Zusammenfassung
Trotz der Tatsache, dass Weichteilsarkome mit einer Inzidenz von ca. 2–4 pro 100.000/Jahr eher zu den seltenen Tumoren zählen, erfordern sie in ganz besonderer Weise multimodale Therapiekonzepte. Basierend auf einer Referenzpathologie steht zunächst der Versuch einer kompletten chirurgischen Resektion im Vordergrund. Durch eine adjuvante Strahlentherapie kann bei den meisten Weichteilsarkomen nach erfolgter R0-Resektion die Lokalrezidivrate weiter gesenkt werden. Bei primär inoperablen oder nur unvollständig resezierbaren Tumoren ist eine neoadjuvante Chemotherapie unter Einschluss einer regionalen Tiefenhyperthermie zu diskutieren. Patienten mit metastasiertem Weichteilsarkom erhalten in palliativer Intention in der Regel eine anthrazyklinbasierte Chemotherapie. Prognostisch günstiger, auch im metastasierten Stadium, verlaufen gastrointestinale Stromatumoren, bei denen unter Einsatz der Tyrosinkinase-Inhibitoren Imatinib und Sunitinib lange Remissionszeiten induziert werden können.
Abstract
Despite the fact that soft tissue sarcomas are representing a rare tumor entity with a low incidence rate of about 2–4 per 100.000 per year, they highly require a multimodality therapeutic approach. Based on a reference pathology a complete surgical resection is the first treatment goal. After accomplished R0 resection the local relapse rate can be further decreased by an adjuvant radiotherapy. For primarily irresectable or only partially respectable tumors a neoadjuvant chemotherapy combined with regional hyperthermia should be considered. Patients with metastasized soft tissue sarcomas should receive an anthracyclin-based chemotherapy in a palliative intention. Prognostically more favorable are gastrointestinal stroma tumor, also in advanced stages with metastases, since the tyrosine kinase inhibitors imatinib and sunitinib can induce durable remissions.
Literatur
Antoch G, Kanja J, Bauer S et al (2004) Comparison of PET, CT, and dual-modality PET/CT imaging for monitoring of imatinib (STI571) therapy in patients with gastrointestinal stromal tumors. Nucl Med 45:357–365
Baldini EH, Goldberg, J, Jenner C et al (1999) Long-term outcomes after function-sparing surgery without radiotherapy for soft tissue sarcoma of the extremities and trunk. J Clin Oncol 17:3252–3259
Bauer S, Seeber S, Schütte J (2004) Gemcitabine in the treatment of soft tissue sarcomas. Onkologie 27:180–186
Cohen MH, Cortazar P, Justice R, Pazdur R (2010) Approval summary: imatinib mesylate in the adjuvant treatment of malignant gastrointestinal stromal tumors. Oncologist 15:300–307
Coindre JM, Binh Bui N, Bonichon F et al (1988) Histopathologic grading in spindle cell soft tissue sarcomas. Cancer 61:2305–2309
D’Adamo DR, Keohan M, Schuetze S et al (2007) Clinical results of a phase II study of sorafenib in patients with non-GIST sarcomas (CTEP study #7060). Proc Am Soc Clin Oncol 25:18 s (Abstr)
Debiec-Rychter M, Sciot R, Le Cesne A et al (2006) KIT mutations and dose selection for imatinib in patients with advanced gastrointestinal stromal tumours. Eur J Cancer 42:1093–1103
Delaloge S, Yovine A, Taamma A et al (2001) Ecteinascidin-743: a marine-derived compound in advanced, pretreated sarcoma patients – preliminary evidence of activity. J Clin Oncol 19:1248–1255
Demetri GD, Oosterom AT van, Garrett CR et al (2006) Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet 368:1329–1338
Fata F, O’Reilly E, Ilson D et al (1999) Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer 86:2034–2037
Ferrari A et al (2003) Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution. Cancer 98:571–580
Fletcher CD, Berman JJ, Corless C et al (2002) Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol 33:459–465
Hartmann JT, Patel S (2005) Recent developments in salvage chemotherapy for patients with metastatic soft tissue sarcoma. Drugs 65:167–178
