Rhabdomyosarcoma and Undifferentiated Sarcoma in the First Two Decades of Life: A Selective Review of Intergroup Rhabdomyosarcoma Study Group Experience and Rationale for Intergroup Rhabdomyosarcoma Study V

Raney, R. Beverly; Anderson, James R.; Barr, Frederic G.; Donaldson, Sarah S.; Pappo, Alberto S.; Qualman, Stephen J.; Wiener, Eugene S.; Maurer, Harold M.; Crist, William M.

doi:10.1097/00043426-200105000-00008

Cited by 348 publications

(252 citation statements)

References 27 publications

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“…The first IRSG study reported a 55% 5-year OS, which increased to 63% in the IRS-II study [2]. Similarly, the first SIOP study described a 52% OS (EFS 47%) [3].…”

Section: Discussionmentioning

confidence: 96%

“…This has been mainly due to the adoption of well-designed protocols based on a multidisciplinary approach. The first Italian protocol was designed on the strength of the initial IRSG experience [2].…”

Section: Discussionmentioning

confidence: 99%

“…Such trials were initially conducted in the USA, by the Intergroup Rhabdomyosarcoma Study Group (IRSG) [2], and in Europe [3,4]. The Italian Cooperative Group for Pediatric Soft Tissue Sarcomas (now STSC: Soft Tissue Sarcoma Committee) started its first trial in 1979, called the RMS 79.…”

Section: Introductionmentioning

confidence: 99%

“…The treatment plan was based on a risk-related approach, depending on the tumor's characteristics at diagnosis and the results of initial surgery. The primary aims of the RMS 79 protocol were: (1) to treat children with RMS and other sarcomas on a national basis; (2) to improve the survival rates (and patients' quality of life, trying to preserve organ function) in the frame of a multimodality treatment.…”

Section: Introductionmentioning

confidence: 99%

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

Bisogno

Pastore

Perilongo

et al. 2011

Pediatric Blood & Cancer

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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.

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“…The first IRSG study reported a 55% 5-year OS, which increased to 63% in the IRS-II study [2]. Similarly, the first SIOP study described a 52% OS (EFS 47%) [3].…”

Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Bisogno

Pastore

Perilongo

et al. 2011

Pediatric Blood & Cancer

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“…19 There are o20 prostatic rhabdomyosarcomas that have been reported in adults ranging in age from 17 to 68 years old. 20,21 As in younger patients, embryonal subtype is the predominant pattern.…”

Section: Rhabdomyosarcomamentioning

confidence: 99%

Spindle cell lesions of the adult prostate

et al. 2007

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Prostatic spindle cell lesions are diagnostically challenging and encompass a broad array of benign and malignant processes. A subset of these lesions arises only within the prostate and generally represents entities that originate from the prostate epithelium or stroma, such as sclerosing adenosis, sarcomatoid carcinoma, stromal tumors of uncertain malignant potential (STUMP), and stromal sarcoma. Another subset of spindle cell tumors that involve the prostate are also found at other sites and include solitary fibrous tumor, leiomyosarcoma, and neural lesions among others. Finally, tumors may secondarily involve the prostate yet present as primary prostatic processes, as is evident with several cases of gastrointestinal stromal tumors (GIST). The utility of ancillary studies, including immunohistochemistry, is often limited and the main criteria for diagnosis are the morphologic findings by routine H&E stain. This review addresses the various entities that may present as spindle cell tumors within the adult prostate and discusses the functional aspects of the differential diagnosis of these lesions.

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Laryngeal Embryonal Rhabdomyosarcoma

Sivanandan¹,

Kong²,

Kaplan³

et al. 2004

Arch Otolaryngol Head Neck Surg

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Rhabdomyosarcoma and Undifferentiated Sarcoma in the First Two Decades of Life: A Selective Review of Intergroup Rhabdomyosarcoma Study Group Experience and Rationale for Intergroup Rhabdomyosarcoma Study V

Abstract: Better understanding of biologic differences and new, active agents are needed to improve outcome of patients with unfavorable features at presentation.

Cited by 348 publications

References 27 publications

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

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

Spindle cell lesions of the adult prostate

Laryngeal Embryonal Rhabdomyosarcoma

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