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Soft Tissue Sarcoma of the Trunk and Extremities

MedGen UID:
1714237
•Concept ID:
C5236972
•
Neoplastic Process

Definition

A soft tissue sarcoma that arises from the trunk or the extremities. [from NCI]

Professional guidelines

PubMed

Management of adult soft-tissue sarcoma of the extremities and trunk.
Abraham JA, Baldini EH, Butrynski JE
Expert Rev Anticancer Ther 2010 Feb;10(2):233-48. doi: 10.1586/era.09.193. PMID: 20131999

Recent clinical studies

Etiology

Management of Synovial Sarcoma and Myxoid Liposarcoma.
Hindi N, Haas RL
Surg Oncol Clin N Am 2022 Jul;31(3):547-558. doi: 10.1016/j.soc.2022.03.012. PMID: 35715149
Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Radiation Therapy for Treatment of Soft Tissue Sarcoma in Adults: Executive Summary of an ASTRO Clinical Practice Guideline.
Salerno KE, Alektiar KM, Baldini EH, Bedi M, Bishop AJ, Bradfield L, Chung P, DeLaney TF, Folpe A, Kane JM, Li XA, Petersen I, Powell J, Stolten M, Thorpe S, Trent JC, Voermans M, Guadagnolo BA
Pract Radiat Oncol 2021 Sep-Oct;11(5):339-351. Epub 2021 Jul 26 doi: 10.1016/j.prro.2021.04.005. PMID: 34326023
Update on pediatric rhabdomyosarcoma: A report from the APSA Cancer Committee.
Rhee DS, Rodeberg DA, Baertschiger RM, Aldrink JH, Lautz TB, Grant C, Meyers RL, Tracy ET, Christison-Lagay ER, Glick RD, Mattei P, Dasgupta R; American Pediatric Surgical Association Cancer Committee
J Pediatr Surg 2020 Oct;55(10):1987-1995. Epub 2020 Jun 18 doi: 10.1016/j.jpedsurg.2020.06.015. PMID: 32674846
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444

Diagnosis

Soft Tissue Sarcoma, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology.
von Mehren M, Kane JM, Agulnik M, Bui MM, Carr-Ascher J, Choy E, Connelly M, Dry S, Ganjoo KN, Gonzalez RJ, Holder A, Homsi J, Keedy V, Kelly CM, Kim E, Liebner D, McCarter M, McGarry SV, Mesko NW, Meyer C, Pappo AS, Parkes AM, Petersen IA, Pollack SM, Poppe M, Riedel RF, Schuetze S, Shabason J, Sicklick JK, Spraker MB, Zimel M, Hang LE, Sundar H, Bergman MA
J Natl Compr Canc Netw 2022 Jul;20(7):815-833. doi: 10.6004/jnccn.2022.0035. PMID: 35830886Free PMC Article
Management of Synovial Sarcoma and Myxoid Liposarcoma.
Hindi N, Haas RL
Surg Oncol Clin N Am 2022 Jul;31(3):547-558. doi: 10.1016/j.soc.2022.03.012. PMID: 35715149
Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Osteosarcoma of the Jaws: A Literature Review.
Ricotta F, Bassi M, Tomasetti N, Campobassi A, Maiolo V, Bertoni F, Bacchini P, Marchetti C, Tarsitano A
Curr Med Imaging 2021;17(2):225-235. doi: 10.2174/1573405616666200806173948. PMID: 32767948
Soft-tissue sarcoma.
Gilbert NF, Cannon CP, Lin PP, Lewis VO
J Am Acad Orthop Surg 2009 Jan;17(1):40-7. doi: 10.5435/00124635-200901000-00006. PMID: 19136426

Therapy

Neoadjuvant chemotherapy in high-risk soft tissue sarcomas: A Sarculator-based risk stratification analysis of the ISG-STS 1001 randomized trial.
Pasquali S, Palmerini E, Quagliuolo V, Martin-Broto J, Lopez-Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz-Beveridge R, Ferraresi V, Lugowska I, Infante G, Braglia L, Merlo DF, Fontana V, Marchesi E, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Miceli R, Casali PG, Gronchi A
Cancer 2022 Jan 1;128(1):85-93. Epub 2021 Oct 13 doi: 10.1002/cncr.33895. PMID: 34643947
Pathological response in children and adults with large unresected intermediate-grade or high-grade soft tissue sarcoma receiving preoperative chemoradiotherapy with or without pazopanib (ARST1321): a multicentre, randomised, open-label, phase 2 trial.
Weiss AR, Chen YL, Scharschmidt TJ, Chi YY, Tian J, Black JO, Davis JL, Fanburg-Smith JC, Zambrano E, Anderson J, Arens R, Binitie O, Choy E, Davis JW, Hayes-Jordan A, Kao SC, Kayton ML, Kessel S, Lim R, Meyer WH, Million L, Okuno SH, Ostrenga A, Parisi MT, Pryma DA, Randall RL, Rosen MA, Schlapkohl M, Shulkin BL, Smith EA, Sorger JI, Terezakis S, Hawkins DS, Spunt SL, Wang D
Lancet Oncol 2020 Aug;21(8):1110-1122. Epub 2020 Jul 20 doi: 10.1016/S1470-2045(20)30325-9. PMID: 32702309Free PMC Article
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
Phase II study of neoadjuvant checkpoint blockade in patients with surgically resectable undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma.
Keung EZ, Lazar AJ, Torres KE, Wang WL, Cormier JN, Ashleigh Guadagnolo B, Bishop AJ, Lin H, Hunt KK, Bird J, Lewis VO, Patel SR, Wargo JA, Somaiah N, Roland CL
BMC Cancer 2018 Sep 24;18(1):913. doi: 10.1186/s12885-018-4829-0. PMID: 30249211Free PMC Article
Rhabdomyosarcoma.
Hays DM
Clin Orthop Relat Res 1993 Apr;(289):36-49. PMID: 8472431

