Soft Tissue Sarcomas: Integrating Primary Care Recognition with Tertiary Care Center Treatment
Am Fam Physician. 1999 Aug 1;60(2):567-572.
Soft tissue sarcomas account for fewer than 1 percent of malignancies diagnosed annually in the United States. These tumors usually present as an asymptomatic mass. Any lesion larger than 5 cm in diameter should be considered suspicious. Radiographs should be obtained as the initial step in assessing a suspicious lesion. Magnetic resonance imaging has become the preferred diagnostic examination for tumors involving the extremities, and computed tomographic scanning may be the best technique for imaging lesions in the thoracic, abdominal, and head and neck areas. In general, the patient with a suspicious soft tissue mass located in a surgically difficult area should be referred to a regional center for biopsy and multidisciplinary consultation before resection is attempted. Careful preoperative planning is necessary for a good outcome. The prognosis for the patient with a soft tissue sarcoma is primarily determined by the grade, size and depth of the tumor and the presence of tumor at the surgical margins.
Although soft tissues constitute much of the human body, sarcomas account for fewer than 1 percent of all malignancies.1,2 In 1997, only 7,000 new cases of these tumors were diagnosed in the United States.2 Fewer than 20 percent of sarcomas are metastatic at the time of discovery, but the 4,300 deaths attributed to these masses in 1997 indicate how difficult they are to manage.3,4
Family physicians frequently encounter masses in their patients, but they will probably see only two soft tissue sarcomas over the course of a professional career. Preoperative diagnosis of these tumors improves the prognosis, and pretreatment planning is essential.5 The goal is to spare as much normal tissue as possible, with no local recurrence and no distant metastases. Limb- and tissue-sparing surgery and adjuvant radiation therapy have replaced amputation as the principal treatments for localized sarcomas.
Soft tissue sarcomas arise from mesenchymal and neural crest tissue in the extremities, chest wall, retroperitoneum and mediastinum. Children are most likely to present with rhabdomyosarcomas or poorly differentiated sarcomas of the head and neck, or genitourinary area. In adults, soft tissue sarcomas occur most often in late middle age. Adults tend to have sarcomas of the extremities, retroperitoneum or head and neck. Most soft tissue sarcomas arise de novo, but a small percentage originate in injured tissues such as scars or radiation-exposed areas.6 In the rare inherited Li-Fraumeni syndrome, germ-line p53 mutations result in breast cancer, sarcomas and other neoplasms.7 Neurofibrosarcomas develop in 10 percent of patients with von Recklinghausen's disease.8
Little information is available on the prevalence of sarcomas in primary care practice. Fewer than one in 100 soft tissue masses seen by family physicians are found to be malignant, and only about one in three masses seen in cancer centers actually prove to be malignant.9–11
The only published population-based study on sarcomas is from the Southern Swedish Health Care Region.12 In this study, the reported annual incidence of 18 sarcomas per 1 million masses evaluated translates into one sarcoma seen every 15 to 20 years by a family physician with a panel of 2,500 patients. High-grade malignant fibrous histiocytoma, the soft tissue sarcoma most commonly seen in the study, occurred at a median age of 64 years. Metastasis occurred within three years in 40 percent of the patients with sarcomas.
The World Health Organization recognizes 121 types of soft tissue sarcoma13 (Table 1).10,11 Liposarcoma and malignant fibrous histiocytoma are the most common histopathologic types affecting the extremities and head and neck areas, whereas leiomyosarcomas are more common in the retroperitoneum and viscera. Many soft tissue sarcomas contain clonal chromosomal aberrations and dysfunctional RB1 or p53 suppressor genes.4,14
Distribution of Diagnoses for Benign and Malignant Soft Tissue Tumors in a Large Referral Population*
Malignant tumors (N = 12,370)
Malignant fibrous histiocytoma
Benign tumors (N = 18,677)
With soft tissue sarcomas, the main determinants of prognosis are the grade, size and depth of the tumor, and the presence of tumor at the surgical margins3,15 (Table 2).9 Large high-grade tumors, characterized by poor cellular differentiation, are associated with a significantly higher incidence of local recurrence and distant metastasis16–18 (Table 3).15 Local spread tends to occur along fascial planes. Metastasis to the lung is common and is often the cause of death.4 Rhabdomyosarcomas, synovial sarcomas, and epithelioid and clear cell sarcomas of kidney frequently metastasize to lymph nodes.
