Treatment of Nonmetastatic Rhabdomyosarcoma in Childhood and Adolescence: Third Study of the International Society of Paediatric Oncology—SIOP Malignant Mesenchymal Tumor 89

Stevens, Michaël C.G.; Rey, Annie; Bouvet, Nathalie; Ellershaw, Caroline; Flamant, F; Habrand, Jean Louis; Marsden, H. B.; Martelli, Hélène; Toledo, José Sánchez de; Spicer, R. D.; Spooner, D.; Terrier-Lacombe, Marie-Josée; Unnik, Adrian van; Oberlin, Odile

doi:10.1200/jco.2005.08.130

Cited by 300 publications

(262 citation statements)

References 29 publications

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“…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%

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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: 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%

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

et al. 2018

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“…Good local control has been reported historically with radiotherapy for clinical group III patients also, with a local failure rate of only 13% at 5 years on IRS-IV [15]. Some cooperative groups have attempted to minimize the impact of local therapy by withholding radiotherapy in clinical group III patients who had favorable response to chemotherapy or patients whose disease is resected after induction chemotherapy, but this approach is associated with unfavorable local control and decreased survival [16,17].…”

Section: Evaluation and Managementmentioning

confidence: 99%

Pediatric Soft Tissue Sarcomas

Loeb

Thornton

Shokek

2008

Surgical Clinics of North America

106

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Soft tissue sarcomas in children are relatively rare. Approximately 850 to 900 children and adolescents are diagnosed each year with rhabdomyosarcoma (RMS) or one of the non-RMS soft tissue sarcomas (NRSTS). Of these, 350 are cases of RMS. RMS is the most common soft tissue sarcoma in children 14 years old and younger, and NRSTS is more common in adolescents and young adults. Infants also get NRSTS, but their tumors constitute a distinctive set of histologies, including infantile fibrosarcoma and malignant hemangiopericytoma, not seen in adolescents. Surgery is a major therapeutic modality for all pediatric soft tissue sarcomas, and radiation can play a role in the local therapy for these tumors. RMS is always treated with adjuvant chemotherapy, whereas chemotherapy is reserved for the subset of NRSTS that are high grade or unresectable. This review discusses the etiology, biology, and treatment of pediatric soft tissue sarcomas, including new approaches to therapy aimed at improving the dismal prognosis of patients who have recurrent and metastatic disease. Rhabdomyosarcoma EpidemiologyRMS is the most common soft tissue sarcoma among children less than 15 years old, with an incidence of 4.6 per million per year [1]. This represents 50% of all soft tissue sarcomas in this age range. It is slightly more common in boys than in girls, with a ratio of 1. slightly more common in white children than in black children less than 5 years old (1.1:1) but is more common in black children than in white children 5 years of age or older (1.2:1). Over the past 30 years, the incidence of RMS in the pediatric age group has been constant [1]. EtiologyLittle is known about the etiology of RMS. A few cases are associated with Li-Fraumeni syndrome (caused by germline mutations in p53) [2] or with neurofibromatosis (caused by mutations in NF1) [3]. There also is a weak association with congenital anomalies, especially in boys [4]. These tumors sometimes are seen as second malignant neoplasms after radiation therapy. Molecular and cellular biologyThere are two major histologic variants of RMS-embryonal and alveolar. Other, minor, histologic types include spindle cell, botryoid, and pleomorphic. Embryonal RMS is named for its resemblance to immature skeletal muscle, accounts for 60% of RMS cases in patients less than 20 years of age, and tends to arise in the head and neck region, orbits, and genitourinary region (including bladder and prostate). Alveolar RMS, named for its resemblance to normal lung parenchyma, arises predominantly in the head and neck region and the extremities [3]. Histologically, RMS is a small round blue cell tumor, characterized by expression of muscle-specific antigens, such as desmin and MyoD, and by the presence of eosinophilic rhabdomyoblasts on standard pathologic staining.Alveolar RMS is characterized by the presence of one of two recurrent chromosomal translocations: t(2;13)(q35;q14), seen in 55% of cases, or t(1;13)(p36;q14), seen in 22% of cases [5]. These fuse the FKHR gene on chromosome 13 with...

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“…74,75 The chemotherapy backbone utilized in North America is vincristine, actinomycin, and cyclophosphamide, whereas the European studies have substituted ifosfamide for cyclophosphamide in their studies. [75][76][77] Approximately 90% of patients with favorable histology, completely resected (or only microscopic residual disease) tumors in favorable sites (eg, the female genital tract, paratesticular area, nonparameningeal head and neck region), or nonresected orbital tumors (all low-risk disease) can be cured with minimal therapy consisting of vincristine and actinomycin, with or without lower doses of cyclophosphamide, and radiation in the case of residual disease. more intense, and North American studies are exploring the role of topoisomerase inhibitors.…”

Section: 73mentioning

confidence: 99%

Common Musculoskeletal Tumors of Childhood and Adolescence

Arndt¹,

Rose²,

Folpe³

et al. 2012

SciVee

129

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Osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma are the most common malignant musculoskeletal tumors in children and adolescents. Today, most patients can be cured. Numerous factors have contributed to improved outcome for these patients over the past several decades. These include multidisciplinary care involving oncologists, radiation oncologists, surgeons, pathologists, and radiologists and enrollment of patients in clinical trials. Better understanding of molecular mechanisms of disease have resulted in studies using molecular targets in addition to standard chemotherapeutic agents, which hopefully will lead to better outcomes in the future. Moreover, new orthopedic techniques and devices as well as new technologies in radiation oncology hold promise for better local control of primary tumors and the potential for fewer late adverse effects. Despite this progress, patients must undergo lifelong follow-up for possible late effects of intense chemotherapy and radiation therapy. We review the diagnosis, prognosis, staging, multidisciplinary therapy, new directions in therapy, and long-term complications of treatment for these tumors. For this review, we searched MEDLINE using the terms rhabdomyosarcoma, osteosarcoma, Ewing sarcoma, biology, and humans and limited the search to articles from 2000 to September 2011. Additional references found in these articles were utilized as appropriate, as well as references from the background information in current therapeutic studies of the Children's Oncology Group. The same database and time frame were searched for articles written by leading authorities in the field.

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Treatment of Nonmetastatic Rhabdomyosarcoma in Childhood and Adolescence: Third Study of the International Society of Paediatric Oncology—SIOP Malignant Mesenchymal Tumor 89

Cited by 300 publications

References 29 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

Pediatric Soft Tissue Sarcomas

Common Musculoskeletal Tumors of Childhood and Adolescence

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