Download PDF
Z Orthop Unfall 2019; 157(06): 644-653
DOI: 10.1055/a-0820-6366
Review/Übersicht
Georg Thieme Verlag KG Stuttgart · New York

Soft Tissue Sarcoma – a Current Review of the Diagnostic and Treatment Strategies

Article in several languages: English | deutsch
Sebastian Scheidt
1   Klinik und Poliklinik für Orthopädie und Unfallchirurgie, Universitätsklinikum Bonn
,
Cornelius Jacobs
1   Klinik und Poliklinik für Orthopädie und Unfallchirurgie, Universitätsklinikum Bonn
,
Sebastian Koob
1   Klinik und Poliklinik für Orthopädie und Unfallchirurgie, Universitätsklinikum Bonn
,
Kristian Welle
1   Klinik und Poliklinik für Orthopädie und Unfallchirurgie, Universitätsklinikum Bonn
,
Sebastian Walter
1   Klinik und Poliklinik für Orthopädie und Unfallchirurgie, Universitätsklinikum Bonn
,
Jens Jakob
2   Universitätsmedizin Göttingen, Klinik für Allgemein-, Viszeral- und Kinderchirurgie
,
Michael Kehrer
1   Klinik und Poliklinik für Orthopädie und Unfallchirurgie, Universitätsklinikum Bonn
,
Jan Schmolders
1   Klinik und Poliklinik für Orthopädie und Unfallchirurgie, Universitätsklinikum Bonn
› Author Affiliations
Further Information

Publication History

Publication Date:
08 February 2019 (online)

Also available at
    Permissions and Reprints

Abstract

Soft tissue sarcomas are a heterogeneous group of neoplasias that due to their often clinically silent appearance often remain undetected or experience delayed treatment. Especially soft tissue swelling is often misinterpreted by patients and doctors and trivialized or verified with an incorrect biopsy technique. The hereby evoked complications for the patients are serious and may be reduced by simply following the available guidelines. The treatment of soft tissue sarcomas requires a close interdisciplinary coordination between specialists in tumor orthopedics, oncology, radiology, pathology and radiotherapy. On the basis of a selective literature review, the following article points out the current evidence on the treatment and illustrates a treatment algorithm.

Key words

soft tissue sarcoma - tumor orthopedics - treatment algorithm - guidelines - review
 