Cancer Classification and Staging of Soft Tissue Sarcomas*
The rightsholder did not grant rights to reproduce this item in electronic media. For the missing item, see the original print version of this publication.
Characteristics of Soft Tissue Sarcoma
|Tumor characteristics and treatment||Metastasis-free five-year survival rate (%)|
Tumor size (greatest diameter)
Less than 5 cm
5 to 9 cm
10 cm or more
Head and neck
Low (well differentiated)
High (poorly differentiated)
Wide resection or amputation
Adapted with permission from Coindre JM, Terrier P, Bui NB, Bonichon F, Collin F, Le Doussal V, et al. Prognostic factors in adult patients with locally controlled soft tissue sarcoma. A study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol 1996;14:869–77.
Tumor size is an important determinant of survival. Patients with soft tissue sarcomas less than 5 cm in greatest diameter have a metastasis-free five-year survival rate of 81 percent15 regardless of tumor grade, depth and location, patient gender, surgical technique or use of adjuvant chemotherapy or radiation therapy.19 The five-year metastasis-free survival rate drops to 64 percent for tumors 5 to 9 cm in diameter and to 48 percent for tumors over 10 cm in diameter.15
Tumor depth is also a critical prognostic factor. If the tumor does not extend beyond the fascia, the five-year metastasis-free survival rate is 87 percent. In contrast, the five-year survival rate is 56 percent for deep tumors.15
A coordinated referral system can improve the prognosis for sarcomas. Regionalization of the Swedish health care system during the 1980s increased the likelihood that suspicious masses would be referred to cancer treatment centers. In these centers, patients with suspected sarcomas undergo imaging studies and needle biopsy before resection is attempted. This approach has lowered the incidence of local recurrence by two thirds. Swedish physicians repeatedly receive information to assist in identifying patients with suspicious tumors and accept that only one in every 10 referred patients will have malignant disease.12
Recognizing Soft Tissue Sarcomas
Soft tissue sarcomas usually present initially as an asymptomatic mass. Patients often wait an average of four months before seeking medical attention, and a definitive diagnosis may be delayed another six months in 20 percent of patients.20
Unfortunately, no one feature can reliably indicate if a mass is a sarcoma or whether it is a benign or malignant mass. Two thirds of sarcomas are deep seated and larger than most subcutaneous tumors.21 Compression of a nerve or blood vessel or interference with movement can cause symptoms. A vague but unrelated history of trauma often adds to the confusion.22 The physical examination reveals a firm, nontender mass that may seem well defined as a result of compression by surrounding tissue.
Radiographs should be obtained as the first step in assessing a suspicious lesion, especially one that is over 5 cm in diameter. The radiologist should ascertain if the lesion arises from bone or is associated with a bony deformity. Calcifications are usually associated with benign disease, but they may also be present in liposarcomas or large malignancies with necrotic areas.23
Magnetic resonance imaging (MRI) has emerged as the preferred diagnostic modality for further study of extremity tumors after radiographs have been obtained (Figures 1 and 2). Compared with MRI studies, computed tomographic (CT) scans may provide more information about thoracic, head and neck, and abdominal tumors (Figure 3). MRI and CT scans differentiate benign from malignant lesions in 85 percent of patients.23–26 Ultrasonography can differentiate solid and cystic tumors, but it may confuse necrotic tumors with benign cysts.23,27
A complete physical examination is indicated in all patients with suspicious masses. Most patients should undergo chest radiography and possibly CT scanning of the chest before biopsy samples are obtained to search for possible metastatic disease. Because of the high rate of residual tumor after unplanned excisions, a strategy of “excise and wait for the pathology report” should be avoided unless a mass is less than 5 cm in diameter and imaging studies indicate that the lesion is benign.5,28
