BACKGROUND: In Japan, a nationwide mass screening (MS) program for preclinical detection of neuroblastoma in infants was done by measuring urinary vanillylmandelic acid and homovanillic acid at the age of 6 months. In this study, clinical, histopathologic, and biologic features of 100 neuroblastomas detected through the Japanese MS are presented. METHODS: Clinical data of the MS cases were collected and histologic and biologic studies performed on the surgically resected neuroblastomas. Histopathologic evaluation was done including the Shimada classification (all tumors), N-myc oncogene status (58 tumors), and ploidy analysis (31 tumors). The serum ferritin level was measured before surgical intervention in 27 cases. RESULTS: The primary tumor sites of these cases were adrenal (69), retroperitoneum (21), and mediastinum (10). The tumors were clinical Stage I (31), II (31), III (19), IV (8), and IV-S (9); two children had bilateral primary adrenal tumors. Ninety-three percent (93/100) had favorable histology; 100% (58/58) had nonamplified N-myc oncogene expression; 81% (25/31) showed a favorable ploidy pattern, and 96% (26/27) had normal serum ferritin levels. To date, all children in this series are alive and well, although a total of 13 tumors were associated with one or two poor risk factors; 6 had unfavorable histology (UH), 5 had an unfavorable ploidy (UP) pattern, one had UH and UP, and one had an elevated ferritin level. CONCLUSIONS: The majority of neuroblastomas detected through the MS showed favorable biologic factors (biologically favorable group). However, there was a small group with histopathologic and/or biologic unfavorable factors. Patients with unfavorable factors apparently benefit most from early surgical intervention.
BACKGROUND: In Japan, a nationwide mass screening (MS) program for preclinical detection of neuroblastoma in infants was done by measuring urinary vanillylmandelic acid and homovanillic acid at the age of 6 months. In this study, clinical, histopathologic, and biologic features of 100 neuroblastomas detected through the Japanese MS are presented. METHODS: Clinical data of the MS cases were collected and histologic and biologic studies performed on the surgically resected neuroblastomas. Histopathologic evaluation was done including the Shimada classification (all tumors), N-myc oncogene status (58 tumors), and ploidy analysis (31 tumors). The serum ferritin level was measured before surgical intervention in 27 cases. RESULTS: The primary tumor sites of these cases were adrenal (69), retroperitoneum (21), and mediastinum (10). The tumors were clinical Stage I (31), II (31), III (19), IV (8), and IV-S (9); two children had bilateral primary adrenal tumors. Ninety-three percent (93/100) had favorable histology; 100% (58/58) had nonamplified N-myc oncogene expression; 81% (25/31) showed a favorable ploidy pattern, and 96% (26/27) had normal serum ferritin levels. To date, all children in this series are alive and well, although a total of 13 tumors were associated with one or two poor risk factors; 6 had unfavorable histology (UH), 5 had an unfavorable ploidy (UP) pattern, one had UH and UP, and one had an elevated ferritin level. CONCLUSIONS: The majority of neuroblastomas detected through the MS showed favorable biologic factors (biologically favorable group). However, there was a small group with histopathologic and/or biologic unfavorable factors. Patients with unfavorable factors apparently benefit most from early surgical intervention.
Authors: T Iehara; H Hosoi; K Akazawa; Y Matsumoto; K Yamamoto; S Suita; T Tajiri; T Kusafuka; E Hiyama; M Kaneko; F Sasaki; T Sugimoto; T Sawada Journal: Br J Cancer Date: 2006-05-22 Impact factor: 7.640