Non-Rhabdomyosarcoma Soft Tissue Sarcomas in Children: A Surveillance, Epidemiology, and End Results Analysis Validating COG Risk Stratifications

Waxweiler, Timothy V.; Rusthoven, Chad G.; Proper, Michelle; Cost, Carrye R.; Cost, Nicholas G.; Donaldson, Nathan; Garrington, Timothy P.; Greffe, Brian; Heare, Travis; Macy, Margaret E.; Liu, Arthur K.

doi:10.1016/j.ijrobp.2015.02.007

Cited by 27 publications

(26 citation statements)

References 18 publications

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“…First, we replicated results from prior studies that whites have a significant survival advantage over blacks and Hispanics for several childhood cancers including leukemias, 2, 3 lymphomas, 4, 5 CNS tumors, 6 neuroblastoma, 7 and NRSTS. 9 In no instance was survival among whites significantly worse than that of either black or Hispanic children. Racial and ethnic survival differences were not uniform across cancers, and some variability between black-white and Hispanic-white comparisons was observed.…”

Section: Discussionmentioning

confidence: 98%

“…1 Compared to non-Hispanic white (white) children, non-Hispanic black (black) and Hispanic children experience lower survival from many cancers including leukemias, 2, 3 lymphomas, 4, 5 central nervous system (CNS) tumors, 6 and extracranial solid tumors. 7–9 The underlying causes of racial/ethnic survival differences are not well understood, and may vary by cancer type. As outlined in Figure 1, both biological and socioeconomic pathways have been proposed in the literature.…”

Section: Introductionmentioning

confidence: 99%

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Does socioeconomic status account for racial and ethnic disparities in childhood cancer survival?

Kehm

Spector

Poynter

et al. 2018

Cancer

123

139

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Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

Does socioeconomic status account for racial and ethnic disparities in childhood cancer survival?

Kehm

Spector

Poynter

et al. 2018

Cancer

123

139

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“…Of these, 40% of cases are rhabdomyosarcomas, and the other 60% are non‐rhabdomyosarcoma soft‐tissue sarcomas (NRSTS). NRSTS consists of over 40 histologic subtypes, with the most common subtypes in the pediatric population including synovial sarcoma, malignant peripheral nerve sheath tumor, and undifferentiated pleomorphic sarcoma (previously malignant fibrous histiocytoma) 1‐11 …”

Section: Disease Overviewmentioning

confidence: 99%

Non‐rhabdomyosarcoma soft‐tissue sarcoma

Milgrom

Million

Mandeville

et al. 2021

Pediatric Blood & Cancer

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Non‐rhabdomyosarcoma soft‐tissue sarcomas (NRSTS) comprise 4% of childhood cancers and consist of numerous histologic subtypes. Prognostic factors associated with poor outcome include high histologic grade, large tumor size, presence of metastases, and unresectability. Complete surgical resection is critical for the best oncologic outcomes and is prioritized in treatment algorithms. The use of radiation therapy (RT) and chemotherapy is based upon factors such as resectability, histologic grade, tumor size, and stage. North American and European trials are defining a risk‐based approach to NRSTS to limit treatment‐related toxicity and to maximize therapeutic efficacy. In this paper, we summarize the current roles of surgery, RT, and chemotherapy in NRSTS and describe ongoing research that is advancing the care of NRSTS patients.

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“…Despite the curative intent of treatment in localized MPNST, local recurrences and distant metastases are very common and survival remains poor . Overall survival in MPNSTs is also poorer compared with other NRSTS . Additionally, factors influencing survival are not evident yet in pediatric MPNSTs.…”

Section: Introductionmentioning

confidence: 99%

“…2,3,11 Overall survival in MPN-STs is also poorer compared with other NRSTS. 19 Additionally, factors influencing survival are not evident yet in pediatric MPNSTs. Recently, the influence of NF1 on survival has been subject of debate as studies report conflicting results.…”

Section: Introductionmentioning

confidence: 99%

Neurofibromatosis‐associated malignant peripheral nerve sheath tumors in children have a worse prognosis: A nationwide cohort study

Martin

Coert

Flucke

et al. 2019

Pediatric Blood & Cancer

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Background Malignant peripheral nerve sheath tumors (MPNST) are rare and aggressive non‐rhabdomyoblastic soft‐tissue sarcomas (NRSTS) in children. This study set out to investigate clinical presentation, treatment modalities, and factors associated with survival in pediatric MPNST using Dutch nationwide databases. Methods Data were obtained from the Netherlands Cancer Registry (NCR) and the Dutch Pathology Database (PALGA) from 1989 to 2017. All primary MPNSTs were collected. Demographic differences were analyzed between adult and pediatric (age ≤18 years) MPNST. In children, demographic and treatment differences between neurofibromatosis type 1 (NF1) and non‐NF1 were analyzed. A Cox proportional hazard model was constructed for localized pediatric MPNSTs. Results A total of 70/784 MPNST patients were children (37.1% NF1). Children did not present differently from adults. In NF1 children, tumor size was more commonly large (> 5 cm, 92.3% vs 59.1%). Localized disease was primarily resected in 90.6%, and radiotherapy was administered in 37.5%. Non‐NF1 children tended to receive chemotherapy more commonly (39.5% vs 26.9%). Overall, estimated five‐year survival rates of localized NF1‐MPNST was 52.4% (SE: 10.1%) compared with 75.8% (SE: 7.1%) in non‐NF1 patients. The multivariate model showed worse survival in NF1 patients (HR: 2.98; 95% CI, 1.17‐7.60, P = 0.02) and increased survival in patients diagnosed after 2005 (HR: 0.20; 95% CI, 0.06‐0.69, P = 0.01). No treatment factors were independently associated with survival. Conclusion Pediatric MPNSTs have presentations similar to adult MPNSTs. In children, NF1 patients present with larger tumors, but are treated similarly to non‐NF1 MPNSTs. In localized pediatric MPNST, NF1 is associated with worse survival. Promisingly, survival has increased for pediatric MPNSTs after 2005.

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Non-Rhabdomyosarcoma Soft Tissue Sarcomas in Children: A Surveillance, Epidemiology, and End Results Analysis Validating COG Risk Stratifications

Cited by 27 publications

References 18 publications

Does socioeconomic status account for racial and ethnic disparities in childhood cancer survival?

Does socioeconomic status account for racial and ethnic disparities in childhood cancer survival?

Non‐rhabdomyosarcoma soft‐tissue sarcoma

Neurofibromatosis‐associated malignant peripheral nerve sheath tumors in children have a worse prognosis: A nationwide cohort study

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