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Chap 37 - SURGICAL MANAGEMENT OF SOFT TISSUE SARCOMA: HISTOLOGIC TYPE AND GRADE GUIDE SURGICAL PLANNING AND INTEGRATION OF MULTIMODALITY THERAPY

Published online by Cambridge University Press:  01 March 2011

Markku Miettinen
Affiliation:
Armed Forces Institute of Pathology, Washington DC
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Summary

The primary treatment of sarcoma is surgical resection; however, as the understanding of this rare disease has improved, it is now clear that different sarcoma subtypes have varying growth patterns and metastatic potential. Although surgery continues to play the central role in the treatment of sarcomas, pathologic subtyping has allowed characterization of sarcomas that have an improved sensitivity to chemotherapy or radiation therapy. This has translated into a refinement of the surgical approach, and the addition of multidisciplinary treatment in an adjuvant or neo-adjuvant fashion. This review focuses on the surgical management of sarcoma, which is driven by the pathologic subtype.

CLINICAL EVALUATION AND BIOPSY

Soft tissue sarcomas (STSs) are often asymptomatic and are usually found incidentally. A survey on the clinical presentation demonstrated that most patients present with a mass, only one third of which cause pain symptoms. There can be an antecedent history of trauma, which is usually not related but often draws attention to a mass. There is often a hesitation to diagnose sarcoma; up to 20% of patients have been found to have a delay of 6 or more months between presentation to a physician and tissue diagnosis.

The differential diagnosis of a mass includes a hematoma, benign lipoma, lymphoma, germ cell tumor, and sarcoma. A history of an enlarging or changing lesion should raise the suspicion of a malignancy. On physical examination it is important to differentiate between a soft, mobile lesion and one that is fixed or invading local structures.

Type
Chapter
Information
Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
, pp. 1057 - 1069
Publisher: Cambridge University Press
Print publication year: 2010