Biopsy samples should always be obtained from suspicious masses. Where the biopsy procedure is performed—and by whom—is an important primary care decision. Sarcomas are rare, their histologic interpretation can be difficult, and biopsy procedures often procure only small amounts of tissue for study.29 The error rate for biopsies performed by orthopedic surgeons in community hospitals has been higher than the error rate for biopsy and interpretation procedures performed at cancer centers.30
Core-needle biopsy is the method of choice. Although fine-needle aspiration biopsy is somewhat easier to perform, it provides an inadequate specimen 18 percent of the time.31,32 In contrast, core-needle biopsy provides an adequate sample 93 percent of the time and correct differentiation of malignant and benign lesions more than 90 percent of the time. In addition, core-needle samples usually provide enough material to determine tumor grade.31,33
With soft tissue sarcomas, the initial surgical procedure is extremely important. Both high- and low-grade tumors less than 5 cm in diameter may be treated by wide local excision (margins of at least 1 cm). The surgical margins must be carefully examined for residual tumor. Even at referral centers, 7 percent of patients require a second resection to achieve tumor-free margins.20
Outcomes for wide-margin excision of tumors less than 5 cm in diameter are equivalent whether or not adjuvant radiation therapy is employed. In contrast, high-grade tumors larger than 5 cm in diameter require wide surgical excision plus radiation therapy administered either preoperatively or postoperatively.3,20 Large tumors close to vascular, bony or nerve structures may be resected more successfully when radiation therapy is used preoperatively. Although radiation therapy decreases local recurrence dramatically, it may not affect survival because high-grade tumors tend to metastasize early.34
The role of chemotherapy in the treatment of soft tissue sarcomas remains unclear. Nonetheless, patients with high-risk tumors should be considered for entry into chemotherapy trials.15
Unfortunately, the 20 percent five-year survival rate for patients with lung metastases has yet to be improved.20
When to Refer
Because the initial surgical procedure is so important, the family physician creates greater opportunity for the patient when a soft tissue sarcoma is diagnosed before treatment. Referral to a cancer treatment center and an experienced cancer surgeon should be considered for any patient with a suspicious mass.
Preoperative diagnosis potentiates proper staging and the use of a multidisciplinary team (medical and surgical oncologists, radiation therapists, physiatrists and pathologists) to plan the best treatment. A “low suspicion” mass in a surgically accessible area requires less advance planning than a suspicious tumor in a technically difficult location. High-grade lesions require thoughtful management, regardless of location.
Treatment goals, including local tumor control, preservation of function and avoidance of amputation, should be discussed with the patient.
1. Chang AE, Sondak VK. Clinical evaluation and treatment of soft tissue tumors. In: Enzinger FM, Weiss SW, eds. Soft tissue tumors. 3d ed. St. Louis: Mosby, 1995:17–38.
2. Cancer facts and figures–1998. Atlanta: American Cancer Society, 1998.
3. Valle AA, Kraybill WG. Management of soft tissue sarcomas of the extremity in adults. J Surg Oncol. 1996;63:271–9.
4. Kampe CE, Eilber FR. Soft tissue sarcomas. In: Haskell CM, ed. Cancer treatment. 4th ed. Philadelphia: Saunders, 1995.
5. Noria S, Davis A, Kandel R, Levesque J, O'Sullivan B, Wunder J, et al. Residual disease following unplanned excision of a soft-tissue sarcoma of an extremity. J Bone Joint Surg [Am]. 1996;78:650–5.
6. Rosenthal TC. Localized fibrous mesothelioma in the postpartum period. J Fam Pract. 1987;25:174–5.
7. Malkin D, Li FP, Strong LC, Fraumeni JF Jr, Nelson CE, Kim DH, et al. Germ line p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms. Science. 1990;250:1233–8.
8. Lawrence W Jr. Soft tissue sarcomas in adults and children: a comparison [Editorial]. CA Cancer J Clin. 1994;44:197–9.
9. General considerations. In: Enzinger FM, Weiss SW, eds. Soft tissue tumors. 3d ed. St. Louis: Mosby, 1995:1–16.
10. Kransdorf MJ. Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location. AJR Am J Roentgenol. 1995;164:129–34.
11. Kransdorf MJ. Benign soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location. AJR Am J Roentgenol. 1995;164:395–402.
