. In a survey of the case reports published in literature the patients' data, their therapy and the outcome are d emonstrated. These observations lead to the conclusion, that an 'adjuv ant' oncological therapy following a surgical resection as extensive a s possible has to be required as the up-to-date treatment of PCC. As m ost of the patients are infants chemotherapy should be prefered to imm ediate postoperative radiotherapy.