  • References/Literatur

  • 1 Vilanova JC. WHO Classification of soft Tissue Tumors. In: Vanhoenacker FM, Parizel PM, Gielen JL. eds. Imaging of soft Tissue Tumors. Cham: Springer International Publishing; 2017: 187-196
    CrossrefGoogle Scholar
  • 2 Casali PG, Abecassis N, Bauer S. et al. Soft tissue and visceral sarcomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2018; 29 (Suppl. 04) iv268-iv269
    CrossrefPubMedGoogle Scholar
  • 3 Stiller CA, Trama A, Serraino D. et al. Descriptive epidemiology of sarcomas in Europe: report from the RARECARE project. Eur J Cancer 2013; 49: 684-695
    CrossrefPubMedGoogle Scholar
  • 4 Robert Koch-Institut (RKI). Gesundheitsberichtserstattung und Epidemiologie. Im Internet: https://www.umweltbundesamt.de/sites/default/files/medien/376/publikationen/umid_2_2016_solarien.pdf Stand: 13.11.2018
    PubMedGoogle Scholar
  • 5 Brodowicz T, Amann G, Leithner A. et al. Consensus diagnosis and therapy of soft tissue sarcoma. Wien Klin Wochenschr 2011; 124: 85-99
    PubMedGoogle Scholar
  • 6 Breitenseher M, Dominkus M, Amann G. Bildgebende Diagnostik und Therapie der Weichteiltumoren. Stuttgart: Thieme; 2008
    Google Scholar
  • 7 Tumorzentrum München (TMZ). Tumorregister München (TRM) des Tumorzentrums München (TZM) am Institut für Medizinische Informationsverarbeitung, Biometrie und Epidemiologie (IBE) der Ludwig-Maximilians-Universität (LMU) München. Im Internet: https://www.tumorregister-muenchen.de Stand: 13.11.2018
    PubMedGoogle Scholar
  • 8 Saltus CW, Calingaert B, Candrilli S. et al. Epidemiology of adult soft-tissue sarcomas in Germany. Sarcoma 2018; 2018: 5671926 doi:10.1155/2018/5671926
    PubMedGoogle Scholar
  • 9 Bauer HC, Trovik CS, Alvegård TA. et al. Monitoring referral and treatment in soft tissue sarcoma: study based on 1,851 patients from the Scandinavian Sarcoma Group Register. Acta Orthop Scand 2001; 72: 150-159
    CrossrefPubMedGoogle Scholar
  • 10 Thway K. Pathology of soft tissue sarcomas. Clin Oncol (R Coll Radiol) 2009; 21: 695-705
    CrossrefPubMedGoogle Scholar
  • 11 Stürzl M, Blasig C, Schreier A. et al. Expression of HHV‐8 latency‐associated T0.7 RNA in spindle cells and endothelial cells of AIDS‐associated, classical and African Kaposiʼs sarcoma. Int J Cancer 1997; 72: 68-71
    CrossrefPubMedGoogle Scholar
  • 12 Hatley ME, Tang W, Garcia MR. et al. A mouse model of rhabdomyosarcoma originating from the adipocyte lineage. Cancer Cell 2012; 22: 536-546
    CrossrefPubMedGoogle Scholar
  • 13 Patel SR. Radiation-induced sarcoma. Curr Treat Options Oncol 2000; 1: 258-261
    CrossrefPubMedGoogle Scholar
  • 14 Kirova YM, Vilcoq JR, Asselain B. et al. Radiation-induced sarcomas after radiotherapy for breast carcinoma: a large-scale single-institution review. Cancer 2005; 104: 856-863
    CrossrefPubMedGoogle Scholar
  • 15 Gilbert NF, Cannon CP, Lin PP. et al. Soft-tissue sarcoma. J Am Acad Orthop Surg 2009; 17: 40-47
    CrossrefPubMedGoogle Scholar
  • 16 Schaser KD, Melcher I, Settmacher U. et al. [The multidisciplinary approach to reconstructive surgery of the extremities–considerations for trauma and orthopedic surgery]. Chirurg 2004; 75: 399-410
    CrossrefPubMedGoogle Scholar
  • 17 Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res 1980; (153) 106-120
    PubMedGoogle Scholar
  • 18 Johnson CJ, Pynsent PB, Grimer RJ. Clinical features of soft tissue sarcomas. Ann R Coll Surg Engl 2001; 83: 203-205
    PubMedGoogle Scholar
  • 19 Nandra R, Forsberg J, Grimer R. If your lump is bigger than a golf ball and growing, think Sarcoma. Eur J Surg Oncol 2015; 41: 1400-1405
    CrossrefPubMedGoogle Scholar
