Soft tissue tumours and other diseases

Published on 11/03/2015 by admin

Filed under Orthopaedics

Last modified 22/04/2025

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10 Soft tissue tumours and other diseases

Co-written by Nigel Raby

SWELLINGS AND TUMOURS OF SOFT TISSUE

Soft tissue swellings are a common presentation to primary care physicians and a cause of great anxiety to patients when they discover a lump in their limb or trunk which they interpret as a cancer. In fact malignant tumours are very rare and benign tumours are much more common in a ratio of more than 100:1. These tumours frequently cause problems in diagnosis and treatment because of the difficulty in differentiating them from other, much commoner, causes of lumps and swellings in the limbs. These include cysts and ganglia around joints, normal muscle variants, muscle rupture, haematoma, vascular aneurysm, and myositis ossificans.

Myositis ossificans (heterotopic ossification)

This is an uncommon but troublesome lesion because of its apparent malignant behaviour. It presents as a painful and tender lump in a limb muscle, with or without a history of recent trauma to the site. Imaging is crucial to obtain the correct diagnosis and MRI and ultrasound are the most useful in the very early stages as they can detect the presence of calcification in the lesion which is the key to diagnosis (Fig. 10.3A). Later imaging with CT scanning will reveal a diagnostic rim of ossification at the periphery of the well-circumscribed lesion. Plain radiographs after several weeks will show some calcification in the muscle which should indicate the correct diagnosis (Fig. 10.3B). However, in some patients where the early changes are not recognised these appearances may be misinterpreted and may lead to a presumed diagnosis of soft tissue osteosarcoma. This assumption may be reinforced if a biopsy is performed, as this will show a very active lesion containing immature mesenchymal cells with atypical nuclei and osteoid formation. No active treatment is required, other than careful observation, as the lesion will resolve in 2–3 months.

TUMOURS OF SOFT TISSUE

The soft-tissue tumours that are met with in orthopaedic practice arise from the connective tissues or blood vessels of the limbs or trunk. They may be benign or malignant. They usually present clinically as painless soft-tissue lumps, and cause symptoms only when quite large.

BENIGN TUMOURS OF SOFT TISSUE

Benign peripheral nerve sheath tumours

Tumours arising from nerves are of two types: schwannomas and neurofibromas.

MALIGNANT TUMOURS OF SOFT TISSUE

Malignant tumours of soft tissue (soft-tissue sarcomas) are uncommon, comprising 1% of adult malignant neoplasms. Of mesenchymal origin, they arise from connective tissues such as fascia, aponeurosis, tendon sheath, intermuscular septa, voluntary muscle, and synovial membrane. Such a tumour presents difficulties both in diagnosis and in treatment. It may be hard to distinguish from its benign counterpart, presenting as a progressively enlarging but usually painless swelling. It is of firm consistency and may appear to be well localised, though this is usually a false impression. It may spread widely within the soft tissues and the surrounding pseudocapsule forms no barrier. Metastasis occurs through the blood stream, mainly to the lungs.

Investigation of these tumours is aided by magnetic resonance imaging (MRI). MRI scans provide good definition of the anatomical extent of the tumour and of its relationship to the neurovascular structures (Fig. 10.7), thereby allowing correct planning of surgical treatment. However the scans cannot reliably differentiate between benign and malignant lesions, which must ultimately depend on expert histological examination from a representative biopsy.

Biopsy must be planned in such a way as to minimise the spillage of malignant cells. It may be by needle aspiration or localised incision, but never by local excision of the tumour (excision biopsy).

The results of the surgical treatment of most types of soft-tissue sarcoma have been improved by adjuvant therapy with radiation or potent chemotherapeutic agents used in combination as for bone sarcomas. Radiotherapy may be used after operative excision, to reduce the risk of local recurrence, but does not remove the need for adequate surgical treatment, which demands excision with wide margins of healthy tissue rather than simple local excision or enucleation.

OTHER SOFT TISSUE DISEASES

Soft tissues constitute a much larger part of the locomotor system than the bony skeleton, but the disorders that affect them receive much less attention from orthopaedic surgeons. In the past they were regarded as the province of the rheumatologist or sports medicine specialist and attracted little surgical interest. This is now changing with the introduction of more sophisticated investigations, particularly laboratory tests utilising molecular biology techniques and improved imaging with ultrasound and MRI. Today’s surgeons require a greater understanding of the diseases that affect muscles, tendons, ligaments, and connective tissue to enable them to work as part of the multi-disciplinary team needed for their management.

INFLAMMATORY LESIONS OF SOFT TISSUE

Irritative (frictional) tenosynovitis and peritendinitis

This is caused by excessive friction from over-use. The synovial sheath is mildly inflamed and there is an exudate of watery fluid within it, visible on ultrasound scanning (Fig. 10.9). A similar traumatic inflammation may affect the flimsy paratenon surrounding those tendons that are devoid of synovial sheaths. This is termed paratendinitis and is a common problem around the wrist and hand. The controversial condition known as repetitive stress syndrome comes into this category (p. 327).

POLYMYALGIA RHEUMATICA

This connective tissue disorder has been recognised with increased frequency in older patients presenting with pain and stiffness in the muscles of the neck and shoulder girdle, or sometimes the low back and buttock regions. Because of these features it may be confused with degenerative disc disease, though characteristically the associated symptoms of malaise, low-grade fever, night sweats, and loss of weight should lead to the diagnosis. It tends to be commoner in women than in men, usually affecting patients over the age of 60 years, and in about one-third of cases it is associated with the condition of giant-cell arteritis. This is a more serious disease as it may lead to sudden blindness because of involvement of the cranial arteries.

The clinical features are ill-defined muscle pain around the shoulders or low back, with associated stiffness, and the characteristic features of systemic disturbance. When giant-cell arteritis is also present there may be marked tenderness over the temporal arteries. There may be marked elevation of the erythrocyte sedimentation rate, but the rheumatoid factor is negative. When associated giant-cell arteritis is suspected on clinical grounds, urgent administration of prednisolone in high doses is advisable pending confirmation of the diagnosis by biopsy, in order to reduce the risk of blindness. Both conditions show a good response to steroid therapy, usually in a dose of 15 mg of prednisolone daily. Once the clinical features have subsided this may be reduced to a maintenance dose of 5 mg daily.

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