Zusammenfassung
Weichgewebesarkome sind seltene, heterogene Tumore, welche häufig an den Extremitäten lokalisiert sind. Die Therapie beinhaltet die chirurgische Resektion, Kombinationschemotherapie und/oder Strahlentherapie sowie ergänzende Verfahren wie isolierte Extremitätenperfusion und regionale Tiefenhyperthermie. Die Prognose ist abhängig vom Tumorstadium und den etwa 70 histologischen Subtypen, wobei nur für einige Subtypen spezifische Therapieansätze existieren. Diese Übersichtsarbeit fasst die Empfehlungen der deutschen S3-Leitlinie „Adulte Weichgewebesarkome“ und der Leitlinie der European Society for Medical Oncology (ESMO) „Soft tissue and visceral sarcomas“ zur Diagnostik und Therapie lokalisierter Weichgewebesarkome der Extremitäten zusammen.
Abstract
Soft tissue sarcomas are rare, heterogeneous tumors that are frequently in the extremities. Treatment includes surgical resection, combination chemotherapy and/or radiotherapy, as well as supplementary procedures such as isolated limb perfusion and regional deep hyperthermia. The prognosis depends on the tumor stage and the approximately 70 histological subtypes, with specific treatment approaches existing only for some subtypes. This review summarizes the recommendations of the German S3 guideline “Adult Soft Tissue Sarcomas” and the European Society for Medical Oncology (ESMO) guideline “Soft tissue and visceral sarcomas” regarding the diagnostic workup and therapy of soft tissue sarcomas of the extremities.
Literatur
Saltus CW, Calingaert B, Candrilli S, Lorenzo M, Yulia D, Otto T et al (2018) Epidemiology of adult soft-tissue sarcomas in Germany. Sarcoma. https://doi.org/10.1155/2018/5671926
Ressing M, Wardelmann E, Hohenberger P, Jakob J, Kasper B, Emrich K et al (2018) Strengthening health data on a rare and heterogeneous disease: sarcoma incidence and histological subtypes in Germany. BMC Public Health. https://doi.org/10.1186/s12889-018-5131-4
Sbaraglia M, Bellan E, Tos APD (2021) The 2020 WHO Classification of Soft Tissue Tumours : news and perspectives, S 70–84
Gatta G, Capocaccia R, Botta L, Mallone S, De Angelis R, Ardanaz E et al (2017) Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study. Lancet Oncol 18(8):1022–1039
Gage MM, Nagarajan N, Ruck JM, Canner JK, Khan S, Giuliano K et al (2019) Sarcomas in the United States : recent trends and a call for improved staging. Oncotarget 10(25):2462–2474
Virtanen A, Pukkala E, Auvinen A (2006) Incidence of bone and soft tissue sarcoma after radiotherapy: A cohort study of 295,712 Finnish cancer patients. Int J Cancer 118(4):1017–1021. https://doi.org/10.1002/ijc.21456
Menu-Branthomme A, Rubino C, Shamsaldin A, Hawkins MM, Grimaud E, Dondon M‑G et al (2004) Radiation dose, chemotherapy and risk of soft tissue sarcoma after solid tumours during childhood. Int J Cancer 110(1):87–93. https://doi.org/10.1002/ijc.20002
Gronchi A, Miah AB, Dei Tos AP, Abecassis N, Bajpai J, Bauer S et al (2021) Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 32(11):1348–1365. https://doi.org/10.1016/j.annonc.2021.07.006
Blay J‑Y, Soibinet P, Penel N, Bompas E, Duffaud F, Stoeckle E et al (2017) Improved survival using specialized multidisciplinary board in sarcoma patients. Ann Oncol 28(11):2852–2859 (https://www.sciencedirect.com/science/article/pii/S0923753419346046)
Lazarides AL, Kerr DL, Nussbaum DP, Kreulen RT, Somarelli JA, Blazer DGIII et al (2019) Soft tissue sarcoma of the extremities: what is the value of treating at high-volume centers? Clin Orthop Relat Res 477(4):718
