PAPILLOMAS AND CARCINOMAS OF THE CHOROID-PLEXUS IN CHILDREN

Object. Choroid plexus tumors are rare intraventricular tumors (1% of all intracranial tumors) that occur mainly in children. The pathophysi ological characteristics of associated hydrocephalus, surgical managem ent, and oncological issues related to these tumors remain a matter of debate. To understand more about these tumors, the authors have revie wed their experience with the management of 38 children with choroid p lexus tumors. Methods. There were 25 cases of papilloma and 13 of carc inoma. The mean age of the patients at presentation was 22.5 months, a nd one-half of the patients were younger than 2 years of age. Hydrocep halus was present in 33 patients and poorly correlated with the size, site, and pathological characteristics of the tumor. In nine children, a ventriculoperitoneal shunt was required after tumor excision, calli ng into question the notion that cerebrospinal fluid oversecretion is the only cause of hydrocephalus. Complete excision was achieved in 96% of the cases of papilloma and 61.5% of the cases of carcinoma. These surgical procedures were complicated by the risks of intraoperative he morrhage, which proved to be fatal in two cases, and postoperative bra in collapse, which led to subdural fluid collections requiring subdura l shunt placement in six patients. Preoperative embolization was parti ally successful in four cases and significantly assisted surgery. Preo perative controlled drainage of excessively dilated ventricles and int raoperative gluing of the cortical incision have been used to address the problem of postoperative brain collapse. Patients with carcinomas were treated postoperatively by chemotherapy alone (seven cases), radi otherapy (one case), or chemotherapy plus radiotherapy (one case). The overall 5-year survival rate was 100% for patients with papillomas an d 40% for those with carcinomas. Conclusions. Total surgical excision is curative in cases of papillomas. For carcinomas, the most effective treatment remains total surgical excision; however, adjuvant treatmen t in the form of chemotherapy in patients younger than age 3 years, su pplemented by radiation therapy in old er children, can moderately red uce the risk of recurrence.