  • 20 Buvarp Dyrop H, Vedsted P, Rædkjær M. et al. Routes to diagnosis for suspected sarcoma: the impact of symptoms and clinical findings on the diagnostic process. Sarcoma 2016; 2016: 8639272 doi:10.1155/2016/8639272
    PubMedGoogle Scholar
  • 21 Lakkaraju A, Sinha R, Garikipati R. et al. Ultrasound for initial evaluation and triage of clinically suspicious soft-tissue masses. Clin Radiol 2009; 64: 615-621
    CrossrefPubMedGoogle Scholar
  • 22 Brisse HJ, Orbach D, Klijanienko J. Soft tissue tumours: imaging strategy. Pediatr Radiol 2010; 40: 1019-1028
    CrossrefPubMedGoogle Scholar
  • 23 Massengill AD, Seeger LL, Eckardt JJ. The role of plain radiography, computed tomography, and magnetic resonance imaging in sarcoma evaluation. Hematol Oncol Clin North Am 1995; 9: 571-604
    CrossrefPubMedGoogle Scholar
  • 24 Crim JR, Seeger LL, Yao L. et al. Diagnosis of soft-tissue masses with MR imaging: can benign masses be differentiated from malignant ones?. Radiology 1992; 185: 581-586
    CrossrefPubMedGoogle Scholar
  • 25 van Rijswijk CSP, Geirnaerdt MJA, Hogendoorn PCW. et al. Soft-tissue tumors: value of static and dynamic gadopentetate dimeglumine-enhanced MR imaging in prediction of malignancy. Radiology 2004; 233: 493-502
    CrossrefPubMedGoogle Scholar
  • 26 Wahl RL, Jacene H, Kasamon Y. et al. From RECIST to PERCIST: Evolving Considerations for PET response criteria in solid tumors. J Nucl Med 2009; 50 (Suppl. 01) 122S-150S
    CrossrefPubMedGoogle Scholar
  • 27 Benz MR, Czernin J, Allen-Auerbach MS. et al. FDG-PET/CT imaging predicts histopathologic treatment responses after the initial cycle of neoadjuvant chemotherapy in high-grade soft-tissue sarcomas. Clin Cancer Res 2009; 15: 2856-2863
    CrossrefPubMedGoogle Scholar
  • 28 Fendler WP, Lehmann M, Todica A. et al. PET response criteria in solid tumors predicts progression-free survival and time to local or distant progression after chemotherapy with regional hyperthermia for soft-tissue sarcoma. J Nucl Med 2015; 56: 530-537
    CrossrefPubMedGoogle Scholar
  • 29 Benz MR, Dry SM, Eilber FC. et al. Correlation between glycolytic phenotype and tumor grade in soft-tissue sarcomas by 18F-FDG PET. J Nucl Med 2010; 51: 1174-1181
    CrossrefPubMedGoogle Scholar
  • 30 Fendler WP, Chalkidis RP, Ilhan H. et al. Evaluation of several FDG PET parameters for prediction of soft tissue tumour grade at primary diagnosis and recurrence. Eur Radiol 2015; 25: 2214-2221
    CrossrefPubMedGoogle Scholar
  • 31 Varma DG. Optimal radiologic imaging of soft tissue sarcomas. Semin Surg Oncol 1999; 17: 2-10
    CrossrefPubMedGoogle Scholar
  • 32 Leithner A, Maurer-Ertl W, Windhager R. Biopsy of bone and soft tissue tumours: hints and hazards. Recent Results Cancer Res 2009; 179: 3-10
    PubMedGoogle Scholar
  • 33 Kasraeian S, Allison DC, Ahlmann ER. et al. A comparison of fine-needle aspiration, core biopsy, and surgical biopsy in the diagnosis of extremity soft tissue masses. Clin Orthop Relat Res 2010; 468: 2992-3002
    CrossrefPubMedGoogle Scholar
  • 34 Pohlig F, Kirchhoff C, Lenze U. et al. Percutaneous core needle biopsy versus open biopsy in diagnostics of bone and soft tissue sarcoma: a retrospective study. Eur J Med Res 2012; 17: 29
    CrossrefPubMedGoogle Scholar
  • 35 Bickels J, Jelinek JS, Shmookler BM. et al. Biopsy of musculoskeletal tumors. Current concepts. Clin Orthop Relat Res 1999; (368) 212-219
    PubMedGoogle Scholar
  • 36 Skrzynski MC, Biermann JS, Montag A. et al. Diagnostic accuracy and charge-savings of outpatient core needle biopsy compared with open biopsy of musculoskeletal tumors. J Bone Joint Surg Am 1996; 78: 644-649
    CrossrefPubMedGoogle Scholar
  • 37 Ng VY, Thomas K, Crist M. et al. Fine needle aspiration for clinical triage of extremity soft tissue masses. Clin Orthop Relat Res 2009; 468: 1120-1128
