Skip to main content
Erschienen in: Die Onkologie 11/2023

19.10.2023 | Weichteilsarkome | CME

Diagnostik und Behandlung von Weichgewebesarkomen

verfasst von: Dr. med. Rainer Hamacher, Moritz Kaths, Wiebke K. Guder

Erschienen in: Die Onkologie | Ausgabe 11/2023

zum CME-Kurs Einloggen, um Zugang zu erhalten

Zusammenfassung

Die Behandlung von Weichgewebesarkomen bedarf einer hohen interdisziplinären Spezialisierung. Gründe sind ihre Seltenheit und die vielen histologischen Subtypen, die sich biologisch unterschiedlich verhalten und an theoretisch jedem anatomischen Ort auftreten können. Studien zeigen eine signifikante Prognoseverbesserung durch prätherapeutische Vorstellung in einer spezialisierten, interdisziplinären Tumorkonferenz und durch Operationen in spezialisierten chirurgischen Zentren. Entscheidungen zur Operationsplanung und perioperativen Strahlentherapie sowie die Einschätzung des Stellenwerts der Chemotherapie werden in Abhängigkeit vom histologischen Subtyp und der Tumorlokalisation getroffen. Auch für die Palliativbehandlung variieren die Behandlungsempfehlungen in Abhängigkeit vom jeweiligen Subtyp. Im vorliegenden Beitrag geben wir eine Übersicht über die diagnostischen Abläufe und Therapiekonzepte bei Weichgewebesarkomen mit Ausnahme der gastrointestinalen Stromatumoren (GIST).
Literatur
1.
Zurück zum Zitat Onkologie L (2021) S3-Leitlinie Adulte Weichgewebesarkome, Langversion, Version 1.0. Deutsche Krebsgesellschaft, Deutsche Krebshilfe, AWMF Onkologie L (2021) S3-Leitlinie Adulte Weichgewebesarkome, Langversion, Version 1.0. Deutsche Krebsgesellschaft, Deutsche Krebshilfe, AWMF
2.
Zurück zum Zitat Gronchi A, Miah AB, Dei Tos AP et al (2021) Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS clinical practice guidelines for diagnosis, treatment and follow-up(☆). Ann Oncol 32:1348–1365CrossRefPubMed Gronchi A, Miah AB, Dei Tos AP et al (2021) Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS clinical practice guidelines for diagnosis, treatment and follow-up(☆). Ann Oncol 32:1348–1365CrossRefPubMed
3.
Zurück zum Zitat Von Mehren M, Kane JM, Agulnik M et al (2022) Soft tissue sarcoma, version 2.2022, NCCN clinical practice guidelines in oncology. J Natl Compr Canc Netw 20:815–833CrossRef Von Mehren M, Kane JM, Agulnik M et al (2022) Soft tissue sarcoma, version 2.2022, NCCN clinical practice guidelines in oncology. J Natl Compr Canc Netw 20:815–833CrossRef
4.
Zurück zum Zitat Fletcher CDM et al (2020) Soft tissue and bone tumours WHO classification of tumours Fletcher CDM et al (2020) Soft tissue and bone tumours WHO classification of tumours
5.
Zurück zum Zitat De Pinieux G, Karanian M, Le Loarer F et al (2021) Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network. PLoS ONE 16:e246958CrossRefPubMedPubMedCentral De Pinieux G, Karanian M, Le Loarer F et al (2021) Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network. PLoS ONE 16:e246958CrossRefPubMedPubMedCentral
6.
Zurück zum Zitat Coindre JM (2006) Grading of soft tissue sarcomas: review and update. Arch Pathol Lab Med 130:1448–1453CrossRefPubMed Coindre JM (2006) Grading of soft tissue sarcomas: review and update. Arch Pathol Lab Med 130:1448–1453CrossRefPubMed
7.
Zurück zum Zitat Stiller CA, Trama A, Serraino D et al (2013) Descriptive epidemiology of sarcomas in Europe: report from the RARECARE project. Eur J Cancer 49:684–695CrossRefPubMed Stiller CA, Trama A, Serraino D et al (2013) Descriptive epidemiology of sarcomas in Europe: report from the RARECARE project. Eur J Cancer 49:684–695CrossRefPubMed
8.
Zurück zum Zitat Blay JY, Soibinet P, Penel N et al (2017) Improved survival using specialized multidisciplinary board in sarcoma patients. Ann Oncol 28:2852–2859CrossRefPubMedPubMedCentral Blay JY, Soibinet P, Penel N et al (2017) Improved survival using specialized multidisciplinary board in sarcoma patients. Ann Oncol 28:2852–2859CrossRefPubMedPubMedCentral
9.