Birgin E, Yang C, Hetjens S, Reissfelder C, Hohenberger P, Rahbari NN (2020) Core needle biopsy versus incisional biopsy for differentiation of soft-tissue sarcomas: a systematic review and meta-analysis. Cancer 126(9):1917–1928
Wu C, Gong S, Osterhoff G, Schopow N (2021) A novel four-gene prognostic signature for prediction of survival in patients with soft tissue sarcoma. Cancers. https://doi.org/10.3390/cancers13225837
Ray-Coquard I, Montesco MC, Coindre JM, Dei Tos AP, Lurkin A, Ranchère-Vince D et al (2012) Sarcoma: concordance between initial diagnosis and centralized expert review in a population-based study within three European regions. Ann Oncol 23(9):2442–2449
Trovik CS, Bauer HCF, Alvegård TA, Anderson H, Blomqvist C, Berlin Ö et al (2000) Surgical margins, local recurrence and metastasis in soft tissue sarcomas: 559 surgically-treated patients from the Scandinavian Sarcoma Group Register. Eur J Cancer 36(6):710–716. https://doi.org/10.1016/S0959-8049(99)00287-7
Gingrich AA, Bateni SB, Monjazeb AM, Darrow MA, Thorpe SW, Kirane AR et al (2017) Neoadjuvant radiotherapy is associated with R0 resection and improved survival for patients with extremity soft tissue sarcoma undergoing surgery: a national cancer database analysis. Ann Surg Oncol 24(11):3252–3263. https://doi.org/10.1245/s10434-017-6019-8
Willeumier JJ, Rueten-Budde AJ, Jeys LM, Laitinen M, Pollock R, Aston W et al (2017) Individualised risk assessment for local recurrence and distant metastases in a retrospective transatlantic cohort of 687 patients with high-grade soft tissue sarcomas of the extremities: a multistate model. BMJ Open 7(2):e12930
Fujiwara T, Kaneuchi Y, Tsuda Y, Stevenson J, Parry M, Jeys L (2020) Low-grade soft-tissue sarcomas: What is an adequate margin for local disease control? Surg Oncol 35:303–308
Fujiwara T, Sumathi V, Parry M, Stevenson J, Tsuda Y, Kaneuchi Y et al (2021) The role of surgical margin quality in myxofibrosarcoma and undifferentiated pleomorphic sarcoma. Eur J Surg Oncol 47(7):1756–1762. https://doi.org/10.1016/j.ejso.2020.11.144
Wittenberg S, Paraskevaidis M, Jarosch A, Flörcken A, Brandes F, Striefler J et al (2022) Surgical margins in soft tissue sarcoma management and corresponding local and systemic recurrence rates: a retrospective study covering 11 years and 169 patients in a single institution. Life. https://doi.org/10.3390/life12111694
Lin PP, Pino ED, Normand AN, Deavers MT, Cannon CP, Ballo MT et al (2007) Periosteal margin in soft-tissue sarcoma. Cancer 109(3):598–602. https://doi.org/10.1002/cncr.22429
Wortmann M, Alldinger I, Böckler D, Ulrich A, Hyhlik-Dürr A (2017) Vascular reconstruction after retroperitoneal and lower extremity sarcoma resection. Eur J Surg Oncol 43(2):407–415. https://doi.org/10.1016/j.ejso.2016.10.029
Sweiti H, Tamimi N, Bormann F, Divo M, Schulz-Ertner D, Ahrens M et al (2018) Limb-salvage surgery of soft tissue sarcoma with sciatic nerve involvement. Sarcoma 2018:6483579. https://doi.org/10.1155/2018/6483579
Biau DJ, Ferguson PC, Chung P, Griffin AM, Catton CN, O’Sullivan B et al (2012) Local recurrence of localized soft tissue sarcoma. Cancer 118(23):5867–5877. https://doi.org/10.1002/cncr.27639
Chandrasekar CR, Wafa H, Grimer RJ, Carter SR, Tillman RM, Abudu A (2008) The effect of an unplanned excision of a soft-tissue sarcoma on prognosis. J Bone Joint Surg Br 90(2):203–208