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References

Lawrence, W Jr, Donegan, WL, Natarajan, N, Mettlin, C, Beart, R, Winchester, D. Adult soft tissue sarcomas. A pattern of care survey of the American College of Surgeons. Ann Surg Apr 1987;205(4):349–359.CrossRefGoogle ScholarPubMed
Heslin, MJ, Lewis, JJ, Woodruff, JM, Brennan, MF. Core needle biopsy for diagnosis of extremity soft tissue sarcoma. Ann Surg Oncol Jul-Aug 1997;4(5):425–431.CrossRefGoogle ScholarPubMed
Lewis, JJ, Leung, D, Espat, J, Woodruff, JM, Brennan, MF. Effect of reresection in extremity soft tissue sarcoma. Ann Surg May 2000;231(5):655–663.CrossRefGoogle Scholar
Melendez, M, Brandt, K, Evans, GR. Sciatic nerve reconstruction: limb preservation after sarcoma resection. Ann Plast Surg Apr 2001;46(4):375–381.CrossRefGoogle ScholarPubMed
Brooks, AD, Gold, JS, Graham, D. Resection of the sciatic, peroneal, or tibial nerves: assessment of functional status. Ann Surg Oncol Jan-Feb 2002;9(1):41–47.CrossRefGoogle ScholarPubMed
Saint-Cyr, M, Langstein, HN. Reconstruction of the hand and upper extremity after tumor resection. J Surg Oncol Nov 1 2006;94(6):490–503.CrossRefGoogle ScholarPubMed
Baxter, BT, Mahoney, C, Johnson, PJ. Concomitant arterial and venous reconstruction with resection of lower extremity sarcomas. Ann Vasc Surg May 2007;21(3):272–279.CrossRefGoogle ScholarPubMed
Adelani, MA, Holt, GE, Dittus, RS, Passman, MA, Schwartz, HS. Revascularization after segmental resection of lower extremity soft tissue sarcomas. J Surg Oncol May 1 2007; 95(6):455–460.CrossRefGoogle ScholarPubMed
Ghert, MA, Davis, AM, Griffin, AM. The surgical and functional outcome of limb-salvage surgery with vascular reconstruction for soft tissue sarcoma of the extremity. Ann Surg Oncol Dec 2005;12(12):1102–1110.CrossRefGoogle ScholarPubMed
Ariyan, S. The functional pectoralis major musculocutaneous island flap for head and neck reconstruction. Plast Reconstr Surg Oct 1990;86(4):807–808.CrossRefGoogle ScholarPubMed
Ross, DA, Hundal, JS, Son, YH. Microsurgical free flap reconstruction outcomes in head and neck cancer patients after surgical extirpation and intraoperative brachytherapy. Laryngoscope Jul 2004;114(7):1170–1176.CrossRefGoogle ScholarPubMed
Duman, H, Evans, GR, Reece, G. Brachytherapy: reconstructive options and the role of plastic surgery. Ann Plast Surg Nov 2000;45(5):477–480.CrossRefGoogle ScholarPubMed
Bui, DT, Cordeiro, PG, Hu, QY, Disa, JJ, Pusic, A, Mehrara, BJ. Free flap reexploration: indications, treatment, and outcomes in 1193 free flaps. Plast Reconstr Surg Jun 2007;119(7):2092–2100.CrossRefGoogle ScholarPubMed
Kooby, DA, Antonescu, CR, Brennan, MF, Singer, S. Atypical lipomatous tumor/well-differentiated liposarcoma of the extremity and trunk wall: importance of histological subtype with treatment recommendations. Ann Surg Oncol Jan 2004;11(1):78–84.CrossRefGoogle ScholarPubMed
Bowne, WB, Antonescu, CR, Leung, DH. Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution. Cancer Jun 15 2000;88(12):2711–2720.3.0.CO;2-M>CrossRefGoogle Scholar
Merchant, NB, Lewis, JJ, Woodruff, JM, Leung, DH, Brennan, MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer Nov 15 1999;86(10):2045–2052.3.0.CO;2-F>CrossRefGoogle Scholar
Rosenberg, SA, Tepper, J, Glatstein, E. 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 Sep 1982;196(3):305–315.CrossRefGoogle ScholarPubMed
Williard, WC, Hajdu, SI, Casper, ES, Brennan, MF. Comparison of amputation with limb-sparing operations for adult soft tissue sarcoma of the extremity. Ann Surg Mar 1992;215(3):269–275.CrossRefGoogle ScholarPubMed
