Long‐term results in childhood rhabdomyosarcoma: A report from the Italian cooperative study RMS 79

Abstract

BackgroundThe results obtained by protocols for children with rhabdomyosarcoma (RMS) have improved in recent decades. Survival curves usually reach a plateau 3 years after the diagnosis, suggesting that long‐term survival can be expected, but late events are known to occur. We analyzed the long‐term results of the RMS 79 protocol to investigate the type and impact of such events.ProcedureFrom 1979 to 1987, 163 children with RMS diagnosed at 21 Italian institutions were registered. Each institution was contacted every year to record patients' status after the end of treatment. When patients were lost to follow‐up, their status was checked by inquiring at the Registry Offices of the towns of residence and the cause of death or occurrence of second cancers was investigated by contacting the patients or their family by phone.ResultsOverall, 16 patients had late events, that is, 7 tumor recurrences, 6 second tumors, and 3 deaths due to treatment‐related complications. The overall survival rates dropped from 62.6 at 3 years to 52.8 at 20 years. By multivariate analysis, the characteristics influencing long‐term survival were histology, tumor site and size, and IRS group. Factors predictive of any kind of late event were tumor site and IRS group.ConclusionsMajor late events can significantly affect the long‐term survival of children with RMS. Modern protocols should provide for a much longer follow‐up than is usually considered to confirm the results achieved and enable possible correlations between primary treatment and late events to be investigated. Pediatr Blood Cancer 2012; 58: 872–876. © 2011 Wiley Periodicals, Inc.

Long‐term results in children with head and neck rhabdomyosarcoma: A report from the Italian Soft Tissue Sarcoma Committee

“…In all other cases only a biopsy was performed, followed by chemotherapy and delayed surgery and/or radiotherapy (RT). The multidrug combinations used have already been published elsewhere …”

“…Patients in Group III, and those with an alveolar histology received a longer treatment comprising 18 alternating courses of CAV/VAC. All patients were eligible to receive RT except for those in Group I and those in complete remission after primary chemotherapy or delayed surgery …”

Prognostic Factors for Outcome in Localized Extremity Rhabdomyosarcoma. Pooled Analysis from Four International Cooperative Groups

“…Rhabdomyosarcoma (RMS) arises in a multiplicity of sites, and primary location in the extremities accounts for approximately 15% of localized RMS. Sequential clinical studies by international collaborative groups have failed to improve the outcome for children with extremity RMS, which remains suboptimal compared with that of children with RMS at more favorable sites . Several adverse factors are frequently associated with extremity RMS, including older age, alveolar subtype (ARMS), and nodal involvement …”

“…Sequential clinical studies by international collaborative groups have failed to improve the outcome for children with extremity RMS, which remains suboptimal compared with that of children with RMS at more favorable sites. [1][2][3][4][5] Several adverse factors are frequently associated with extremity RMS, including older age, alveolar subtype (ARMS), and nodal involvement. [5][6][7] We describe the results of collaboration between four cooperative study groups in Europe and North America to evaluate prognostic factors for extremity RMS and offer data on the largest number of prospectively treated extremity RMS patients reported to date.…”

An unresolved issue in rhabdomyosarcoma treatment: The duration of chemotherapy

“…Similar to what observed in the SIOP experience, the overall duration of chemotherapy in the Soft Tissue Sarcoma Committee (STSC) trials was reduced in all risk groups from the initial to the most recent studies. This reduction did not compromise the 5‐year survival that has progressively increased from 62.8% in the RMS79 to 79.5% in RMS96 study …”