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Elastofibroma is a rarely diagnosed benign fibroproliferative lesion which occurs most commonly in the periscapular region of middle aged to elderly women.1 Recognition of the lesion is important as the differential diagnosis includes other benign and also malignant tumours. We report a case of elastofibroma in a patient who presented with shoulder pain to a rheumatology clinic, and review previous publications. Although elastofibroma is uncommon, it has received attention in radiological and orthopaedic publications but not in rheumatology published reports.
A 43 year old Turkish woman, previously fit and healthy, was referred to our outpatient clinic with a two year history of right shoulder pain. The pain was described as a dull ache of gradual onset, around the posterior aspect of the shoulder over the scapula, which was worse on movement of the arm. There was no weakness. Over the preceding four months the patient had noticed a swelling below the inferior angle of the right scapula which would appear and disappear with movement of the arm. The patient had no other medical history or relevant family history.
On examination there was a full range of movement of both shoulders and neck with no wasting or neurological signs. Pain was reproduced around the right shoulder when the arm was circumducted. In this position a firm, poorly circumscribed, and minimally mobile mass of 5×5 cm was apparent underlying the inferior angle of the scapula. The rest of the examination was normal.
Initial investigations showed a normal full blood count, bone profile, and inflammatory markers, and a normal radiograph of the right shoulder and scapula. Subsequent magnetic resonance imaging (MRI) showed a poorly circumscribed heterogeneous soft tissue mass between the chest wall and the scapula (fig 1). The signal intensity was similar to that of adjacent muscles with interspersed strands of high signals similar to those of fat. No significant contrast enhancement was seen. The lesion was biopsied under computed tomography guidance and a histological examination showed elastic fibres within a collagenous fibrous tissue with entrapped adipose tissue, consistent with a diagnosis of elastofibroma. Surgical excision was performed because the mass was causing pain. Postoperative histology confirmed an elastofibroma. The patient has remained asymptomatic after surgery with no recurrence of the mass.
Elastofibroma dorsi, first described in 1961,2 is a benign, slow growing, mesenchymal soft tissue lesion.3 They usually occur in active subjects above the age of 50 with a male:female ratio of 1:5.4 Most (99%) occur in the subscapular region, usually on the right side. The lesions have occasionally been found in the extremities, head, abdominal and thoracic cavities.5 Of those in the subscapular region approximately 10% are bilateral.5 The cause and pathogenesis are unclear, but it is suspected that subclinical microtrauma may lead to reactive hyperplasia of elastic fibres with consequently increased production of fibrous tissue.6 Clinically, over 50% of subjects are asymptomatic and may present with a painless swelling; approximately 25% present with a clicking sensation when the arm is moved, while fewer than 10% present with pain.7
Plain radiographs may be normal or may show soft tissue density in the periscapular region when the scapula is raised.5 Computed tomography usually shows a heterogeneous soft tissue mass with poorly defined margins.5 MRI is the best non-invasive technique and most useful for diagnosis. Elastofibromas appear as poorly circumscribed soft tissue lesions with similar signal intensity to that of skeletal muscle but interspersed with high signal intensity areas representing adipose strands.8 The differential diagnosis includes desmoid tumours, neurofibroma, and liposarcoma. However, these tumours usually show strong enhancement after gadolinium injection. Usually faint enhancement is seen with elastofibromas, although marked enhancement, mimicking malignant tumour, has been occasionally reported.9 Biopsy should therefore be undertaken as the confirmatory procedure and to exclude sarcoma.
In cases where the patient is asymptomatic excision is unnecessary. Malignant transformation is unknown. In symptomatic cases local excision is the best treatment.10 Recurrence has not been reported.
We conclude that elastofibroma should be considered in the differential diagnosis of subscapular pain. Although an uncommon lesion with a variable clinical presentation, the site and MRI appearances are characteristic. Awareness of the benign nature avoids unnecessary surgery and reassures a symptomatic patient.
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