Adrenocortical carcinoma (ACC), a very rare tumor in children in the United States, is apparently more common among Brazilian children. We reviewed the medical records of 40 children whose disease was diagnosed between 1966 and 1987. There were 12 boys and 28 girls; their median age was 3.9 years (range, 1 day to 15.7 years). Virilization was the most common clinical sign (37 of 40); other signs included abdominal mass, deepened voice, plethora, hypertension, seizures (seven patients) and, rarely, weight loss (two patients). The median time between first signs or symptoms and diagnosis was 1.4 years (range, 3 days to 5 years). Four of 33 tumors were classified as benign according to the Weiss, van Slooten, or Hough systems (tumor tissue was unavailable for seven patients). Tumors were completely resected in 26 of 38 patients; of those, 17 are in continuous complete remission, five relapsed, and four have been lost to follow-up. One patient, who had local recurrence, has been in a third complete remission for 18+ months after tumor resection and chemotherapy (cisplatin and etoposide). Of the remaining 14 patients, 11 died of progressive disease, the diagnosis was confirmed at autopsy in two, and one has been lost to follow-up. Univariate analysis disclosed that age greater than or equal to 3.5 years at diagnosis, interval of greater than or equal to 6 months between first symptoms and diagnosis, tumor weight greater than 100 g, tumor size greater than 200 cm3, and high levels of urinary 17-ketosteroids (17-KS) and 17-hydroxycorticosteroids (17-OH) were associated with an unfavorable outcome. Multivariate analysis disclosed that only a tumor size greater than 200 cm3 independently identifies those patients with an unfavorable prognosis. Among the variables known before surgery, age, and the interval between first symptoms and diagnosis were important predictors of outcome. Our data suggest that some children with ACC and certain clinical characteristics are at high risk of primary treatment failure and, therefore, are good candidates for investigational adjuvant therapy.
Radiation therapy remains the only treatment that provides clinical benefit to children with diffuse brainstem tumors. Their median survival, however, rarely exceeds 9 months. The authors report a prospective trial of frontline chemotherapy aimed at delaying radiation until time of clinical progression. The aim was to investigate the possibility that radiotherapy would maintain its activity in children whose disease progressed after chemotherapy. Twenty-three patients took part in this protocol, the BSG 98 protocol, which consisted of frontline chemotherapy alternating hematotoxic and nonhematotoxic schedules. Each cycle included three courses delivered monthly; the first course was 1,3-bis(2-chloroethyl)-1-nitrosoureacisplatin, and the second and third were high-dose methotrexate. Three patients underwent one cycle; 5 patients each, two and three cycles; and 10 patients, four cycles. Twenty of the 23 patients eventually received local radiation therapy. A historical cohort of 14 patients who received at least local radiation therapy served as controls. Four patients experienced severe iatrogenic infections, and 11 patients required platelet transfusions. Median survival increased significantly in patients participating in the protocol compared to that in the historical controls (17 months, 95% confidence interval [CI], 10-23 months, vs. 9 months, 95% CI, 8-10 months; p = 0.022), though hospitalization was prolonged (57 vs. 25 days, p = 0.001). Although frontline chemotherapy alternating hematotoxic and nonhematotoxic schedules significantly increases overall median survival, its cost from infection and hospitalization deserves honest discussion with the children and their parents.
The present study compares the outcome of 40 children (39%) transplanted without prior dialysis, i.e., preemptive transplantation (PET), with 63 children (61%) transplanted after a variable duration of dialysis, i.e., pretransplantation dialysis (PTD). The two groups were matched for recipient and donor age and for immunological risk factors. There was no statistical difference in the time to first acute rejection episode nor in the number of acute rejection episodes during the 1st year after renal transplantation. In the PET group, 78% of the recipients received blood transfusion versus 92.5% in the PTD group (P < 0.05), and the average number of blood units per patient was 3.2 and 7.8, respectively (P < 0.05). Arterial hypertension was found in 55% of the patients in the PET group versus 73% in the PTD group (P < 0.05). The number of functioning grafts at the end of the study period was 87.5% in the PET group and 73% in the PTD group (NS). The major cause of graft failure was vascular thrombosis in the PET group (3/5) and chronic allograft rejection in the PTD group (10/17). In the PET group, the actuarial graft survival rate was 100%, 84%, 81%, and 76% at 1, 3, 5, and 7 years, which was not statistically different from the PTD group at 1, 3, and 5 years (98%, 91%, and 73%, respectively) but there was a significantly lower graft survival (59%) after 7 years in the PTD (P < 0.05). The 7-year actuarial patient survival rate was 97% in the PET group and 90% in the PTD group (NS). In the PTD group, children on dialysis for less than 1 year (group 1, n = 25) were compared with those on dialysis for more than 1 year (group 2, n = 38). Arterial hypertension was noted in 40% of patients from group 1 and 65% from group 2 (P < 0.05); there was no significant difference in graft loss rate. In conclusion, these results confirm PET as the preferred approach rather than PTD in children who need renal replacement therapy.
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