Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: A preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991-1997)

Neville, Holly L.; Andrassy, Richard J.; Lobe, Thom E.; Bagwell, Charles E.; Anderson, James R.; Womer, Richard B.; Crist, William M.; Wiener, Eugene S.

doi:10.1016/s0022-3468(00)90031-9

Cited by 110 publications

(99 citation statements)

References 14 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%

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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%

“…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%

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

et al. 2018

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“…All these clinical variables are known to have a negative influence on the final outcome. Various previous reports have confirmed that the results of treatment for extremity RMS are suboptimal, to say the least, by comparison with RMS cases at other sites [13][14][15][16][17]. This may be for various reasons, particularly the strict inter-relationships between the site variable and other prognostic factors (histology, tumor size, node involvement, and patient's age).…”

Section: Discussionmentioning

confidence: 90%

“…For RMS cases in particular, North-American IRS studies have reported that around 40-50% of patients whose lymph nodes were assessed surgically (by biopsy or dissection) revealed nodal involvement [15][16][17]: the IRS IV study, for instance, showed that 17% of patients with clinically negative nodes had microscopic evidence of nodal spread [16]). Moreover, the final outcome was reportedly better in patients with histologically confirmed negative nodes (pN0) than in those whose nodes were judged to be negative at clinical or radiological evaluation alone (cN0) [15].…”

Section: Discussionmentioning

confidence: 99%

Soft-tissue sarcomas of the extremities in patients of pediatric age

Casanova

Meazza

Gronchi

et al. 2007

Journal of Children's Orthopaedics

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Purpose The extremity site is a peculiar location for softtissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006. Methods The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy. Results For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors. Discussion While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.

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“…In addition to staging, a clinical group is assigned to each patient (Table 2), based on the extent of initial resection, margin status, lymph node involvement, and distant spread. Intergroup Rhabdomyosarcoma Study Group (IRSG) studies have demonstrated that clinical group is one of the most important predictors of treatment failure [10], further emphasizing the important role the initial surgery plays in overall patient outcome. After assignment of a stage and clinical group, this information is combined to categorize patients as low, intermediate, or high risk (Table 3), which determines the specific treatment course.…”

Section: Evaluation and Managementmentioning

confidence: 99%

Pediatric Soft Tissue Sarcomas

Loeb

Thornton

Shokek

2008

Surgical Clinics of North America

108

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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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Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: A preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991-1997)

Cited by 110 publications

References 14 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

Soft-tissue sarcomas of the extremities in patients of pediatric age

Pediatric Soft Tissue Sarcomas

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