Heinrich MC, Corless CL, Demetri GD et al (2003) Kinase mutations and imatinib response in patients with metastatic gastrointestinal stromal tumor. J Clin Oncol 21:4342–4349
Hensley ML, Maki R, Venkatraman E et al (2002) Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 20:2824–2831
Issels RD, Lindner LH, Verweij J et al (2010) Neo-adjuvant chemotherapy alone or with regional hyperthermia for localized high-risk soft-tissue sarcoma: a randomized phase 3 multicentre study. Lancet Oncol 11:561–570
Lewis JJ, Leung D, Woodruff JM, Brennan MF (1998) Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg 228:355–365
Lindner NJ, Scarborough MT, Powell GJ et al (1999) Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. Eur J Surg Oncol 25:392–397
Manoso MW, Frassica DA, Deune EG, Frassica FJ (2005) Outcomes of re-excision after unplanned excisions of soft-tissue sarcomas. Surg Oncol 91:153–158
Miettinen M, Lasota J (2006) Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med 130:1466–1478
O’Sullivan B, Davis A (2001) A randomized phase III trial of pre-operative compared to post-operative radiotherapy in extremity soft tissue sarcoma. Int J Radiat Oncol Biol Phys 51–151
Patel SR, Gandhi V, Jenkins J et al (2001) Phase II clinical investigation of gemcitabine in advanced soft tissue sarcomas and window evaluation of dose rate on gemcitabine triphosphate accumulation. J Clin Oncol 19:3483–3489
Pisters PW, Harrison LB, Woodruff JM et al (1994) A prospective randomized trial of adjuvant brachytherapy in the management of low-grade soft tissue sarcomas of the extremity and superficial trunk. J Clin Oncol 12:1150–1155
Pisters PW, Leung DH, Woodruff J et al (1996) Analysis of prognostic factors in 1041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 14:1679–1689
Pollack A, Zagars GK, Goswitz MS et al (1998) Preoperative vs. postoperative radiotherapy in the treatment of soft tissue sarcomas: a matter of presentation. Int J Radiat Oncol Biol Phys 42:563–572
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 196:305–315
Sarcoma Meta-Analysis Collaboration (1997) Adjuvant chemotherapy for localized respectable soft-tissue sarcoma of adults: meta-analysis of individual data. Lancet 350:1647–1654
Schlemmer M, Wendtner CM, Falk M et al (2006) Efficacy of consolidation high-dose chemotherapy with ifosfamide, carboplatin and etoposide (HD-ICE) followed by autologous peripheral blood stem cell rescue in chemosensitive patients with metastatic soft tissue sarcomas. Oncology 71:32–39
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 36:710–716
Verweij J, Casali PG, Zalcberg J et al (2004) Progression-free survival in gastrointestinal stromal tumors with high-dose imatinib: randomised trial. Lancet 364:1127–1134
Wendtner C-M, Abdel-Rahman S, Krych M et al (2002) Response to neoadjuvant chemotherapy combined with regional hyperthermia predicts long-term survival for adult patients with retroperitoneal and visceral high-risk soft tissue sarcomas. J Clin Oncol 20:3156–3164
Woll PJ, Glabbeke M van, Hohenberger P et al (2007) Adjuvant chemotherapy with doxorubicin and ifosfamide in resected soft tissue sarcoma: interim analysis of a randomised phase III trial. Proc Am Soc Clin Oncol 25:547 s
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 16:197–203
Zalcberg JR, Verweij J, Casali PG et al (2005) Outcome of patients with advanced gastro-intestinal stromal tumours crossing over to a daily imatinib dose of 800 mg after progression on 400 mg. Eur J Cancer 41:1751–1757
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Wendtner, CM., Delank, S. & Eich, H. Multimodale Therapiekonzepte bei Weichteilsarkomen. Internist 51, 1388–1396 (2010). https://doi.org/10.1007/s00108-010-2672-8
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DOI: https://doi.org/10.1007/s00108-010-2672-8