Parameningeal rhabdomyosarcoma in pediatric age: results of a pooled analysis from North American and European cooperative groups

Merks, Johannes H. M.; Salvo, Gian Luca De; Bergeron, Christophe; Bisogno, Gianni; Paoli, Angela De; Ferrari, Andrea; Rey, Annie; Oberlin, Odile; Stevens, Megan; Kelsey, Anna; Michalski, Jeff M.; Hawkins, Douglas S.; Anderson, James R.

doi:10.1093/annonc/mdt426

Cited by 73 publications

(76 citation statements)

References 22 publications

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“…These findings suggest that the additional AEs introduced by surgery, as part of AMORE, do not outweigh the reduction of AEs achieved with brachytherapy. Overall survival was similar for both treatment groups and comparable to international figures [8,30]. The (non-significant) difference in FFS between treatment groups may be explained by the higher number of patients that initially received no local treatment in the AMORE-based treatment group compared to the EBRT-based treatment group (19/80 (24%) versus 5/73 (7%), p = 0.006), as is allowed in the SIOP approach.…”

Section: Discussionsupporting

confidence: 57%

“…This may also partly explain the difference in FFS between the two time eras in the AMORE-based treatment group (Table S3); local treatment was more rigorously applied in the most recent decade. The advantage of withholding radiotherapy in patients achieving complete remission (CR) with chemotherapy alone is that a number of patients with favourable prognostic characteristics can be cured despite withholding radiotherapy [8,30]. However, this gain should be weighed against the increased burden of (systemic) treatment in patients suffering a relapse [31].…”

Section: Discussionmentioning

confidence: 99%

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Adverse events of local treatment in long-term head and neck rhabdomyosarcoma survivors after external beam radiotherapy or AMORE treatment

Schoot

Slater

Ronckers

et al. 2015

European Journal of Cancer

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Section: Discussionsupporting

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Adverse events of local treatment in long-term head and neck rhabdomyosarcoma survivors after external beam radiotherapy or AMORE treatment

Schoot

Slater

Ronckers

et al. 2015

European Journal of Cancer

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“…In contrast with other anatomic locations, HNRMS encompass certain particularities such as an increased rate of spindle/sclerosing (SRMS/ScRMS) histology, high stage and recurrence rates, with often limited benefit from surgical management due to its proximity to vital structures. Prior studies of HNRMS have focused mainly on specific subsites and a recent study used the National Cancer Institute's (NCI) SEER database of multiple geographic regions and different therapy regimen [6, 45, 46]. In this study, we performed a detailed clinicopathologic and survival analysis of HNRMS managed at a single tertiary center during a 20-year period (1996-2015).…”

Section: Discussionmentioning

confidence: 99%

A clinicopathologic study of head and neck rhabdomyosarcomas showing FOXO1 fusion-positive alveolar and MYOD1 -mutant sclerosing are associated with unfavorable outcome

et al. 2016

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Background Based on their distinctive histologic and genetic features, the latest WHO classification of soft tissue tumors includes four pathologic variants of rhabdomyosarcoma (RMS): embryonal (ERMS), alveolar (ARMS), spindle cell-sclerosing (SRMS-ScRMS) and pleomorphic RMS. The aim of this study focused on a detailed clinicopathologic and survival analysis of head and neck RMS (HNRMS) using the latest pathologic and molecular criteria reflecting this new subclassification in a large cohort. Patients and Methods Patients managed for HNRMS in our institution (1996 - 2015) were analyzed. The presence of a FOXO1 fusion was required for the classification of ARMS. MYOD1 mutations in SRMS-ScRMS were tested when material available. Univariate and multivariate analyses were performed to evaluate variables related to overall survival (OS). Results Ninety-nine HNRMS patients (52 males and 47 females, mean of 16 years) were included in the study after pathologic re-review. The most common location was parameningeal (PM) (n=64), followed by non-orbital/non-PM (n=25) and orbital (n=10). There were 53 ERMS, 33 fusion-positive ARMS and 13 SRMS-ScRMS [SRMS (8); ScRMS (5)]. The 5-year OS rate for ERMS patients was significantly higher (82%) compared to ARMS (53%) and SRMS-ScRMS (50%) [SRMS (75%); ScRMS (30%)]. Univariate analysis showed that survival was dependent on histology (P=0.012), tumor size > 5 cm (P<0.001), regional lymph node involvement (P=0.002), metastasis at initial presentation (P<0.001), stage (P<0.001), and recurrence (P=0.002). Multivariate analysis confirmed histologic subtype to be significant (P=0.043). Conclusion Our findings reinforce that HNRMS is a heterogenous disease with ARMS and SRMS-ScRMS having an equally unfavorable outcome.

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“…Advances in systemic and local therapy have led to increased survival rates, with more than 70% of children becoming long term survivors 3,4 . Radiation therapy (RT) is an integral component of treatment in many of these patients but can be associated with both short and long-term morbidity, depending upon the volume treated and the dose delivered 5-10 . RMS may occur at almost any site in the body, and acute toxicity and late complications from radiation therapy depend on the location being treated.…”

Section: Introductionmentioning

confidence: 99%

A dosimetric comparison of proton and intensity modulated radiation therapy in pediatric rhabdomyosarcoma patients enrolled on a prospective phase II proton study

Ladra

Edgington

Mahajan

et al. 2014

Radiotherapy and Oncology

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Background Pediatric rhabdomyosarcoma (RMS) is highly curable, however, cure may come with significant radiation related toxicity in developing tissues. Proton therapy (PT) can spare excess dose to normal structures, potentially reducing the incidence of adverse effects. Methods Between 2005 and 2012, 54 patients were enrolled on a prospective multi-institutional phase II trial using PT in pediatric RMS. As part of the protocol, intensity modulated radiation therapy (IMRT) plans were generated for comparison with clinical PT plans. Results Target coverage was comparable between PT and IMRT plans with a mean CTV V95 of 100% for both modalities (p=0.82). However, mean integral dose was 1.8 times higher for IMRT (range 1.0-4.9). By site, mean integral dose for IMRT was 1.8 times higher for H&N (p<0.01) and GU (p=0.02), 2.0 times higher for trunk/extremity (p<0.01), and 3.5 times higher for orbit (p<0.01) compared to PT. Significant sparing was seen with PT in 26 of 30 critical structures assessed for orbital, head and neck, pelvic, and trunk/extremity patients. Conclusions Proton radiation lowers integral dose and improves normal tissue sparing when compared to IMRT for pediatric RMS. Correlation with clinical outcomes is necessary once mature long-term toxicity data are available.

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Parameningeal rhabdomyosarcoma in pediatric age: results of a pooled analysis from North American and European cooperative groups

Cited by 73 publications

References 22 publications

Adverse events of local treatment in long-term head and neck rhabdomyosarcoma survivors after external beam radiotherapy or AMORE treatment

Adverse events of local treatment in long-term head and neck rhabdomyosarcoma survivors after external beam radiotherapy or AMORE treatment

A clinicopathologic study of head and neck rhabdomyosarcomas showing FOXO1 fusion-positive alveolar and MYOD1 -mutant sclerosing are associated with unfavorable outcome

A dosimetric comparison of proton and intensity modulated radiation therapy in pediatric rhabdomyosarcoma patients enrolled on a prospective phase II proton study

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