Venkatesan M, Richards CJ, McCulloch TA, Perks AGB, Raurell A, Ashford RU (2012) Inadvertent surgical resection of soft tissue sarcomas. Eur J Surg Oncol 38(4):346–351
Grignol VP, Lopez-Aguiar AG (2022) The implications of an unplanned sarcoma excision (the “whoops” operation). Surg Clin North Am 102(4):529–538
Bianchi G, Sambri A, Cammelli S, Galuppi A, Cortesi A, Righi A et al (2017) Impact of residual disease after “unplanned excision” of primary localized adult soft tissue sarcoma of the extremities: evaluation of 452 cases at a single Institution. Musculoskelet Surg 101(3):243–248. https://doi.org/10.1007/s12306-017-0475-y
Albertsmeier M, Rauch A, Roeder F, Hasenhütl S, Pratschke S, Kirschneck M et al (2018) External beam radiation therapy for Resectable soft tissue sarcoma: a systematic review and meta-analysis. Ann Surg Oncol 25(3):754–767. https://doi.org/10.1245/s10434-017-6081-2
Qu X, Lubitz CC, Rickard J, Bergeron SG, Wasif N (2018) A meta-analysis of the association between radiation therapy and survival for surgically resected soft-tissue sarcoma. Am J Clin Oncol 41(4):348
Davis AM, O’Sullivan B, Turcotte R, Bell R, Catton C, Chabot P et al (2005) Late radiation morbidity following randomization to preoperative versus postoperative radiotherapy in extremity soft tissue sarcoma. Radiother Oncol 75(1):48–53
Judson I, Verweij J, Gelderblom H, Hartmann JT, Schöffski P, Blay JY et al (2014) Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial. Lancet Oncol 15(4):415–423
Pervaiz N, Colterjohn N, Farrokhayr F (2008) A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer 113:573–581
Gronchi A, Ferrari S, Quagliuolo V, Broto JM, Pousa AL, Grignani G et al (2017) Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial. Lancet Oncol 18(6):812–822
Pasquali S, Pizzamiglio S, Touati N, Litiere S, Marreaud S, Kasper B et al (2019) The impact of chemotherapy on survival of patients with extremity and trunk wall soft tissue sarcoma: revisiting the results of the EORTC-STBSG 62931 randomised trial. Eur J Cancer 109:51–60
Pasquali S, Palmerini E, Quagliuolo V, Martin-Broto J, Lopez-Pousa A, Grignani G et al (2022) Neoadjuvant chemotherapy in high-risk soft tissue sarcomas: a Sarculator-based risk stratification analysis of the ISG-STS 1001 randomized trial. Cancer 128(1):85–93. https://doi.org/10.1002/cncr.33895
Issels RD, Lindner LH, Verweij J, Wessalowski R, Reichardt P, Wust P et al (2018) Effect of neoadjuvant chemotherapy plus regional hyperthermia on long-term outcomes among patients with localized high-risk soft tissue sarcoma: the EORTC 62961-ESHO 95 randomized clinical trial. JAMA Oncol 4(4):483–492. https://doi.org/10.1001/jamaoncol.2017.4996
Eggermont AMM, Koops HS, Klausner JM, Kroon BBR, Schlag PM, Liénard D et al (1996) Isolated limb perfusion with tumor necrosis factor and melphalan for limb salvage in 186 patients with locally advanced soft tissue extremity sarcomas: the cumulative multicenter European experience. Ann Surg 224(6):756 (discussion 764-5)
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N. Schopow, P. Hohenberger, I. Gockel und G. Osterhoff geben an, dass kein Interessenkonflikt besteht.
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Schopow, N., Hohenberger, P., Gockel, I. et al. Multimodale Therapie der lokalisierten High-grade-Weichgewebesarkome der Extremitäten. Chirurgie 94, 424–431 (2023). https://doi.org/10.1007/s00104-023-01872-3
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DOI: https://doi.org/10.1007/s00104-023-01872-3