Zurück zum Zitat Nandra R, Forsberg J, Grimer R (2015) If your lump is bigger than a golf ball and growing, think sarcoma. Eur J Surg Oncol 41:1400–1405CrossRefPubMed Nandra R, Forsberg J, Grimer R (2015) If your lump is bigger than a golf ball and growing, think sarcoma. Eur J Surg Oncol 41:1400–1405CrossRefPubMed
10.
Zurück zum Zitat Lee JH, Yoon YC, Seo SW et al (2020) Soft tissue sarcoma: DWI and DCE-MRI parameters correlate with Ki-67 labeling index. Eur Radiol 30:914–924CrossRefPubMed Lee JH, Yoon YC, Seo SW et al (2020) Soft tissue sarcoma: DWI and DCE-MRI parameters correlate with Ki-67 labeling index. Eur Radiol 30:914–924CrossRefPubMed
12.
Zurück zum Zitat Messiou C, Morosi C (2018) Imaging in retroperitoneal soft tissue sarcoma. J Surg Oncol 117:25–32CrossRefPubMed Messiou C, Morosi C (2018) Imaging in retroperitoneal soft tissue sarcoma. J Surg Oncol 117:25–32CrossRefPubMed
13.
Zurück zum Zitat Birgin E, Yang C, Hetjens S et al (2020) Core needle biopsy versus incisional biopsy for differentiation of soft-tissue sarcomas: a systematic review and meta-analysis. Cancer 126:1917–1928CrossRefPubMed Birgin E, Yang C, Hetjens S et al (2020) Core needle biopsy versus incisional biopsy for differentiation of soft-tissue sarcomas: a systematic review and meta-analysis. Cancer 126:1917–1928CrossRefPubMed
14.
Zurück zum Zitat Ray-Coquard I, Montesco MC, Coindre JM et al (2012) Sarcoma: concordance between initial diagnosis and centralized expert review in a population-based study within three European regions. Ann Oncol 23:2442–2449CrossRefPubMedPubMedCentral Ray-Coquard I, Montesco MC, Coindre JM et al (2012) Sarcoma: concordance between initial diagnosis and centralized expert review in a population-based study within three European regions. Ann Oncol 23:2442–2449CrossRefPubMedPubMedCentral
15.
Zurück zum Zitat Carvalho NA, Santiago KM, Maia JML et al (2023) Prevalence and clinical implications of germline pathogenic variants in cancer predisposing genes in young patients across sarcoma subtypes. J Med Genet Carvalho NA, Santiago KM, Maia JML et al (2023) Prevalence and clinical implications of germline pathogenic variants in cancer predisposing genes in young patients across sarcoma subtypes. J Med Genet
16.
Zurück zum Zitat Jennie V, Matthew SD, Joshua DS et al (2022) Germline predisposition to soft tissue sarcoma. J Cancer Metastasis Treat 8:31CrossRef Jennie V, Matthew SD, Joshua DS et al (2022) Germline predisposition to soft tissue sarcoma. J Cancer Metastasis Treat 8:31CrossRef
17.
Zurück zum Zitat Bonvalot S, Gaignard E, Stoeckle E et al (2019) Survival benefit of the surgical management of Retroperitoneal sarcoma in a reference center: a nationwide study of the French sarcoma group from the NetSarc database. Ann Surg Oncol 26:2286–2293CrossRefPubMed Bonvalot S, Gaignard E, Stoeckle E et al (2019) Survival benefit of the surgical management of Retroperitoneal sarcoma in a reference center: a nationwide study of the French sarcoma group from the NetSarc database. Ann Surg Oncol 26:2286–2293CrossRefPubMed
18.
Zurück zum Zitat Gingrich AA, Bateni SB, Monjazeb AM 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 24:3252–3263CrossRefPubMedPubMedCentral Gingrich AA, Bateni SB, Monjazeb AM 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 24:3252–3263CrossRefPubMedPubMedCentral
19.
Zurück zum Zitat Trovik CS, Bauer HC, Alvegård TA 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 36:710–716CrossRefPubMed Trovik CS, Bauer HC, Alvegård TA 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 36:710–716CrossRefPubMed
20.
Zurück zum Zitat Kasper B, Baumgarten C, Garcia J et al (2017) An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol 28:2399–2408CrossRefPubMedPubMedCentral Kasper B, Baumgarten C, Garcia J et al (2017) An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol 28:2399–2408CrossRefPubMedPubMedCentral
21.
