Treatment of children and adolescents with localized parameningeal sarcoma: experience of the Intergroup Rhabdomyosarcoma Study Group protocols IRS-II through -IV, 1978-1997

BACKGROUND: We reviewed 611 patients with parameningeal sarcoma entered on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-II through IV (1978-1997), to delineate treatment results and evaluate prognostic factors. PROCEDURE: Primary sites were the middle ear/mastoid (N = 138), nasopharynx/nasal cavity (N = 235), paranasal sinuses (N = 132), parapharyngeal region (N = 29), and the pterygopalatine/infratemporal fossa (N = 77). Treatment was initial biopsy or surgery followed by multiagent chemotherapy and radiation therapy (XRT). Beginning in 1977, patients with cranial nerve palsy, cranial base bony erosion, and/or intracranial extension at diagnosis were considered as having meningeal involvement. They received triple intrathecal medications, whole brain XRT, and then spinal XRT. These treatments were successively eliminated from 1980 to 1991. RESULTS: The 611 patients' overall survival rate at 5 years was 73% (95% confidence interval, 70-77%). Favorable prognostic factors were: age 1-9 years at diagnosis; primary tumor in the nasopharynx/nasal cavity, middle ear/mastoid, or parapharyngeal areas; no meningeal involvement; and non-invasive tumors (T1). Thirty-five of 526 patients (6.7%) with information about presence/absence of meningeal involvement at diagnosis developed central nervous system (CNS) extension at 5-164 weeks (median, 46 weeks) after starting therapy. The estimated 5-year cumulative incidence rate of CNS extension during the study period was 5-7% (P = 0.88). CONCLUSIONS: Biopsy, XRT to the target volume, and systemic chemotherapy are successful treatments for the large majority of patients with localized parameningeal sarcoma. Carefully defining and irradiating the initial volume should reduce the risk of CNS failure. Aggressive initial surgical management of these patients is unnecessary.     

Treatment results for patients with localized,completely resected (Group I) alveolar rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984–1997: A report from the Children's Oncology Group

Purpose

To assess local control, event‐free survival (EFS), and overall survival (OS) rates in 71 patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma (ALV RMS) and their relation to radiation therapy (RT) on IRSG Protocols III and IV, 1984–1997.

Methods

Chart review and standard statistical procedures.

Patients and Tumors

Patients were 1–18 years at diagnosis (median, 6 years). Primary tumor sites were extremity/trunk (N = 54), head/neck (N = 9), genitourinary tract (N = 7), and perineum (N = 1). Thirty patients received VA ± C with RT; 41 received VA ± C alone. RT was assigned, not randomized.

Results

Fifty‐four patients had Stage 1 (favorable site, any size) or Stage 2 (unfavorable site, ≤5 cm) tumors. Eight‐year EFS was 90%, with 100% local control for 17 patients given RT. Eight‐year EFS was 88%, with 92% local control for 37 patients without RT; P = 0.52 for EFS comparisons, 0.3 for local control comparisons. In 17 Stage 3 patients (unfavorable site, tumors >5 cm, N0), 8‐year EFS was 84% with 100% local control in 13 patients given RT; 8‐year EFS was only 25% and local control 50% in 4 patients without RT. Local recurrence was the most common site of first failure in non‐irradiated patients.

Conclusion

Patients with Stage 1–2 ALV RMS had slightly but statistically insignificantly improved local control, EFS, and OS rates when local RT was given. The need for local RT in Stage 1–2 patients deserves evaluation in a randomized study. Local control, EFS, and OS rates were significantly improved in Stage 3 patients receiving local RT. Pediatr Blood Cancer. 2010;55:612–616. © 2010 Wiley‐Liss, Inc.     

Prevalence,risk factors,and consequences of overweight in children and adolescents who underwent renal transplantation – Short‐ and medium‐term analysis

To determine the prevalence and risk factors for (i) overweight/obesity and (ii) weight gain six months after transplantation and to study the effect of weight excess on graft function and survival. We performed a retrospective study of kidney transplanted children. Endpoints: (i) prevalence of overweight/obesity at sixth month, (ii) gaining 1.0 BMI SDS from one to six months. To study the effects of weight excess, graft function and survival at 36 months were the endpoints. The study included 197 individuals. At sixth month, 57/197 (29%) presented overweight/obesity, and the factors associated to this outcome were: (i) age at transplantation (OR = 3.04) and (ii) overweight/obesity in the first month (OR = 22.16). Groups presented no difference on graft function and survival at 36 months. From one to six months, 90/197 (46%) patients gained >1.0 BMI SDS. This outcome was associated with (i) female sex (OR = 2.50), (ii) steroids' pulses (OR = 2.98), (iii) steroids exposure (OR = 1.04), and (iv) living donor (OR = 2.69). The group that gained BMI presented a lower 36 months graft survival (86% vs. 98%, p < 0.001). Weight excess and gain after transplantation are frequent, particularly in younger female recipients and in those receiving high steroids exposure. The lower graft survival in patients with rapid weight gain deserves investigation.     

Outcomes of children with rhabdomyosarcoma treated with intensive chemotherapy,surgery, and radiotherapy through a period of protocol revision at a South African center, 1990–2010

A total of 75 children with biopsy‐proven rhabdomyosarcoma were treated at our institution between 1990 and 2010. Five‐year overall survival (OS) for the entire cohort was 58.7%. OS by stage was as follows: Stage 1 (80%), Stage 2 (80%), Stage 3 (54.1%), and Stage 4 (38.5%). There was a trend to suggest that revision of treatment approaches improved crude survival over time: pre‐2003 (OS 42.1%); 2003–2005 (OS 50.0%); 2005–2010 (OS 60.8%).