best prognosis, and patients who undergo complete resection have the best long-term survival.
Due to the rarity of STSs, trials often have limited enrollment, and little progress has been
made with regards to treatment and survival
rates for metastatic and unresectable disease. All
patients should be evaluated and treated at specialized sarcoma centers. This case highlights
the need for continued research and clinical
trials to improve overall survival of patients with
1. American Cancer Society. Cancer facts and figures 2016. American Cancer Society Web site. www.cancer.org/acs/groups/
pdf. Accessed December 20, 2016.
2. National Comprehensive Cancer Network. NCCN clinical
guidelines in oncology: soft tissue sarcoma. 2016
3. Coindre J, Terrier P, Guillou L, et al. Predictive value of grade
for metastasis development in the main histologic types of
adult soft tissue sarcomas: a study of 1240 patients from the
French Federation of Cancer Centers Sarcoma Group. Cancer
4. Dei Tos A. Liposarcoma: new entities and evolving concepts.
Ann Diagn Pathol 2000;4:252–66.
5. Wile AG, Evans HL, Romsdahl MM. Leiomyosarcoma of soft
tissue: a clinicopathologic study. Cancer 1981;48:1022–32.
6. Hashimoto H, Daimaru Y, Tsuneyoshi M, Enjoji M. Leiomyosarcoma of the external soft tissues. A clinicopathologic, immunohistochemical, and electron microscopic study. Cancer
7. Fisher C. Synovial sarcoma. Ann Diagn Pathol 1998;2:401–21.
8. Newton WA Jr, Gehan EA, Webber BL, et al. Classification of
rhabdomyosarcomas and related sarcomas. Pathologic aspects
and proposal for a new classification--an Intergroup Rhabdomyosarcoma Study. Cancer 1995;76:1073–85.
9. Furlong MA. Pleomorphic rhabdomyosarcoma in adults: a
clinicopathologic study of 38 cases with emphasis on morphologic variants and recent skeletal muscle-specific markers. Mod
10. Anghileri M, Miceli R, Fiore M. Malignant peripheral nerve
sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer 2006;107:1065–74.
11. Miettinen M, Lasota J. Gastrointestinal stromal tumors–
definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows
Archive 2001;438: 1–12.
12. Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol
13. Young RJ, Brown NJ, Reed MW, et al. Angiosarcoma. Lancet
14. Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin
15. Penel N, Grosjean J, Robin YM, et al. Frequency of certain established risk factors in soft tissue sarcomas in adults: a prospective descriptive study of 658 cases. Sarcoma 2008;2008:459386.
16. Guillou L, Coindre JM, Bonichon F, et al. Comparative study
of the National Cancer Institute and French Federation of
Cancer Centers Sarcoma Group grading systems in a population of 410 adult patients with soft tissue sarcoma. J Clin Oncol
17. Maki RG, Moraco N, Antonescu CR, et al. Toward better soft
tissue sarcoma staging: building on American joint committee
on cancer staging systems versions 6 and 7. Ann Surg Oncol
18. Shiraki M, Enterline HT, Brooks JJ, et al. Pathologic analysis of
advanced adult soft tissue sarcomas, bone sarcomas, and mesotheliomas. The Eastern Cooperative Oncology Group (ECOG)
experience. Cancer 1989;64:484–90.
19. Presant CA, Russell WO, Alexander RW, Fu YS. Soft-tissue and
bone sarcoma histopathology peer review: The frequency of
disagreement in diagnosis and the need for second pathology
opinions. The Southeastern Cancer Study Group experience. J
Clin Oncol 1986; 4:1658–61.
20. Sundaram M, McLeod RA. MR imaging of tumor and tumor-like lesions of bone and soft tissue. AJR Am J Roentgenol
21. Ioannidis JP, Lau J. 18F-FDG PET for the diagnosis and grading
of soft-tissue sarcoma: a meta-analysis. J Nucl Med 2003;44:717–
22. Tateishi U, Yamaguchi U, Seki K, et al. Bone and soft-tissue sarcoma: preoperative staging with fluorine 18 fluoro-deoxyglucose PET/CT and conventional imaging. Radiology
23. Zagars GK, Ballo MT, Pisters PW, et al. Prognostic factors for patients with localized soft-tissue sarcoma treated with conservation surgery and radiation therapy: an analysis of 1225 patients.
24. Rosenberg S, 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;196:305–14.
25. Lewis J, Leung D, Woodruff J, et al. Retroperitoneal soft-tissue
sarcoma: analysis of 500 patients treated and followed at a
single institution. Ann Surg 1998;288:355–65.
26. Zagars GK, Ballo MT, Pisters PW, et al. Surgical margins and
reresection in the management of patients with soft tissue sarcoma using conservative surgery and radiation therapy. Cancer
27. Stojadinovic A, Leung DH, Hoos A. Analysis of the prognostic
significance of microscopic margins in 2,084 localized primary
adult soft tisusse sarcomas. Ann Surg 2002;235:424–34.
28. O’Sullivan B, Davis AM, Turcotte R, et al. Preoperative versus
postoperative radiotherapy in soft-tissue sarcoma of the limbs:
a randomized trial. Lancet 2002;359:2235–41.
29. 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
BOARD REVIEW QUESTIONS
Test your knowledge of this topic. Go to
click the Board Review Questions button.