Extremity sarcomas: An analysis of prognostic factors from the intergroup rhabdomyosarcoma study III

Andrassy, Richard J.; Corpron, Cynthia A.; Hays, Daniel M.; Raney, R. Beverly; Wiener, Eugene S.; Lawrence, Walter; Lobe, Thom E.; Bagwell, Charles E.; Maurer, Harold M.

doi:10.1016/s0022-3468(96)90346-2

Cited by 49 publications

(29 citation statements)

References 14 publications

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“…Chemotherapy is still a matter of debate and must be discussed in a dedicated multidisciplinary committee with an individual approach basis 28. For all adult sarcoma in a localized phase, R0 surgery remains the cornerstone of treatment 29, 30, 31, 32, 33, 34, 35. The present study underlines the heterogeneity of adult RMS management.…”

Section: Discussionmentioning

confidence: 75%

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: 75%

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

et al. 2018

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“…The clinical-and probably biological-peculiarity of soft part tumors arising in the limbs is especially evident for RMS, where the extremities are involved in around 15-20% of cases [13][14][15][16][17]. Table 3 compares ours with a large series of RMS of all sites, showing that RMS of the extremities are more likely to occur in adolescents, to be of alveolar subtype, to be large in size and locally invasive, and to spread to the lymph nodes.…”

Section: Discussionmentioning

confidence: 76%

“…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: 89%

“…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]. Unfortunately, our series adds no significant information on this issue: in the past, regional lymph nodes were evaluated by physical and/or radiological examination, and few children were biopsied in the event of clinically negative nodes (and none of those who were biopsied revealed microscopic tumor spread).…”

Section: Discussionmentioning

confidence: 99%

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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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“…The knowledge of the LN status is crucial for RMS of extremities. All the ongoing protocols on RMS recommend their histological evaluation, since a high incidence of LN metastases has been described in many series [2,3]. About 50% of the LN biopsied were involved with tumor on extremity RMS from the Intergroup RMS Study IV [4].…”

Section: Introductionmentioning

confidence: 99%

Sentinel node biopsy in pediatric soft tissue sarcomas of extremities

Corti

Dall’Igna

Bisogno

et al. 2008

Pediatric Blood & Cancer

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BackgroundSentinel Node Biopsy is an established staging technique in many adult malignancies. However, only few reports describe this procedure for the evaluation of regional lymph nodes in childhood and adolescents. Our experience with sentinel node biopsy in soft tissue sarcomas of extremities in children is reported.MethodsSeventeen children were evaluated with sentinel node biopsy between 2002 and 2007: 11 at initial surgery, 5 at primary re‐excision, 1 at local relapse. The diagnosis was rhabdomyosarcoma in 5 and other soft tissue sarcomas in 12: Ewing/PNET sarcoma 6, epithelioid sarcoma 1, malignant peripheral‐nerve‐sheath tumor 1, undifferentiated sarcoma 1, myxoid liposarcoma 2, adult‐type fibrosarcoma 1. Primary sites included lower limbs (8), upper limbs (9). Mapping of nodes was performed with lymphoscintigraphy plus intra‐operative injections with blue‐dye in 14 cases, with lymphoscintigraphy and intra‐operative injections alone in 2 and 1, respectively.ResultsOf the 17 lymphatic regions (9 axilla, 8 inguinal), 16 were identified with lymphoscintigraphy, 15 by intra‐operative injections. Thirty‐five lymph nodes were removed. Nodes were positive for metastasis in two patients with alveolar rhabdomyosarcoma and undifferentiated sarcoma. There were no complications. No further lymph node metastases were recognized either at diagnosis or during the follow‐up (6–78 months).ConclusionsSentinel node biopsy was technically feasible, reliable, and free of complications. It could be an alternative to aggressive or random biopsies for extremity rhabdomyosarcoma and it can contribute to define those non‐rhabdomyosarcoma soft tissue sarcomas that spread to regional nodes. Pediatr Blood Cancer 2009;52:51–54. © 2008 Wiley‐Liss, Inc.

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Extremity sarcomas: An analysis of prognostic factors from the intergroup rhabdomyosarcoma study III

Cited by 49 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

Sentinel node biopsy in pediatric soft tissue sarcomas of extremities

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