Pisters, PW, Harrison, LB, Leung, DH, Woodruff, JM, Casper, ES, Brennan, MF. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol Mar 1996;14(3):859–868.CrossRefGoogle ScholarPubMed
O'Sullivan, B, Davis, AM, Turcotte, R. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet Jun 29 2002; 359(9325):2235–2241.CrossRefGoogle ScholarPubMed
Yang, JC, Chang, AE, Baker, AR. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol Jan 1998;16(1):197–203.CrossRefGoogle ScholarPubMed
Baldini, EH, Goldberg, J, Jenner, C. Long-term outcomes after function-sparing surgery without radiotherapy for soft tissue sarcoma of the extremities and trunk. J Clin Oncol Oct 1999;17(10):3252–3259.CrossRefGoogle ScholarPubMed
Lin, PP, Schupak, KD, Boland, PJ, Brennan, MF, Healey, JH. Pathologic femoral fracture after periosteal excision and radiation for the treatment of soft tissue sarcoma. Cancer Jun 15 1998;82(12):2356–2365.3.0.CO;2-Q>CrossRefGoogle ScholarPubMed
Alektiar, KM, Hong, L, Brennan, MF, Della-Biancia, C, Singer, S. Intensity modulated radiation therapy for primary soft tissue sarcoma of the extremity: preliminary results. Int J Radiat Oncol Biol Phys Jun 1 2007;68(2):458–464.CrossRefGoogle ScholarPubMed
Rosenberg, SA, Tepper, J, Glatstein, E. Prospective randomized evaluation of adjuvant chemotherapy in adults with soft tissue sarcomas of the extremities. Cancer Aug 1 1983;52(3):424–434.3.0.CO;2-8>CrossRefGoogle ScholarPubMed
Chang, AE, Kinsella, T, Glatstein, E. Adjuvant chemotherapy for patients with high-grade soft-tissue sarcomas of the extremityJ Clin Oncol. Sep 1988;6(9):1491–1500.CrossRefGoogle ScholarPubMed
,Sarcoma Meta-analysis Collaboration. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Lancet 1997;350(9092): 1647–1654.CrossRefGoogle Scholar
Elias, A, Ryan, L, Sulkes, A, Collins, J, Aisner, J, Antman, KH. Response to mesna, doxorubicin, ifosfamide, and dacarbazine in 108 patients with metastatic or unresectable sarcoma and no prior chemotherapy. J Clin Oncol Sep 1989;7(9):1208–1216.CrossRefGoogle ScholarPubMed
Elias, AD, Eder, JP, Shea, T, Begg, CB, Frei, E, 3rd, Antman KH. High-dose ifosfamide with mesna uroprotection: a phase I study. J Clin Oncol Jan 1990;8(1):170–178.CrossRefGoogle ScholarPubMed
Antman, KH, Elias, A, Ryan, L. Ifosfamide and mesna: response and toxicity at standard- and high-dose schedules. Semin Oncol Apr 1990;17(2 Suppl 4):68–73.Google ScholarPubMed
Frustaci, S, Gherlinzoni, F, Paoli, A. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 2001;19(5):1238–1247.CrossRefGoogle ScholarPubMed
Frustaci, S, Paoli, A, Bidoli, E. Ifosfamide in the adjuvant therapy of soft tissue sarcomas. Oncology 2003;65 Suppl 2:80–84.CrossRefGoogle Scholar
Grobmyer, SR, Maki, RG, Demetri, GD. Neo-adjuvant chemotherapy for primary high-grade extremity soft tissue sarcoma. Ann Oncol Nov 2004;15(11):1667–1672.CrossRefGoogle ScholarPubMed
Chang, HR, Hajdu, SI, Collin, C, Brennan, MF. The prognostic value of histologic subtypes in primary extremity liposarcoma. Cancer Oct 1 1989;64(7):1514–1520.3.0.CO;2-2>CrossRefGoogle ScholarPubMed
Gebhard, S, Coindre, JM, Michels, JJ. Pleomorphic liposarcoma: clinicopathologic, immunohistochemical, and follow-up analysis of 63 cases: a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol May 2002;26(5):601–616.CrossRefGoogle ScholarPubMed
Antonescu, CR, Tschernyavsky, SJ, Decuseara, R. Prognostic impact of P53 status, TLS-CHOP fusion transcript structure, and histological grade in myxoid liposarcoma: a molecular and clinicopathologic study of 82 cases. Clin Cancer Res Dec 2001;7(12):3977–3987.Google ScholarPubMed
McCormick, D, Mentzel, T, Beham, A, Fletcher, CD. Dedifferentiated liposarcoma: Clinicopathologic analysis of 32 cases suggesting a better prognostic subgroup among pleomorphic sarcomas. Am J Surg Pathol Dec 1994;18(12):1213–1223.CrossRefGoogle ScholarPubMed