Prognosis

Neoadjuvant chemotherapy in high-risk soft tissue sarcomas: A Sarculator-based risk stratification analysis of the ISG-STS 1001 randomized trial.
Pasquali S, Palmerini E, Quagliuolo V, Martin-Broto J, Lopez-Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz-Beveridge R, Ferraresi V, Lugowska I, Infante G, Braglia L, Merlo DF, Fontana V, Marchesi E, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Miceli R, Casali PG, Gronchi A
Cancer 2022 Jan 1;128(1):85-93. Epub 2021 Oct 13 doi: 10.1002/cncr.33895. PMID: 34643947
Update on pediatric rhabdomyosarcoma: A report from the APSA Cancer Committee.
Rhee DS, Rodeberg DA, Baertschiger RM, Aldrink JH, Lautz TB, Grant C, Meyers RL, Tracy ET, Christison-Lagay ER, Glick RD, Mattei P, Dasgupta R; American Pediatric Surgical Association Cancer Committee
J Pediatr Surg 2020 Oct;55(10):1987-1995. Epub 2020 Jun 18 doi: 10.1016/j.jpedsurg.2020.06.015. PMID: 32674846
Soft-tissue sarcoma in adults: An update on the current state of histiotype-specific management in an era of personalized medicine.
Gamboa AC, Gronchi A, Cardona K
CA Cancer J Clin 2020 May;70(3):200-229. Epub 2020 Apr 10 doi: 10.3322/caac.21605. PMID: 32275330
Soft-tissue sarcoma.
Gilbert NF, Cannon CP, Lin PP, Lewis VO
J Am Acad Orthop Surg 2009 Jan;17(1):40-7. doi: 10.5435/00124635-200901000-00006. PMID: 19136426
Rhabdomyosarcoma.
Hays DM
Clin Orthop Relat Res 1993 Apr;(289):36-49. PMID: 8472431

Clinical prediction guides

Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Neoadjuvant chemotherapy in high-risk soft tissue sarcomas: A Sarculator-based risk stratification analysis of the ISG-STS 1001 randomized trial.
Pasquali S, Palmerini E, Quagliuolo V, Martin-Broto J, Lopez-Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz-Beveridge R, Ferraresi V, Lugowska I, Infante G, Braglia L, Merlo DF, Fontana V, Marchesi E, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Miceli R, Casali PG, Gronchi A
Cancer 2022 Jan 1;128(1):85-93. Epub 2021 Oct 13 doi: 10.1002/cncr.33895. PMID: 34643947
Pathological response in children and adults with large unresected intermediate-grade or high-grade soft tissue sarcoma receiving preoperative chemoradiotherapy with or without pazopanib (ARST1321): a multicentre, randomised, open-label, phase 2 trial.
Weiss AR, Chen YL, Scharschmidt TJ, Chi YY, Tian J, Black JO, Davis JL, Fanburg-Smith JC, Zambrano E, Anderson J, Arens R, Binitie O, Choy E, Davis JW, Hayes-Jordan A, Kao SC, Kayton ML, Kessel S, Lim R, Meyer WH, Million L, Okuno SH, Ostrenga A, Parisi MT, Pryma DA, Randall RL, Rosen MA, Schlapkohl M, Shulkin BL, Smith EA, Sorger JI, Terezakis S, Hawkins DS, Spunt SL, Wang D
Lancet Oncol 2020 Aug;21(8):1110-1122. Epub 2020 Jul 20 doi: 10.1016/S1470-2045(20)30325-9. PMID: 32702309Free PMC Article
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
Rhabdomyosarcoma.
Hays DM
Clin Orthop Relat Res 1993 Apr;(289):36-49. PMID: 8472431

Recent systematic reviews

Leiomyosarcoma in the extremities and trunk wall: systematic review and meta-analysis of the oncological outcomes.
Kannan S, Chong HH, Chew B, Ferguson JD, Galloway E, McCulloch T, Rankin KS, Ashford RU
World J Surg Oncol 2022 Apr 18;20(1):124. doi: 10.1186/s12957-022-02584-4. PMID: 35436892Free PMC Article
Marginal resection for patients with atypical lipomatous tumours of the extremities and trunk wall: a systematic review and meta-analysis.
Kido A, Kitagawa Y, Tsukushi S, Iwata S, Ishida Y, Tsukamoto S, Kawai A
Jpn J Clin Oncol 2022 Feb 5;52(2):151-156. doi: 10.1093/jjco/hyab185. PMID: 34865069
A Meta-Analysis of the Association Between Radiation Therapy and Survival for Surgically Resected Soft-Tissue Sarcoma.
Qu X, Lubitz CC, Rickard J, Bergeron SG, Wasif N
Am J Clin Oncol 2018 Apr;41(4):348-356. doi: 10.1097/COC.0000000000000274. PMID: 26886948
Liposarcoma of Hypopharynx and Esophagus: a Unique Entity?
Riva G, Sensini M, Corvino A, Garzaro M, Pecorari G
J Gastrointest Cancer 2016 Jun;47(2):135-42. doi: 10.1007/s12029-016-9808-6. PMID: 26875081
A systematic overview of radiation therapy effects in soft tissue sarcomas.
Strander H, Turesson I, Cavallin-Ståhl E
Acta Oncol 2003;42(5-6):516-31. doi: 10.1080/02841860310014732. PMID: 14596510

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