Diagnosis and treatment of soft-tissue sarcomas of the extremities and trunk

Abstract

The relatively low incidence and often atypical clinical presentation of soft-tissue sarcomas (STS) impedes early and adequate diagnosis. Patients may report on recently enlarged soft-tissue swellings, infrequently complain of painful lesions, or even have no symptoms at all.A thorough diagnostic work-up is essential in order to distinguish between benign soft-tissue tumours and STSs. Patient history, clinical features and radiological findings all help in assessing the underlying pathology. 'Worrying' features such as recent increase in size, deep location relative to the fascia, a tumour exceeding 4 cm in size, and invasive growth patterns seen on imaging should prompt verification by biopsy.Even though acquisition of biopsy material may be incomplete, one should bear in mind some essential rules. Regardless of the biopsy technique applied, the most direct route to the lump in question should be identified, contamination of adjacent structures should be avoided and a sufficient amount of tissue acquired.Treatment of STS is best planned by a multidisciplinary team, involving experts from various medical specialities. The benchmark therapy consists of en bloc resection of the tumour, covered by a safety margin of healthy tissue. Depending on tumour histology, grade, local extent and anatomical stage, radiotherapy, chemotherapy and isolated hyperthermic limb perfusion may be employed.Due to the complexity of treatment, any soft-tissue swelling suspected of malignancy is best referred directly to a sarcoma centre, where therapeutic management is carefully planned by an experienced multidisciplinary team. Cite this article: EFORT Open Rev 2017;2:421-431. DOI: 10.1302/2058-5241.2.170005.

Keywords: diagnostic pathway; soft-tissue sarcoma; therapeutic management.

Fig. 1

Large, ulcerated tumour arising from a 30-year-old female patient’s right calf, later confirmed as high-grade spindle cell sarcoma.

Fig. 2

Radiograph of the right hip of a 70-year-old female patient with a high-grade leiomyosarcoma showing moderate soft-tissue opacity (a). MRI scans of the same patient’s leg, displaying a 25 cm × 11 cm × 9 cm partially-necrotic tumour with heterogeneous pathological contrast enhancement (b, c).

Fig. 3

Ulcerating, partially-necrotic tumour on the dorsum of the left foot of a 53-year-old male patient (a). Radiograph shows displacement of the 4th and 5th digit by the mass (b). MRI reveals the actual extent of the tumour, later confirmed as high-grade synovial sarcoma (c).

Fig. 4

Referral algorithm for soft-tissue lumps, as recommended by our department (STS, soft-tissue sarcoma).

Fig. 5

Unplanned excision of a later histologically-verified alveolar soft-part sarcoma located in the left thigh of a 15-year-old female patient. Note the drain’s exit point remote from the surgical wound.

Fig. 6

Inappropriate resection of a tumour at the wrist of an 80-year-old female patient, thought to be a ganglion. Histology revealed a high-grade angiosarcoma. Consequently, a forearm amputation became necessary.

Fig. 7

Wide resection of a high-grade undifferentiated pleomorphic sarcoma arising in the dorsal aspect of the left thigh of a 50-year-old male patient (a). The sciatic nerve was dissected off the tumour and could be spared during en bloc resection (b).