15 YEARS REVIEW OF ADVANCED CHILDHOOD NEUROBLASTOMA FROM A SINGLE INSTITUTION IN HONG-KONG

Objective. To assess the progress in the treatment of advanced childho od neuroblastoma. Methods. From 1981 to 1996, there were 32 children w ith neuroblastoma (NB) diagnosed, staged and treated in our institutio n. There were 4 patients with stage II NE (12%), 5 stage III (16%), 21 stage IV (66%) and 2 stage IVs (6%). The NBs were excised if CT scan indicated that the tumors were operable. For advanced NE, stages III a nd IV, multiple drug chemotherapy was started first and operability wa s assessed with serial CT scan examinations. Once the X-ray imaging in dicated the tumors were operable, surgical interventions were done. Th e medical records of the advanced NE were reviewed. Results. In the in itial period of the study, 9 patients were treated using the VAC proto col [vincristine (vcr), adriamycin(adria) and cyclophosphamide (cyc)]. No patient was convertible to operable and all died with a mean survi val of 10 months. OPEC [vcr, eye, VM26, cisplatin (cis)], Rapid COJEC (carboplatin, VP16, ver, cis and eye) and more recently N6 protocol (c yc, adria, ver, VP16, cis) was used for 17 patients. 80% of them were converted to operable. In 4 patients, surgical specimens showed only n ecrotic tissue without viable tumor tissue and 6 (35%) tumors were con verted to ganglioneuroma or ganglioneuroblastoma. Although 2 (12%) pat ients died of fungal septicemia and 1 (6%) developed Fanconi's syndrom e after chemotherapy, the mean survival period increased to 27 months. In the 10 survivors (60%), 4 had megatherapy with melphalan followed by autologous peripheral blood stem cell (PBSC) transplantation and 2 were waiting for transplantation. Conclusions. There is a high percent age of advanced NE on presentation in Hong Kong. With more potent mult iple drug chemotherapy for advanced stage NE there are (1) improvement in the survival of these patients, (2) opportunities for more operati ons for tumor excision and (3) opportunities for autologous PBSC trans plantation for better tumor eradication.