Intergroup Rhabdomyosarcoma Study-IV: Results for Patients With Nonmetastatic Disease

Crist, William M.; Anderson, James R.; Meza, Jane L.; Fryer, Christopher; Raney, R. Beverly; Ruymann, Frederick B.; Breneman, John C.; Qualman, Stephen J.; Wiener, Eugene S.; Wharam, Moody D.; Lobe, Thom E.; Webber, Bruce L.; Maurer, Harold M.; Donaldson, Sarah S.

doi:10.1200/jco.2001.19.12.3091

Cited by 974 publications

(867 citation statements)

References 22 publications

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“…Sharing the same classification would allow easier comparison between clinical presentation and behavior in adults and children. Finally, specific chemotherapy for A‐RMS and E‐RMS is an independent prognostic value in our study and others 2, 6, 7, 8, 9, 11, 14, 15, 38, 39, 40, 41, 43, 44, 45, 46, 47. North American and European pediatric groups have identified several risk factors (favorable location, IRSG stage, IRSG group, and age) to define the clinical group and deliver a regimen adapted to the risk group.…”

Section: Discussionsupporting

confidence: 78%

“…Since 1972, within IRS group trials, 5‐year OS of children has improved from 55%, 63%, and 69% to 73% in an IRS IV study, representing a threefold increase over these 30 years 6, 7, 8, 9, 36. This improvement did not translate to adult patients, and several hypotheses are suggested involving age, clinical presentation, and intensity/duration of treatment.…”

Section: Discussionmentioning

confidence: 99%

“…Clinical presentation is also less favorable. All retrospective studies note more aggressive disease with more unfavorable sites 1, 36, 37, 38, 39, more LN, more metastasis 9, 36, 40, 41, and more A‐RMS. Nevertheless, all parameters being equal, the outcome of adult patients with RMS is less favorable.…”

Section: Discussionmentioning

confidence: 99%

“…North American and European pediatric groups have identified several risk factors (favorable location, IRSG stage, IRSG group, and age) to define the clinical group and deliver a regimen adapted to the risk group. Thus, reflection should be engaged for common protocols, and PP should be adjusted for risk groups using vincristine, dactinomycin, and alkylating agents 6, 7, 8, 9, 10, 48, 49, 50, 51, 52, 53, as anthracycline is always controversial in PP. For patients up to 50 years old, toxicity in PP is unacceptable with age, as demonstrated through OS or Ewing protocols.…”

Section: Discussionmentioning

confidence: 99%

“…Five‐year overall survival (OS) of children has dramatically improved in the last 30 years based on the results of successive studies of large multinational collaborative trials dedicated to children. Currently, the 5‐year OS exceeds 70% for nonmetastatic RMS 5, 6, 7, 8, 9, 10. In contrast, the outcome of adult patients remains poor.…”

Section: Introductionmentioning

confidence: 99%

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Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

et al. 2018

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Five‐year overall survival (OS) of localized RMS exceeds 70% in children (<18) but is very poor in adult patients. We analyzed the outcome and prognostic factors (PF) of a national series of adult patients with RMS in a large study. The study population consisted of two different cohorts: a retrospective cohort (157 adult patients treated in 13 reference centers between 05/1981 and 02/2010) and the prospective cohort (292 patients with RMS diagnosed and treated between 01/2010 and 12/2014 in France) included in the NetSarc database. A descriptive analysis of patients’ characteristics and prognostic factors was conducted on both series which were compared. In the retrospective series, histological subtypes were embryonal (E‐RMS) for 21% of patients, alveolar (A‐RMS) for 35% of patients, and “adult‐type” P‐RMS (pleomorphic, spindle cell RMS, not otherwise specified) (P) for 44% patients. This distribution significantly differed in the prospective cohort: A‐RMS: 18%; E‐RMS: 17%; and P‐RMS 65%. With a median follow‐up of 8.5 years, 5‐year OS for localized RMS and advanced RMS (with nodes and/or metastases) was 43% and 5%, respectively, (P < 0.0001), and median OS was 51, 33, and 16 months for E‐RMS, A‐RMS, and P‐RMS, respectively, in the retrospective cohort. The median OS was less than 40 months for the prospective nationwide cohort for the entire population. In a multivariate analysis of the retrospective study, independent prognostic factors for OS were A‐RMS, R0 resection, and adjuvant radiotherapy (RT). For localized RMS, age and use of pediatric chemotherapy (CT) regimen are independent prognostic factors. Adult patients with RMS have a poorer overall survival than pediatric patients, and survival varies considerably across histological subtypes.

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Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

et al. 2018

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Arch Surg

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An Aggressive Sinonasal Mass With Parameningeal Extension

Davis

Allison

Mydlarz

2018

JAMA Otolaryngol Head Neck Surg

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A woman in her 60s presented with a 3-month history of right-sided sinus pressure progressing to dental pain. She was prescribed antibiotics and steroids by her family physician, which initially provided intermittent, symptomatic improvement; however, she ultimately experienced a progressive decrease in tactile sensation on her face, anosmia, and changes in her right peripheral vision. She had a remote 2 pack-year smoking history. Sinus computed tomography (CT) and neck magnetic resonance imaging (MRI) revealed a large expansile mass arising from the right side of the skull base and nasopharynx with extension into the pterygopalatine fossa, masticator, parapharyngeal, retropharyngeal, and carotid spaces. In addition, imaging showed involvement of the right posterior orbit with erosion of the posterior maxillary sinus wall, anterior and middle cranial fossa with erosion through the cribriform plate, and cavernous sinus. Physical examination revealed slight rightsided facial fullness, grossly intact extraocular motion, decreased vision in the right lateral field, and palpable bilateral level 2 neck lymph nodes. Flexible laryngoscopy revealed a rightsided lobulated mass involving the medial middle turbinate, posterior septum, and nasal floor, as well as the posterior aspect of the maxillary sinus extending superiorly to the nasopharynx and skull base posteriorly. The mass extended into the left superior nasopharynx and superior septum. In-office biopsy specimens were obtained and revealed a primitive small round cell tumor with the following immunohistochemical (IHC) staining profile: diffusely positive for desmin, myogenin, and CD56; focally positive for low-molecularweight kininogen (LMWCK), p63, and S-100; and negative for AE1/AE3, CK 5/6, CK7, CK20, p40, chromogranin, SOX10, HMB45, CD99, and CD20 (Figure 1).

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Intergroup Rhabdomyosarcoma Study-IV: Results for Patients With Nonmetastatic Disease

Cited by 974 publications

References 22 publications

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

Special Feature

An Aggressive Sinonasal Mass With Parameningeal Extension

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