Pediatric choroid plexus neoplasms

Purpose: Choroid plexus tumors (CPT) are rare childhood neoplasms. The rela tively small number of reported cases and the controversies surrounding the clinical and pathological classification of these tumors have made it diff icult to define a standard of care for these patients. Our intention is to contribute to the body of knowledge of these tumors and further define the role of adjuvant therapy. Methods and Materials: We performed a retrospective review of 14 children w ith choroid plexus neoplasms referred to St. Jude Children's Research Hospi tal between October 1985 and December 1987. Ten patients had choroid plexus carcinoma (CPC) based on pathologic criteria and evidence of brain invasio n at surgery or leptomeningeal disease (M+); 4 patients had choroid plexus papilloma (CPP), Patients with CPP were initially treated with surgery alon e whereas patients with CPC were generally treated with postoperative thera py that included chemotherapy (CT) and/or craniospinal irradiation (CSI) wi th a focal boost to the primary site. For most patients CT consisted of com binations of cyclophosphamide, etoposide, vincristine, and a platinum agent , The median CSI dose was 35.2 Gy (range 24-46.2 Gy), The median primary si te dose was 55.2 Gy (range 49.6-64 Gy), Results: Seven of the 10 CPC cases presented with leptomeningeal disseminat ion; two of these patients have succumbed to disease. Of the 3 patients wit h MO status, all are alive with no evidence of disease (NED), The medial ti me to relapse from the time of surgery was 5.3 mo (range 3-25 mo), Seven CP C patients were treated with gross total resection (GTR), Three of these pa tients (2 M0, 1 M+) received CT without CSI and are currently NED (27, 69, and 60 mo respectively). One M+ patient progressed on CT and has stable dis ease after CSI (6 mo), one (M0) received CT and CSI and is NED (120 mo), on e (M0) is currently on CT with objective response (3 mo) and one (M+) died of progressive disease (24.5 mo) despite CT and CSI, Three patients with CP C had subtotal resection (STR), One of these patients (M+) received CT and CSI and is NED (23 mo), one (M0) had an elective second resection GTR alone and is currently NED (153 mo), and one (M+) developed progressive disease (13.5 mo) while on CT and died despite CSI, Among the 4 CPP patients, GTR w as performed in two; both were NED at 54 and 81 mo, Two patients with CPP t one with focal atypia) were treated with STR initially; both transformed to CPC at 7 and 27 mo, respectively. Both were currently NED following salvag e with (1) GTR and CSI alone (98 mo) or (2) STR, CT, and CSI (62 mo), Six o f the 12 survivors in this series had significant neuropsychological sequel ae, Conclusion: The prognosis of CPP is good for patients treated with GTR, Mal ignant transformation occurred in 2 CPP patients with less than GTR, Patien ts with localized CPC who undergo GTR have had a favorable outcome with the addition of chemotherapy or irradiation. CSI may not be routinely indicate d in M0 children following GTR, There is evidence that salvage with radiati on therapy may be successful following progression on chemotherapy. For pat ients treated with STR, the use of CT and CSI appears to be necessary. (C) 1999 Elsevier Science Inc.