Parameningeal Rhabdomyosarcoma: Outcomes and Opportunities

Yang, Joanna C.; Wexler, Leonard H.; Meyers, Paul A.; Wolden, Suzanne L.

doi:10.1016/j.ijrobp.2012.08.019

Cited by 29 publications

(31 citation statements)

References 15 publications

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“…However, the rates of local failure for clinical group III patients were similar for both histologies. Reports focusing on local control from large single institutions have also made this observation (7, 8). Therefore, for patients with alveolar RMS, strategies for prevention of distant metastases are important for improving EFS.…”

Section: Discussionmentioning

confidence: 81%

Local Control for Intermediate-Risk Rhabdomyosarcoma: Results From D9803 According to Histology, Group, Site, and Size: A Report From the Children's Oncology Group

Wolden

Lyden

Arndt

et al. 2015

International Journal of Radiation Oncology*Biology*Physics

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Purpose To determine local control according to clinical variables for patients with intermediate-risk rhabdomyosarcoma (RMS) treated on Children’s Oncology Group protocol D9803. Patients and Methods Of 702 patients enrolled, we analyzed 423 patients with central pathology–confirmed group III embryonal (n = 280) or alveolar (group III, n = 102; group I–II, n = 41) RMS. Median age was 5 years. Patients received 42 weeks of VAC (vincristine, dactinomycin, cyclophosphamide) or VAC alternating with VTC (T = topotecan). Local therapy with 50.4 Gy radiation therapy with or without delayed primary excision began at week 12 for group III patients. Patients with group I/II alveolar RMS received 36–41.4 Gy. Local failure (LF) was defined as local progression as a first event with or without concurrent regional or distant failure. Results At a median follow-up of 6.6 years, patients with clinical group I/II alveolar RMS had a 5-year event-free survival rate of 69% and LF of 10%. Among patients with group III RMS, 5-year event-free survival and LF rates were 70% and 19%, respectively. Local failure rates did not differ by histology, nodal status, or primary site, though there was a trend for increased LF for retroperitoneal (RP) tumors (P = .12). Tumors ≥5 cm were more likely to fail locally than tumors <5 cm (25% vs 10%, P = .0004). Almost all (98%) RP tumors were ≥5 cm, with no difference in LF by site when the analysis was restricted to tumors ≥5 cm (P = .86). Conclusion Local control was excellent for clinical group I/II alveolar RMS. Local failure constituted 63% of initial events in clinical group III patients and did not vary by histology or nodal status. The trend for higher LF in RP tumors was related to tumor size. There has been no clear change in local control over RMS studies, including IRS-III and IRS-IV. Novel approaches are warranted for larger tumors (≥5 cm).

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Section: Discussionmentioning

confidence: 81%

Local Control for Intermediate-Risk Rhabdomyosarcoma: Results From D9803 According to Histology, Group, Site, and Size: A Report From the Children's Oncology Group

Wolden

Lyden

Arndt

et al. 2015

International Journal of Radiation Oncology*Biology*Physics

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“…In few patients, surgical removal of the entire tumor was achievable and performed after chemotherapy. Typically, radiation therapy is delivered months after chemotherapy, in the form of intensity-modulated radiation therapy/proton beam radiation therapy [39, 47]. Patients with intermediate and high risk RMS are offered additional chemotherapy, such as irinotecan and carboplatin [48].…”

Section: Methodsmentioning

confidence: 99%

“…The management of HNRMS remains challenging due to an increased failure of local control as well as a high rate of early metastases [6, 39]. In the last three decades, RMS outcomes have improved significantly due to evolving multidisciplinary therapy paradigms, such as improved surgical techniques, aggressive chemotherapy regimen and more effective conformal intensity-modulated radiotherapy [40, 41].…”

Section: Introductionmentioning

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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“…The current treatment guidelines for RMS emphasize the importance of a multimodal approach [4]. However, surgical resection with an adequate margin is difficult because of its deep location, which is close to several important structures, such as the brain, cranial nerve, and many vessels.…”

Section: Introductionmentioning

confidence: 99%

“…The current guideline for RT is a dosage of 1.8 Gy/day, up to a total of 50.4 Gy, using a 1.5–2 cm margin around the tumor [4]. Michalski et al [10] argued that a dose of at least 47.5 Gy was associated with lower rates of local failure.…”

Section: Introductionmentioning

confidence: 99%

The impact of radiotherapy on clinical outcomes in parameningeal rhabdomyosarcoma

Choi

Lim

2016

Radiat Oncol J

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PurposeRadiotherapy (RT) is considered a mainstay of treatment in parameningeal rhabdomyosarcoma (PM-RMS). We aim to determine the treatment outcomes and prognostic factors for PM-RMS patients who treated with RT. In addition, we tried to evaluate the adequate dose and timing of RT.Materials and MethodsTwenty-two patients with PM-RMS from 1995 to 2013 were evaluated. Seven patients had intracranial extension (ICE) and 17 patients had skull base bony erosion (SBBE). Five patients showed distant metastases at the time of diagnosis. All patients underwent chemotherapy and RT. The median radiation dose was 50.4 Gy (range, 40.0 to 56.0 Gy).ResultsThe median follow-up was 28.7 months. Twelve patients (54.5%) experienced failure after treatment; 4 local, 2 regional, and 6 distant failures. The 5-year local control (LC) and overall survival (OS) were 77.7% and 38.5%, respectively. The 5-year OS rate was 50.8% for patients without distant metastases and 0% for patients with metastases (p < 0.001). Radiation dose (<50 Gy vs. ≥50 Gy) did not compromise the LC (p = 0.645). However, LC was affected by ICE (p = 0.031). Delayed administration (>22 weeks) of RT was related to a higher rate of local failure (40.0%).ConclusionRT resulted in a higher rate of local control in PM-RMS. However, it was not extended to survival outcome. A more effective treatment for PM-RMS is warranted.

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Parameningeal Rhabdomyosarcoma: Outcomes and Opportunities

Cited by 29 publications

References 15 publications

Local Control for Intermediate-Risk Rhabdomyosarcoma: Results From D9803 According to Histology, Group, Site, and Size: A Report From the Children's Oncology Group

Local Control for Intermediate-Risk Rhabdomyosarcoma: Results From D9803 According to Histology, Group, Site, and Size: A Report From the Children's Oncology Group

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

The impact of radiotherapy on clinical outcomes in parameningeal rhabdomyosarcoma

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