Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology

Mehren, Margaret von; Randall, R. Lor; Benjamin, Robert S.; Boles, Sarah G; Bui, Marilyn M.; Ganjoo, Kristen N.; George, Suzanne; Gonzalez, Ricardo J.; Heslin, Martin J.; Kane, John M.; Keedy, Vicki L.; Kim, Edward; Koon, Henry; Mayerson, Joel L.; McCarter, Martin; McGarry, Sean V.; Meyer, Christian; Morris, Zachary S.; O’Donnell, Richard J.; Pappo, Alberto S.; Paz, I. Benjamin; Petersen, Ivy A.; Pfeifer, John D.; Riedel, Richard F.; Ruo, Bernice; Schuetze, Scott M.; Tap, William D.; Wayne, Jeffrey D.; Bergman, Mary Anne; Scavone, Jillian L.

doi:10.6004/jnccn.2018.0025

Cited by 566 publications

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“…4 SDH deficiency occurs in 7–8% of gastric GISTs, 5 and the vast majority of gastric WT GISTs are reported to be SDH-deficient. 6 SDH-deficient gastric GISTs commonly occur in young women, frequently metastasize to lymph nodes and distant sites, usually glow slowly, and are commonly resistant to tyrosine kinase inhibitors. 3 Because of the rarity of WT GISTs, the true incidence of SDH germline mutations in patients who have WT GISTs and who do not have a family or personal history of SDH-associated tumors is unknown.…”

Section: In Replymentioning

confidence: 99%

“…4 However, on the basis of available evidence, the National Comprehensive Cancer Network (NCCN) recommends testing gastric WT GISTs for SDHB expression by immunohistochemistry and for germline mutations in the SDH genes. 6 Currently, there are no specific treatment recommendations for patients with SDH-deficient GISTs, as tyrosine kinase inhibitors are ineffective against such tumors, and no effective systemic therapy alternative yet exists. Identifying a germline mutation could alert treating physicians to a presumed increased risk of GIST recurrence and paraganglioma.…”

Section: In Replymentioning

confidence: 99%

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The Hereditary Paraganglioma-Pheochromocytoma Syndrome: No Time to Waste—Reply

2017

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sunitinib patients] were still alive at … interim analysis." 5 Fojo et al 1 indicate no reported OS benefits. Using what it considers to be an "appropriate" rank-preserving structural failure time model to account for crossover, NICE finds a significant OS gain of 7.8 months; significance was not affected by model revisions that were based on recommendations from an independent statistician. 4 Suggestions of zero benefit misinterpret the RCT and HTA evaluations.Yet, Prasad argues against the use of modeling because "different models yield different estimates." This is a valid point, but also precisely why HTA agencies rely on expert statistical evaluations of trial evidence, and why we prefer to review their appraisals. Studies should evaluate whether acceptance of modeled data by HTA agencies incentivizes the avoidance of trials that measure OS. However, modeling today helps authorities handle issues with RCTs, and the use of surrogate efficacy end points. Our article also questions how HTA agencies evaluate clinical benefits, and we agree that further work is needed to assess the external validity of modeled OS benefits. However, if only for the reasons described herein, that hardly justifies dismissing them.

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Section: In Replymentioning

confidence: 99%

Section: In Replymentioning

confidence: 99%

The Hereditary Paraganglioma-Pheochromocytoma Syndrome: No Time to Waste—Reply

2017

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“…Sarcomas, consisted of soft tissue sarcomas (STSs) and bone sarcomas, are a heterogeneous group of mesenchymal malignancies that can develop at any age, comprising approximately 1% of all adult malignancies and 15% of pediatric malignancies 1 . Compared with other common cancer types, STSs can develop at almost any anatomical sites 2 , and are more prone to occur at childhood 3 .…”

Section: Introductionmentioning

confidence: 99%

Incidence, distribution of histological subtypes and primary sites of soft tissue sarcoma in China

et al. 2019

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Objective: Soft tissue sarcomas (STSs) are rare malignancies deriving from mesenchyme. In this study, we reported the epidemiology of STS in China using population-based cancer registry data. Methods: In 2017, qualified data from 339 cancer registries were included in the national database. All STS cases were retrieved based on the morphological and topographical codes of International Classification of Diseases for Oncology, and were categorized into different histological subtypes and primary sites accordingly. Nationwide new STS cases were estimated using incidence rate of STS and the national population, and were reported for gastrointestinal stromal tumor (GIST) and STSs other than GIST separately by sex and region. Distribution of histological subtypes and primary sites of STS were calculated, as well as primary sites of GIST. Results: Approximately 39,900 new STS cases occurred nationwide in China in 2014, accounting for 1.05% of overall cancer incidence. The crude incidence rate was 2.91/100,000 and generally increased with age. An overall female predilection was found. GIST was the most common histological subtype, followed by nerve sheath tumor and malignant peripheral nerve sheath tumor, leiomyosarcoma, liposarcoma, and fibrosarcoma. About 67.5% of GIST occurred in stomach while 1.4% were recorded outside the gastrointestinal tract. Connective, subcutaneous and other soft tissues were the most common primary site, of which extremities were the major subsite. Conclusions: The burden of STS is not serious in China relatively. However, due to their histological and topographical complexity, STSs should not be unnoticed, and more basic and clinical studies should focus on STSs.

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“…There are three major histologic subtypes of rhabdomyosarcoma (RMS), embryonal, alveolar, and pleomorphic, and the first two are also called nonpleomorphic RMS 1,2. The head and neck region is the most affected site, followed closely by the genitourinary tract, in which ~25% of cases occur 3.…”

Section: Introductionmentioning

confidence: 99%

Successful treatment of relapsed testicular embryonal rhabdomyosarcoma with Endostar and traditional chemotherapy: a case report

Han

Chen

Luan

et al. 2018

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Embryonal rhabdomyosarcoma (ERMS) has a low prevalence, poor prognosis, and limited treatment efficacy. We report a case of an 18-year-old male whose disease relapsed in the abdominal cavity after a testicular ERMS curative resection. The patient received eight sequential cycles of rescue therapy using cisplatin and isocyclophosphamide in combination with a vascular targeted drug, Endostar. The therapeutic effect of the combination regimen has been evaluated for complete response. This is the first case to report using Endostar and chemotherapy in relapsed ERMS, and the curative effect results in complete response. Endostar, a new vascular targeted drug, combined with chemotherapy may play a synergistic role and provide a reference for the treatment of ERMS.

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Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology

Cited by 566 publications

References 0 publications

The Hereditary Paraganglioma-Pheochromocytoma Syndrome: No Time to Waste—Reply

The Hereditary Paraganglioma-Pheochromocytoma Syndrome: No Time to Waste—Reply

Incidence, distribution of histological subtypes and primary sites of soft tissue sarcoma in China

Successful treatment of relapsed testicular embryonal rhabdomyosarcoma with Endostar and traditional chemotherapy: a case report

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