Zurück zum Zitat Rauh J, Klein A, Baur-Melnyk A et al (2018) The role of surgical margins in atypical lipomatous tumours of the extremities. BMC Musculoskelet Disord 19:152CrossRefPubMed Rauh J, Klein A, Baur-Melnyk A et al (2018) The role of surgical margins in atypical lipomatous tumours of the extremities. BMC Musculoskelet Disord 19:152CrossRefPubMed
22.
Zurück zum Zitat Rosenberg SA, Tepper J, Glatstein E et al (1982) The treatment of soft-tissue sarcomas of the extremities: prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy. Ann Surg 196:305–315CrossRefPubMed Rosenberg SA, Tepper J, Glatstein E et al (1982) The treatment of soft-tissue sarcomas of the extremities: prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy. Ann Surg 196:305–315CrossRefPubMed
23.
Zurück zum Zitat Budach W, Budach V, Dinges S et al (1997) Correlation between primary chemo- and radiation sensitivity in a panel of highly malignant human soft tissue sarcoma xenografts. Radiother Oncol 42:181–187CrossRefPubMed Budach W, Budach V, Dinges S et al (1997) Correlation between primary chemo- and radiation sensitivity in a panel of highly malignant human soft tissue sarcoma xenografts. Radiother Oncol 42:181–187CrossRefPubMed
24.
Zurück zum Zitat Kepka L, Delaney TF, Suit HD et al (2005) Results of radiation therapy for unresected soft-tissue sarcomas. Int J Radiat Oncol Biol Phys 63:852–859CrossRefPubMed Kepka L, Delaney TF, Suit HD et al (2005) Results of radiation therapy for unresected soft-tissue sarcomas. Int J Radiat Oncol Biol Phys 63:852–859CrossRefPubMed
25.
Zurück zum Zitat Llacer-Moscardo C, Terlizzi M, Bonvalot S et al (2020) Pre- or postoperative radiotherapy for soft tissue sarcomas. Cancer Radiother 24:501–512CrossRefPubMed Llacer-Moscardo C, Terlizzi M, Bonvalot S et al (2020) Pre- or postoperative radiotherapy for soft tissue sarcomas. Cancer Radiother 24:501–512CrossRefPubMed
26.
Zurück zum Zitat Yang JC, Chang AE, Baker AR et al (1998) Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 16:197–203CrossRefPubMed Yang JC, Chang AE, Baker AR et al (1998) Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 16:197–203CrossRefPubMed
27.
Zurück zum Zitat Bonvalot S, Gronchi A, Le Péchoux C et al (2020) Preoperative radiotherapy plus surgery versus surgery alone for patients with primary retroperitoneal sarcoma (EORTC-62092: STRASS): a multicentre, open-label, randomised, phase 3 trial. Lancet Oncol 21:1366–1377CrossRefPubMed Bonvalot S, Gronchi A, Le Péchoux C et al (2020) Preoperative radiotherapy plus surgery versus surgery alone for patients with primary retroperitoneal sarcoma (EORTC-62092: STRASS): a multicentre, open-label, randomised, phase 3 trial. Lancet Oncol 21:1366–1377CrossRefPubMed
28.
Zurück zum Zitat Frustaci S, Gherlinzoni F, De Paoli A et al (2001) Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 19:1238–1247CrossRefPubMed Frustaci S, Gherlinzoni F, De Paoli A et al (2001) Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 19:1238–1247CrossRefPubMed
29.
Zurück zum Zitat Pervaiz N, Colterjohn N, Farrokhyar F et al (2008) A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer 113:573–581CrossRefPubMed Pervaiz N, Colterjohn N, Farrokhyar F et al (2008) A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer 113:573–581CrossRefPubMed
30.
Zurück zum Zitat Woll PJ, Reichardt P, Le Cesne A et al (2012) Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicentre randomised controlled trial. Lancet Oncol 13:1045–1054CrossRefPubMed Woll PJ, Reichardt P, Le Cesne A et al (2012) Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicentre randomised controlled trial. Lancet Oncol 13:1045–1054CrossRefPubMed
31.
Zurück zum Zitat Gronchi A, Palmerini E, Quagliuolo V et al (2020) 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. J Clin Oncol 38:2178–2186CrossRefPubMed Gronchi A, Palmerini E, Quagliuolo V et al (2020) 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. J Clin Oncol 38:2178–2186CrossRefPubMed
