Elastofibroma dorsi; an uncommon and underdiagnosed tumour

Manuscript type : Case Report | Article Date : 2015/07/26

  • Autors

    1. Limaiem Faten
    2. Sahraoui Ghada
    3. Bouraoui Saadia
    4. Lahmar Ahlem
    5. Mzabi Sabeh
  • Abstract

    Elastofibroma dorsi is an infrequent benign, slow growing, soft tissue tumour that is usually located in the scapular zone. A 60-year-old male patient with no particular past medical history, presented with pain in the right scapular region of 8 months duration, which was exacerbated by physical activity. On examination, a hard and painless mass in the right subscapular region measuring approximately 7 cm in diameter was found. Computed tomography scan showed an unencapsulated mass with density similar to muscle, mixed with adipose tissue bands in the right infrascapular region. The patient underwent total excision of the tumour. Histologically, the tumour was poorly circumscribed and was composed of hypocellular fibrous collagenous strands admixed with a large number of coarse, densely eosinophilic elastic fibers and entrapped mature fat cells. The elastic structures sometimes formed discs or globules. These findings were consistent with elastofibroma. Postoperative course was uneventful, but the patient was lost to follow-up.
  • Description

    INTRODUCTION

    Elastofibromas are slow-growing soft tissue benign tumours originating from mesenchymal tissue. They typically occur in the subscapular or infrascapular re-gion at the inferior pole of the scapula and the serra-tus anterior muscle over the ribs of the thoracic cage.1- 3 These tumours are infrequent, representing 1-2% of all primary tumours of the chest wall.4 In this paper, the authors report a new case of elastofibroma. Their aim was to highlight the clinicopathological features of this rare entity.

    CASE REPORT

    A 60-year-old male patient with no particular past medical history, presented with pain in the right scapular region of 8 months duration, which was ex-acerbated by physical activity. Physical examination found a hard consistency, elastic and painless mass in the right subscapular region measuring approximate-ly 7 cm in diameter. Ultrasonography of the affected region reported a solid mass of the chest wall, which was below the serratus anterior. Computed tomogra-phy scan showed an unencapsulated mass with densi-ty similar to muscle, mixed with adipose tissue bands in the right infrascapular region. The patient under-went surgical resection of the tumour. Macroscopic examination showed a poorly defined fibroelastotic mass with a slightly rubbery, elastic consistence mea-suring 7 x 4 cm. The cut surface showed strands of grey-white and yellow tissue. Histologically, the tu-mour was poorly circumscribed and was composed of hypocellular fibrous collagenous strands admixed with a large number of coarse, densely eosinophilic elastic fibers and entrapped mature fat cells. The elas-tic structures sometimes formed discs or globules (Fig. 1). These findings were consistent with elastofi-broma. Postoperative course was uneventful, but the patient was lost to follow-up.

    DISCUSSION

    Elastofibroma was first described in 1961.5 The lit-erature reports an incidence of elastofibroma around 2%, although this increases in autopsy series, where it stands at 24% in females and 11% in males aged over 55 years.6,7 It is a benign connective tissue tumour, with a slow growth, known as elastofibroma dorsi due to its location in the inferior section of the scapula (in 99% of cases), deep within the anterior serratus and latissimus dorsi musculature and sometimes at-tached to the periosteum of the ribs.8 Although its etiology is still under debate, it is considered that the development of elastofibroma may be influenced by continued mechanical friction between the scapula and the ribs and even by a certain genetic component.[8] Elastofibroma appears predominantly in females between the fifth and seventh decades of life and is extremely rare in children.9 Although elastofibro-ma may be asymptomatic, it typically appears as a painful mass which can cause stiffness at the level of the shoulders, a scapular ‘‘snapping’’ or simulate a subacromial syndrome.8,9 Imaging studies are useful for diagnosis, with ultrasound showing an alternat-ing pattern of fasciculated type or laminar, hypo- and hyperechoic lines parallel to the chest wall and the Doppler signal is usually negative, demonstrating the absence of intrinsic vascularity.10 Computed tomog-raphy scan shows the alternation of adipose tissue and fibrous tissue with muscle density, not enhanced by contrast. MRI reported an alternating pattern of adipose and fibrous tissue and, in T1 and T2 sequenc-es, fibrous tissue shows signs of low intensity simi-lar to muscle. [10] Adipose tissue shows signs of high signal intensity on T1 and intermediate T2.10 Patients presenting tolerable symptoms should be treated through regular controls, reserving surgical excision for those cases where symptoms are severe and lim-iting. [11] Macroscopically, elastofibroma is usually ill defined and rubbery and exhibits grey-white fibrous tissue with interposing small areas of yellow fat. The mass varies from 2 cm up to 15 cm in diameter.[11] His-tologically, the tumour is composed of a mixture of paucicellular collagenous tissue and a large number of elastic fibers, associated with small amount of mu-coid stroma and entrapped mature fat cells. The elas-tic fibers are often large, coarse, deeply eosinophilic and fragmented into small, linearly arranged globules or serrated disks simulating beads on the strings.[11] The differential diagnosis of elastofibroma should be done with subcutaneous tumours or lesions such as lipomas, fibrolipomas, cystic formations or more aggressive tumours. The prognosis of elastofibroma after surgical excision is good, with an extremely low recurrence rate. Elastofibroma is usually considered as a connective tissue tumour, benign and slow-grow-ing. However, because its pathogenesis is still un-known, some authors consider it as a pseudotumour and others only define it as a lesion.9 Some authors suggest that there is degeneration of collagen fibres, while others advocate an abnormal fibrillogenesis process. [9,12] Furthermore, a genetic theory has been proposed since the chromosomal regions Xq12q22 and 19 were identified as possible containers of genes involved in certain tumours.13 This theory would ex-plain the familial predisposition observed in 32% of elastofibroma dorsi cases.13 In summary, elastofibroma dorsi is a rare ill-defined lesion of the soft tissues that most commonly affects old females. Its characteristic location and its specific aspect in imaging studies most often provide the di-agnosis following an incidental discovery. Neverthe-less, diagnosis is based mainly on histopathological examination of the surgical specimen.
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