Comparison of outcomes based on treatment algorithms for rhabdomyosarcoma of the bladder/prostate: Combined results from the Children's Oncology Group, German Cooperative Soft Tissue Sarcoma Study, Italian Cooperative Group, and International Society of Pediatric Oncology Malignant Mesenchymal Tumors Committee

Abstract

The purpose of this study was to determine patient characteristics and outcomes for bladder/prostate (BP) rhabdomyosarcoma (RMS) using an international cohort of prospectively treated patients comparing different treatment algorithms. Data were collected from 379 patients (1979-1998) treated on protocol; Intergroup Rhabdomyosarcoma Study, IRS-IV (n 5 239 patients), International Society of Pediatric Oncology Malignant Mesenchymal Tumors (MMT) Committee MMT-84 and -89 (n 5 74), Italian Cooperative Group, RMS-79 and RMS-88 Studies (n 5 37) or German Cooperative Soft Tissue Sarcoma Study CWS-91 protocols (n 5 29). A total of 322 (85%) patients had localized embryonal RMS (ERMS) and 27 had metastatic disease. Thirty patients (21 local disease; 9 metastatic) had nonembryonal BP RMS. Patients with localized ERMS had large tumors (64% >5 cm) that were invasive (54%) with uninvolved regional lymph nodes (N0, 93%). The 5-year failure-free survival (FFS) was 75% and the overall survival (OS) was 84%, with 89% of deaths attributed to disease. Treatment failures were usually local disease recurrence (60%). Predictors of FFS included T-stage (invasiveness), size, and histology. FFS was decreased for patients not receiving initial radiotherapy but this did not translate into a decreased OS. The 21 patients with localized nonembryonal BP RMS had a FFS and OS of 47%. The 36 patients with metastatic disease were more likely to be older and had large tumors that were invasive with alveolar histology and regional lymph node involvement. The 5-year FFS and OS were 41 and 44%, respectively. In conclusion, the majority of BP RMS patients had localized ERMS with a resultant good prognosis using current treatment algorithms. There were differences in FFS between treatment protocols but this did not result in an altered OS.

Improved care of rhabdomyosarcoma in Jordan using less intensive therapy

“…Moreover, there was significant association between risk groups and 4-year PFS and OS rates: patients with LR and IR group had better outcomes than those with HR diseases, which is consistent with results reported by collaborative groups on RMS [9,15,[18][19][20][21][22][23][24]. Seven (16%) patients had alveolar histology, which was lower than expected as population-based estimates of alveolar histology are around one third of all tumors; however, our study sample is small and it is hard to draw any conclusion from this finding.…”

Paediatric genitourinary cancers and late effects of treatment

“…24 Patients with localized alveolar histology fared worse than patients with localized embryonal histology, but this subtype was much less common (5year OS of 47%, n = 21). Among patients with metastatic bladder or pros tate rhabdomyosarcoma (n = 36), 5year OS was only 44%.…”

A Review on Surgical Techniques and Organ Sparing Procedures in Bladder/Prostate Rhabdomyosarcoma

“…5 This is probably a result of the aggressive radiotherapy for local tumor control in the IRS trial. 5 Overall surgical approach advocates tumor excision at the time of diagnosis only, if it is possible without causing major functional deficits or cosmetic sequelae. Initial biopsy of the tumor represents the best option for most B/P RMS, although very few authors suggested debulking surgery for tumors that could not be completely excised before chemotherapy.…”

“…This may be a biased observation since recent data analysis suggests that the COG and SIOP approaches attain similar EFS when the stage and size of the tumors are properly matched. 5 It needs to be underlined that not only short-term survival rates and local tumor control but also long-term complications, bladder function, and toxicity must be considered when comparing different therapeutic strategies. For cancer survivors, bladder and sexual function, as well as management and treatment of complications, gain increasing importance, although data of late functional effects of survivors are still limited.…”

“…Survival rates have significantly improved during the past 30 years mainly due to collaborative trials. 4,5 The approach to RMS differs slightly between the largest study groups, 6 Staging B/P RMS can be suspected when a patient presents with gross hematuria, especially when hemorrhagic cystitis can be excluded, dysfunctional voiding, and stranguria. But the most common symptoms are related to mass effect of the tumor, that is, urinary obstruction, acute urinary retention, voiding urgency, or abdominal mass.…”