32.
Zurück zum Zitat Pasquali S, Palmerini E, Quagliuolo V 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 128:85–93CrossRefPubMed Pasquali S, Palmerini E, Quagliuolo V 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 128:85–93CrossRefPubMed
33.
Zurück zum Zitat Pasquali S, Pizzamiglio S, Touati N 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 109:51–60CrossRefPubMed Pasquali S, Pizzamiglio S, Touati N 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 109:51–60CrossRefPubMed
34.
Zurück zum Zitat D’ambrosio L, Touati N, Blay JY et al (2020) Doxorubicin plus dacarbazine, doxorubicin plus ifosfamide, or doxorubicin alone as a first-line treatment for advanced leiomyosarcoma: a propensity score matching analysis from the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. Cancer 126:2637–2647CrossRefPubMed D’ambrosio L, Touati N, Blay JY et al (2020) Doxorubicin plus dacarbazine, doxorubicin plus ifosfamide, or doxorubicin alone as a first-line treatment for advanced leiomyosarcoma: a propensity score matching analysis from the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. Cancer 126:2637–2647CrossRefPubMed
35.
Zurück zum Zitat Pastorino U, Buyse M, Friedel G et al (1997) Long-term results of lung metastasectomy: prognostic analyses based on 5206 cases. J Thorac Cardiovasc Surg 113:37–49CrossRefPubMed Pastorino U, Buyse M, Friedel G et al (1997) Long-term results of lung metastasectomy: prognostic analyses based on 5206 cases. J Thorac Cardiovasc Surg 113:37–49CrossRefPubMed
36.
Zurück zum Zitat Stork T, Boemans R, Hardes J et al (2021) Number of metastases and their response to chemotherapy impact survival of patients with isolated lung metastases from bone-derived sarcoma. BMC Cancer 21:375CrossRefPubMedPubMedCentral Stork T, Boemans R, Hardes J et al (2021) Number of metastases and their response to chemotherapy impact survival of patients with isolated lung metastases from bone-derived sarcoma. BMC Cancer 21:375CrossRefPubMedPubMedCentral
37.
Zurück zum Zitat Toussi MS, Bagheri R, Dayani M et al (2013) Pulmonary metastasectomy and repeat metastasectomy for soft-tissue sarcoma. Asian Cardiovasc Thorac Ann 21:437–442CrossRefPubMed Toussi MS, Bagheri R, Dayani M et al (2013) Pulmonary metastasectomy and repeat metastasectomy for soft-tissue sarcoma. Asian Cardiovasc Thorac Ann 21:437–442CrossRefPubMed
38.
Zurück zum Zitat Judson I, Verweij J, Gelderblom H 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 15:415–423CrossRefPubMed Judson I, Verweij J, Gelderblom H 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 15:415–423CrossRefPubMed
39.
Zurück zum Zitat Young RJ, Litière S, Lia M et al (2017) Predictive and prognostic factors associated with soft tissue sarcoma response to chemotherapy: a subgroup analysis of the European Organisation for Research and Treatment of Cancer 62012 study. Acta Oncol 56:1013–1020CrossRefPubMed Young RJ, Litière S, Lia M et al (2017) Predictive and prognostic factors associated with soft tissue sarcoma response to chemotherapy: a subgroup analysis of the European Organisation for Research and Treatment of Cancer 62012 study. Acta Oncol 56:1013–1020CrossRefPubMed
40.
Zurück zum Zitat Martín-Broto J, Reichardt P, Jones RL et al (2020) Different approaches to advanced soft tissue sarcomas depending on treatment line, goal of therapy and histological subtype. Expert Rev Anticancer Ther 20:15–28CrossRefPubMed Martín-Broto J, Reichardt P, Jones RL et al (2020) Different approaches to advanced soft tissue sarcomas depending on treatment line, goal of therapy and histological subtype. Expert Rev Anticancer Ther 20:15–28CrossRefPubMed
Metadaten
Titel
Diagnostik und Behandlung von Weichgewebesarkomen
verfasst von
Dr. med. Rainer Hamacher
Moritz Kaths
Wiebke K. Guder
Publikationsdatum
19.10.2023

Weitere Artikel der Ausgabe 11/2023

Die Onkologie 11/2023 Zur Ausgabe