    PubMedGoogle Scholar
  • 38 Le H, Lee S, Munk P. Image-guided musculoskeletal biopsies. Semin Intervent Radiol 2010; 27: 191-198
    Article in Thieme ConnectPubMedGoogle Scholar
  • 39 Ashford RU, Scolyer RA, McCarthy SW. et al. The role of intra-operative pathological evaluation in the management of musculoskeletal tumours. Recent Results Cancer Res 2009; 179: 11-24
    PubMedGoogle Scholar
  • 40 Rougraff BT, Aboulafia A, Biermann JS. et al. Biopsy of soft tissue masses: evidence-based medicine for the musculoskeletal tumor society. Clin Orthop Relat Res 2009; 467: 2783-2791
    CrossrefPubMedGoogle Scholar
  • 41 Heim-Hall J, Yohe SL. Application of immunohistochemistry to soft tissue neoplasms. Arch Pathol Lab Med 2008; 132: 476-489
    CrossrefPubMedGoogle Scholar
  • 42 Trojani M, Contesso G, Coindre JM. et al. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer 1984; 33: 37-42
    CrossrefPubMedGoogle Scholar
  • 43 Edge SB, Compton CC. The American Joint Committee on Cancer: the 7th edition of the AJCC cancer staging manual and the future of TNM. Ann Surg Oncol 2010; 17: 1471-1474
    CrossrefPubMedGoogle Scholar
  • 44 Rosenberg SA, Tepper J, Glatstein E. et al. 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 1982; 196: 305-315
    CrossrefPubMedGoogle Scholar
  • 45 Tunn PU, Kettelhack C, Dürr HR. Standardized approach to the treatment of adult soft tissue sarcoma of the extremities. Recent Results Cancer Res 2009; 179: 211-228
    PubMedGoogle Scholar
  • 46 Maduekwe UN, Hornicek FJ, Springfield DS. et al. Role of sentinel lymph node biopsy in the staging of synovial, epithelioid, and clear cell sarcomas. Ann Surg Oncol 2009; 16: 1356-1363
    CrossrefPubMedGoogle Scholar
  • 47 Behranwala KA, AʼHern R, Omar AM. et al. Prognosis of lymph node metastasis in soft tissue sarcoma. Ann Surg Oncol 2004; 11: 714-719
    CrossrefPubMedGoogle Scholar
  • 48 Daigeler A, Kuhnen C, Moritz R. et al. Lymph node metastases in soft tissue sarcomas: a single center analysis of 1,597 patients. Langenbecks Arch Surg 2008; 394: 321-329
    PubMedGoogle Scholar
  • 49 Ferguson PC, Kulidjian AA, Jones KB. et al. Peripheral nerve considerations in the management of extremity soft tissue sarcomas. Recent Results Cancer Res 2009; 179: 243-256
    PubMedGoogle Scholar
  • 50 Lin PP, Pino ED, Normand AN. et al. Periosteal margin in soft‐tissue sarcoma. Cancer 2007; 109: 598-602
    CrossrefPubMedGoogle Scholar
  • 51 Funovics PT, Vaselic S, Panotopoulos J. et al. The impact of re-excision of inadequately resected soft tissue sarcomas on surgical therapy, results, and prognosis: A single institution experience with 682 patients. J Surg Oncol 2010; 102: 626-633
    CrossrefPubMedGoogle Scholar
  • 52 Strander H, Turesson I, Cavallin-Ståhl E. A systematic overview of radiation therapy effects in soft tissue sarcomas. Acta Oncol 2003; 42: 516-531
    CrossrefPubMedGoogle Scholar
  • 53 Yang JC, Chang AE, Baker AR. et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 1998; 16: 197-203
    CrossrefPubMedGoogle Scholar
  • 54 OʼSullivan B, Davis AM, Turcotte R. et al. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet 2002; 359: 2235-2241
    CrossrefPubMedGoogle Scholar
  • 55 Salduz A, Alpan B, Valiyev N. et al. Neoadjuvant radiotherapy for myxoid liposarcomas: oncologic outcomes and histopathologic correlations. Acta Orthop Traumatol Turc 2017; 51: 355-361
    CrossrefPubMedGoogle Scholar
  • 56 Koseła-Paterczyk H, Szumera-Ciećkiewicz A, Szacht M. et al. Efficacy of neoadjuvant hypofractionated radiotherapy in patients with locally advanced myxoid liposarcoma. Eur J Surg Oncol 2016; 42: 891-898
    CrossrefPubMedGoogle Scholar
