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DOI: 10.1055/a-0820-6366
Soft Tissue Sarcoma – a Current Review of the Diagnostic and Treatment Strategies
Article in several languages: English | deutschPublication History
Publication Date:
08 February 2019 (online)
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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.
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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
- 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
- 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
- 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
- 5 Brodowicz T, Amann G, Leithner A. et al. Consensus diagnosis and therapy of soft tissue sarcoma. Wien Klin Wochenschr 2011; 124: 85-99
- 6 Breitenseher M, Dominkus M, Amann G. Bildgebende Diagnostik und Therapie der Weichteiltumoren. Stuttgart: Thieme; 2008
- 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
- 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
- 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
- 10 Thway K. Pathology of soft tissue sarcomas. Clin Oncol (R Coll Radiol) 2009; 21: 695-705
- 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
- 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
- 13 Patel SR. Radiation-induced sarcoma. Curr Treat Options Oncol 2000; 1: 258-261
- 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
- 15 Gilbert NF, Cannon CP, Lin PP. et al. Soft-tissue sarcoma. J Am Acad Orthop Surg 2009; 17: 40-47
- 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
- 17 Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res 1980; (153) 106-120
- 18 Johnson CJ, Pynsent PB, Grimer RJ. Clinical features of soft tissue sarcomas. Ann R Coll Surg Engl 2001; 83: 203-205
- 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
- 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
- 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
- 22 Brisse HJ, Orbach D, Klijanienko J. Soft tissue tumours: imaging strategy. Pediatr Radiol 2010; 40: 1019-1028
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 31 Varma DG. Optimal radiologic imaging of soft tissue sarcomas. Semin Surg Oncol 1999; 17: 2-10
- 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
- 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
- 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
- 35 Bickels J, Jelinek JS, Shmookler BM. et al. Biopsy of musculoskeletal tumors. Current concepts. Clin Orthop Relat Res 1999; (368) 212-219
- 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
- 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
- 38 Le H, Lee S, Munk P. Image-guided musculoskeletal biopsies. Semin Intervent Radiol 2010; 27: 191-198
- 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
- 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
- 41 Heim-Hall J, Yohe SL. Application of immunohistochemistry to soft tissue neoplasms. Arch Pathol Lab Med 2008; 132: 476-489
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 50 Lin PP, Pino ED, Normand AN. et al. Periosteal margin in soft‐tissue sarcoma. Cancer 2007; 109: 598-602
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 59 Kennedy JE. High-intensity focused ultrasound in the treatment of solid tumours. Nat Rev Cancer 2005; 5: 321-327
- 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
- 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
- 62 [Anonymous] Adjuvant chemotherapy for localised resectable soft tissue sarcoma in adults. Sarcoma Meta-analysis Collaboration (SMAC). Cochrane Database Syst Rev 2000; (02) CD001419
- 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
- 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
- 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
- 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
- 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
- 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
- 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.
- 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
- 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
- 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
- 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