Henricks, WH, Chu, YC, Goldblum, JR, Weiss, SW. Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. Am J Surg Pathol Mar 1997;21(3):271–281.CrossRefGoogle ScholarPubMed
Eilber, FC, Eilber, FR, Eckardt, J. The impact of chemotherapy on the survival of patients with high-grade primary extremity liposarcoma. Ann Surg Oct 2004;240(4):686–695; discussion 695–687.Google ScholarPubMed
Lewis, JJ, Antonescu, CR, Leung, DH. Synovial sarcoma: a multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity. J Clin Oncol May 2000;18(10):2087–2094.CrossRefGoogle ScholarPubMed
Eilber, FC, Brennan, MF, Eilber, FR. Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma. Ann Surg Jul 2007;246(1):105–113.CrossRefGoogle ScholarPubMed
Mentzel, T, Calonje, E, Wadden, C. Myxofibrosarcoma: Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. Am J Surg Pathol Apr 1996;20(4):391–405.CrossRefGoogle ScholarPubMed
Angervall, L, Kindblom, LG, Merck, C. Myxofibrosarcoma: A study of 30 cases. Acta Pathol Microbiol Scand [A] Mar 1977;85A(2):127–140.Google Scholar
Huang, HY, Lal, P, Qin, J, Brennan, MF, Antonescu, CR. Low-grade myxofibrosarcoma: a clinicopathologic analysis of 49 cases treated at a single institution with simultaneous assessment of the efficacy of 3-tier and 4-tier grading systems. Hum Pathol May 2004;35(5):612–621.CrossRefGoogle Scholar
Waters, B, Panicek, DM, Lefkowitz, RA. Low-grade myxofibrosarcoma: CT and MRI patterns in recurrent disease. AJR Am J Roentgenol Feb 2007;188(2):W193–198.CrossRefGoogle ScholarPubMed
Mundt, KA, Dell, LD, Austin, RP, Luippold, RS, Noess, R, Bigelow, C. Historical cohort study of 10 109 men in the North American vinyl chloride industry, 1942–72: update of cancer mortality to 31 December 1995. Occup Environ Med Nov 2000;57(11):774–781.CrossRefGoogle ScholarPubMed
Lee, FI, Smith, PM, Bennett, B, Williams, DM. Occupationally related angiosarcoma of the liver in the United Kingdom 1972–1994. Gut Aug 1996;39(2):312–318.CrossRefGoogle ScholarPubMed
Strobbe, LJ, Peterse, HL, Tinteren, H, Wijnmaalen, A, Rutgers, EJ. Angiosarcoma of the breast after conservation therapy for invasive cancer, the incidence and outcome. An unforseen sequela. Breast Cancer Res Treat Jan 1998;47(2): 101–109.CrossRefGoogle ScholarPubMed
Cha, C, Antonescu, CR, Quan, ML, Maru, S, Brennan, MF. Long-term results with resection of radiation-induced soft tissue sarcomas. Ann Surg Jun 2004;239(6):903–909; discussion 909–910.CrossRefGoogle ScholarPubMed
Stewart, FW, Treves, N. Classics in oncology: lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. CA Cancer J Clin Sep-Oct 1981;31(5):284–299.CrossRefGoogle ScholarPubMed
Naka, N, Ohsawa, M, Tomita, Y. Prognostic factors in angiosarcoma: a multivariate analysis of 55 cases. J Surg Oncol Mar 1996;61(3):170–176.3.0.CO;2-8>CrossRefGoogle ScholarPubMed
Skubitz, KM, Haddad, PA. Paclitaxel and pegylated-liposomal doxorubicin are both active in angiosarcoma. Cancer July 15 2005;104(2):361–366.CrossRefGoogle ScholarPubMed
Fata, F, O'Reilly, E, Ilson, D. Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer Nov 15 1999;86(10):2034–2037.3.0.CO;2-P>CrossRefGoogle ScholarPubMed
Vauthey, JN, Woodruff, JM, Brennan, MF. Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): a 10-year experience. Ann Surg Oncol Mar 1995;2(2):126–131.CrossRefGoogle ScholarPubMed
Grier, HE, Krailo, MD, Tarbell, NJ. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med Feb 20 2003;348(8):694–701.CrossRefGoogle ScholarPubMed
Linet, MS, Ries, , Smith, MA, Tarone, RE, Devesa, SS. Cancer surveillance series: recent trends in childhood cancer incidence and mortality in the United States. J Natl Cancer Inst Jun 16 1999;91(12):1051–1058.CrossRefGoogle ScholarPubMed