12. Gustafson P. Soft tissue sarcoma. Epidemiology and prognosis in 508 patients. Acta Orthop Scand Suppl. 1994;259:1–31.
13. Enzinger FM. Histological typing of soft tissue tumors. Geneva: World Health Organization, 1969.
14. Kawai A, Woodruff J, Healey JH, Brennan MF, Antonescu CR, Ladanyi M. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med. 1998;338:153–60.
15. Coindre JM, Terrier P, Bui NB, Bonichon F, Collin F, Le Doussal V, et al. Prognostic factors in adult patients with locally controlled soft tissue sarcoma: a study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol. 1996;14:869–77.
16. Brennan MF. Management of extremity soft-tissue sarcoma. Am J Surg. 1989;158:71–8.
17. Li XQ, Parkekh SG, Rosenberg AE, Mankin HJ. Assessing prognosis for high-grade soft-tissue sarcomas: search for a marker. Ann Surg Oncol. 1996;3:550–7.
18. Collin C, Godbold J, Hafdu S, Brennan M. Localized extremity soft tissue sarcoma: an analysis of factors affecting survival. J Clin Oncol. 1987;5:601–12.
19. Greer RJ, Woodruff J, Casper ES, Brennan MF. Management of small soft-tissue sarcoma of the extremity in adults. Arch Surg. 1992;127:1285–9.
20. 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. 1987;205:349–59.
21. Rydholm A, Gustafson P, Rooser B, Willen H, Berg NO. Subcutaneous sarcoma. A population-based study of 129 patients. J Bone Joint Surg [Br]. 1991;73:662–7.
22. McLeay GF, Steward WP. High grade soft tissue sarcoma. Ann Oncol. 1993;4:509–14.
23. Kransdorf MJ, Jelinek JS, Moser RP Jr. Imaging of soft tissue tumors. Radiol Clin North Am. 1993;31:359–72.
24. Hanna SL, Fletcher BD. MR imaging of malignant soft-tissue tumors. Magn Reson Imaging Clin N Am. 1995;3:629–50.
25. Soler R, Castro JM, Rodriguez E. Value of MR findings in predicting the nature of the soft tissue lesions: benign, malignant or undetermined lesion? Comput Med Imaging Graph. 1996;20:163–9.
26. Bloem JL, van der Woude HJ, Geirnaerdt M, Hogendoorn PC, Taminiau AH, Hermans J. Does magnetic resonance imaging make a difference for patients with musculoskeletal sarcoma? Br J Radiol. 1997;70:327–37.
27. Hoglund M, Muren C, Brattstrom G. A statistical model for ultrasound diagnosis of soft-tissue tumours in the hand and forearm. Acta Radiol. 1997;38:355–8.
28. Singer S, Antman K, Corson JM, Eberlein TJ. Long-term salvageability for patients with locally recurrent soft-tissue sarcomas. Arch Surg. 1992;127:548–54.
29. Mankin HJ, Mankin CJ, Simon MA. The hazards of the biopsy, revisited. J Bone Joint Surg [Am]. 1996;78:656–63.
30. Tanabe KK, Pollock RE, Ellis LM, Murphy A, Sherman N, Romsdahl MM. Influence of surgical margins on outcome in patients with preoperatively irradiated extremity soft tissue sarcomas. Cancer. 1994;73:1652–9.
31. Barth RJ Jr, Merino MJ, Solomon D, Yang JC, Baker AR. A prospective study of the value of core needle biopsy and fine needle aspiration in the diagnosis of soft tissue masses. Surgery. 1992;112:536–43.
32. Epstein HD. Fine-needle aspiration of soft tissue lesions. Pathology [Phila]. 1996;4:463–92.
33. Heslin MJ, Lewis JJ, Woodruff JM, Brennan MF. Core needle biopsy for diagnosis of extremity soft tissue sarcoma. Ann Surg Oncol. 1997;4:425–31.
34. Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, 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.
Copyright © 1999 by the American Academy of Family Physicians.
This content is owned by the AAFP. A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference. This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP. Contact firstname.lastname@example.org for copyright questions and/or permission requests.