  • 57 Oertel S, Treiber M, Zahlten-Hinguranage A. et al. Intraoperative electron boost radiation followed by moderate doses of external beam radiotherapy in limb-sparing treatment of patients with extremity soft-tissue sarcoma. Int J Radiat Oncol Biol Phys 2006; 64: 1416-1423
    CrossrefPubMedGoogle Scholar
  • 58 Roeder F, Krempien R. Intraoperative radiation therapy (IORT) in soft-tissue sarcoma. Radiat Oncol 2017; 12: 20 doi:10.1186/s13014-016-0751-2
    CrossrefPubMedGoogle Scholar
  • 59 Kennedy JE. High-intensity focused ultrasound in the treatment of solid tumours. Nat Rev Cancer 2005; 5: 321-327
    CrossrefPubMedGoogle Scholar
  • 60 Frustaci S, Gherlinzoni F, De Paoli A. et al. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 2001; 19: 1238-1247
    CrossrefPubMedGoogle Scholar
  • 61 Woll PJ, Reichardt P, Le Cesne A. et al. Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC62931): a multicentre randomised controlled trial. Lancet Oncol 2012; 13: 1045-1054
    CrossrefPubMedGoogle Scholar
  • 62 [Anonymous] Adjuvant chemotherapy for localised resectable soft tissue sarcoma in adults. Sarcoma Meta-analysis Collaboration (SMAC). Cochrane Database Syst Rev 2000; (02) CD001419
    PubMedGoogle Scholar
  • 63 Pervaiz N, Colterjohn N, Farrokhyar F. et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer 2008; 113: 573-581
    CrossrefPubMedGoogle Scholar
  • 64 Issels RD, Lindner LH, Verweij J. et al. Neo-adjuvant chemotherapy alone or with regional hyperthermia for localised high-risk soft-tissue sarcoma: a randomised phase 3 multicentre study. Lancet Oncol 2010; 11: 561-570
    CrossrefPubMedGoogle Scholar
  • 65 Meric F, Hess KR, Varma DGK. et al. Radiographic response to neoadjuvant chemotherapy is a predictor of local control and survival in soft tissue sarcomas. Cancer 2002; 95: 1120-1126
    CrossrefPubMedGoogle Scholar
  • 66 Antman K, Crowley J, Balcerzak SP. et al. An intergroup phase III randomized study of doxorubicin and dacarbazine with or without ifosfamide and mesna in advanced soft tissue and bone sarcomas. J Clin Oncol 1993; 11: 1276-1285
    CrossrefPubMedGoogle Scholar
  • 67 Judson I, Verweij J, Gelderblom H. et al. 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 2014; 15: 415-423
    CrossrefPubMedGoogle Scholar
  • 68 Tap WD, Jones RL, Van Tine BA. et al. Olaratumab and doxorubicin versus doxorubicin alone for treatment of soft-tissue sarcoma: an open-label phase 1b and randomised phase 2 trial. Lancet 2016; 388: 488-497
    CrossrefPubMedGoogle Scholar
  • 69 Gronchi A, Ferrari S, Quagliuolo V. et al. Full-dose neoadjuvant anthracycline + ifosfamide chemotherapy is associated with a relapse free survival (RFS) and overall survival (OS) benefit in localized high-risk adult soft tissue sarcomas (STS) of the extremities and trunk wall: Interim analysis of a prospective randomized trial. Ann Oncol 2016; DOI: 10.1093/annonc/mdw435.52.
    CrossrefPubMedGoogle Scholar
  • 70 Grosso F, Jones RL, Demetri GD. et al. Efficacy of trabectedin (ecteinascidin-743) in advanced pretreated myxoid liposarcomas: a retrospective study. Lancet Oncol 2007; 8: 595-602
    CrossrefPubMedGoogle Scholar
  • 71 Schöffski P, Chawla S, Maki RG. et al. Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial. Lancet 2016; 387: 1629-1637
    CrossrefPubMedGoogle Scholar
  • 72 Miller BJ, Carmody Soni EE, Reith JD. et al. CT scans for pulmonary surveillance may be overused in lower-grade sarcoma. Iowa Orthop J 2012; 32: 28-34
    PubMedGoogle Scholar
  • 73 Stojadinovic A, Leung DHY, Allen P. et al. Primary adult soft tissue sarcoma: time-dependent influence of prognostic variables. J Clin Oncol 2002; 20: 4344-4352
    CrossrefPubMedGoogle Scholar