Paulino, AC, Okcu, MF. Rhabdomyosarcoma. Curr Probl Cancer Jan-Feb 2008;32(1):7–34.CrossRefGoogle ScholarPubMed
Lewis, JJ, Leung, D, Woodruff, JM, Brennan, MF. Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg 1998;228(3):355–365.CrossRefGoogle Scholar
Singer, S, Antonescu, CR, Riedel, E, Brennan, MF. Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma. Ann Surg Sep 2003;238(3):358–370; discussion 370–351.Google ScholarPubMed
Fabre-Guillevin, E, Coindre, JM, Somerhausen Nde, S, Bonichon, F, Stoeckle, E, Bui, NB. Retroperitoneal liposarcomas: follow-up analysis of dedifferentiation after clinicopathologic reexamination of 86 liposarcomas and malignant fibrous histiocytomas. Cancer Jun 15 2006;106(12):2725–2733.CrossRefGoogle ScholarPubMed
Karakousis, CP, Velez, AF, Gerstenbluth, R, Driscoll, DL. Resectability and survival in retroperitoneal sarcomas. Ann Surg Oncol Mar 1996;3(2):150–158.CrossRefGoogle ScholarPubMed
Tepper, JE, Suit, HD, Wood, WC, Proppe, KH, Harmon, D, McNulty, P. Radiation therapy of retroperitoneal soft tissue sarcomas. Int J Radiat Oncol Biol Phys Jun 1984;10(6):825–830.CrossRefGoogle ScholarPubMed
Sindelar, WF, Kinsella, TJ, Chen, PW. Intraoperative radiotherapy in retroperitoneal sarcomas. Final results of a prospective, randomized, clinical trial. Arch Surg Apr 1993;128(4):402–410.CrossRefGoogle ScholarPubMed
Pawlik, TM, Pisters, PW, Mikula, L. Long-term results of two prospective trials of preoperative external beam radiotherapy for localized intermediate- or high-grade retroperitoneal soft tissue sarcoma. Ann Surg Oncol Apr 2006;13(4):508–517.CrossRefGoogle ScholarPubMed
Dematteo, RP, Gold, JS, Saran, L. Tumor mitotic rate, size, and location independently predict recurrence after resection of primary gastrointestinal stromal tumor (GIST). Cancer Feb 1 2008;112(3):608–615.CrossRefGoogle Scholar
Raut, CP, DeMatteo, RP. Prognostic factors for primary GIST: prime time for personalized therapy?Ann Surg Oncol Jan 2008;15(1):4–6.CrossRefGoogle ScholarPubMed
Hirota, S, Isozaki, K, Moriyama, Y. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science Jan 23 1998;279(5350):577–580.CrossRefGoogle ScholarPubMed
Heinrich, MC, Corless, CL, Demetri, GD. Kinase mutations and imatinib response in patients with metastatic gastrointestinal stromal tumor. J Clin Oncol Dec 1 2003;21(23): 4342–4349.CrossRefGoogle ScholarPubMed
Antonescu, CR, Sommer, G, Sarran, L. Association of KIT exon 9 mutations with nongastric primary site and aggressive behavior: KIT mutation analysis and clinical correlates of 120 gastrointestinal stromal tumors. Clin Cancer Res Aug 15 2003;9(9):3329–3337.Google ScholarPubMed
Heinrich, MC, Corless, CL, Duensing, A. PDGFRA activating mutations in gastrointestinal stromal tumors. Science Jan 31 2003;299(5607):708–710.CrossRefGoogle ScholarPubMed
Dematteo, RP, Maki, RG. Adjuvant imatinib mesylate increases recurrence-free survival (RFS) in patients with completely localized primary gastrointestinal stromal tumor (GIST): North American Intergroup Phase III Trial ACOSOG Z9001. Proc Am Soc Clin Oncol 2007;Abtract No(10079).Google Scholar
Verweij, J, Casali, PG, Zalcberg, J. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet Sep 25-Oct 1 2004;364(9440):1127–1134.CrossRefGoogle ScholarPubMed
DeMatteo, RP, Maki, RG, Singer, S, Gonen, M, Brennan, MF, Antonescu, CR. Results of tyrosine kinase inhibitor therapy followed by surgical resection for metastatic gastrointestinal stromal tumor. Ann Surg Mar 2007;245(3):347–352.CrossRefGoogle ScholarPubMed

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