Bilateral Elastofibroma Dorsi in a Symptomatic Male Patient: Is Surgical Resection Justified? A Case Report

Abstract

Elastofibroma dorsi (ED) is a rare, benign, slowly growing soft tissue neoplasm characterized by abnormal elastic fiber proliferation. There is a marked female predominance; bilateral presentation occurs in only 10% of cases. ED is usually located in the infra-scapular area, adherent to the posterior chest wall and ribs. Although many theories have been proposed regarding its pathogenesis, the exact cause remains uncertain. Preoperative diagnosis is established based on clinical picture and radiological imaging. We present a case of a 49-year-old man with bilateral ED who complained of back pain and limited shoulder movement, and who underwent simultaneous bilateral surgical removal; histopathological examination confirmed the diagnosis.

Case report

Elastofibroma dorsi (ED) is a benign, non-capsulated, slowly growing mesenchymal soft tissue tumor of the chest wall, characterized by abundant elastin and fibrous tissue proliferation [1]. ED has a marked female predominance—with a ratio as high as 13:1—and is usually unilateral; bilateral involvement occurs in only 10% of patients [2,3]. ED typically arises in the infra- or sub-scapular area, adherent to the posterior chest wall and ribs. Other reported locations include the infra-olecranon region, ischial tuberosity, greater trochanter, breast, deltoid muscle, and foot [1-4]. Surgical excision is recommended, particularly for symptomatic individuals, for lesions with a maximum diameter of ≥5 cm, or when malignancy is suspected [4].

We present a 49-year-old man, a porter by profession, who complained of chest back pain that worsened with shoulder movement and of bilateral visible swellings for three years. On examination, firm, non-tender bilateral infra-scapular swellings were noted, accompanied by a clicking sensation during shoulder movement, with the right mass being larger than the left. Chest computed tomography (CT) and magnetic resonance imaging (MRI) revealed a right heterogeneous, posterolateral, oval soft tissue mass located beneath the latissimus dorsi and serratus anterior muscles, just inferior to the scapula, measuring 14×10×4 cm; a second mass in the left region measured 9×8×3.5 cm (Fig. 1). Laboratory data were within normal limits. As the diagnosis was established based on clinical and radiological findings, bilateral surgical removal under general anesthesia was planned. The patient was placed in the lateral decubitus position, and a 5-cm transverse incision was made over the lesion, 2 cm below the inferior angle of the scapula, with his arms positioned away to allow optimal exposure. After dividing the latissimus dorsi and serratus anterior muscles, the mass was found to be firm, circumscribed, nonencapsulated, and adherent to the underlying 6th–9th ribs, the inferior angle of the scapula, and the surrounding muscle. The lesion extended 4 cm beneath the scapula and was fixed to the chest wall. Complete macroscopic surgical removal was performed using both sharp and blunt dissection, starting on the right side; the patient was then repositioned to facilitate excision of the left-sided mass (Fig. 2). Two tubular suction drains were placed on each side, and both incisions were closed in layers with interrupted sutures. A compression bandage was applied bilaterally and maintained for 7 days. The postoperative period was uneventful, and the patient was discharged after 8 days with advice to avoid heavy activities. Histopathological analysis revealed a mesenchymal lesion composed of intertwining bands of eosinophilic collagen and abnormal elastic fibers arranged in irregularly shaped areas separated by mature fat cells. The diagnosis of ED was confirmed for both lesions. The patient was followed for 18 months, with no signs of local recurrence or seroma.

Fig. 1.

Chest computed tomography (A) and magnetic resonance imaging (B) demonstrating bilateral elastofibroma in close relation to the scapula and ribs. Written informed consent for the publication of this image was obtained from the patient.

Fig. 2.

Images depicting both swellings before and after complete surgical excision. (A) Bilateral infra-scapular swellings (preoperatively). (B) Gross specimen of the excised right elastofibroma. (C) Gross specimen of the excised left elastofibroma. (D) Incisions 15 months postoperatively. Written informed consent for the publication of this image was obtained from the patient.

The patient provided written informed consent for the publication of the research details and clinical images.

Discussion

ED is an uncommon, benign, slowly growing soft tissue neoplasm that produces abnormal elastic fibers; it was first described in 1961 [5]. The World Health Organization classifies ED as a benign fibroblast-myofibroblast tumor [6]. The most common location for ED is the area related to the inferior pole of the scapula; other reported sites include the infra-olecranon region, ischial tuberosity, greater trochanter, breast, deltoid muscle, and foot [1-4]. Typically, ED occurs unilaterally in middle-aged individuals, with a marked female predominance (ratio of 13:1), and bilateral incidence is low (10%) [2,3]. Our patient is a male who presented with bilateral painful swellings and limited shoulder movement.

There is no established consensus regarding the primary etiologic factor. Initially, it was believed that the pathogenesis was triggered by repeated friction between the inferior angle of the scapula and the chest wall, leading to increased fibrous tissue production [1,3]. Our patient’s occupation involved heavy manual work, which may lead to repeated minor friction between the scapula and chest wall, potentially contributing to the development of ED. However, occurrences in other anatomical locations support alternative theories, including systemic involvement as well as genetic or enzymatic abnormalities [4,7]. Akçam et al. [7] reported 3 sisters with bilateral ED, suggesting that genetic predisposition may play a role in its pathogenesis.

Regarding clinical presentation, patients typically present with a back swelling accompanied by mild pain or a clicking sensation during arm movement; however, 50% of patients may be asymptomatic [1]. ED appears as a swelling just inferior to the scapula and is best palpated when the arm is elevated. ED grows slowly, with its size varying and reaching up to 20 cm [2]. The radiological assessment includes chest X-ray, ultrasonography, CT, and MRI. ED may be incidentally discovered on imaging in asymptomatic patients; therefore, thorough physical examination and appropriate imaging studies are vital. Preoperative biopsy is not required; clinical and radiological evaluations are usually sufficient for diagnosing ED [7]. On ultrasound, ED typically appears as a hypoechoic lesion with linear echogenic strands, resembling muscle, and an indistinct cleavage plane [8]. Chest CT demonstrates a bilateral heterogeneous mass involving the thoracic muscles and ribs, with a density similar to that of muscle, making it difficult to distinguish the lesion’s borders from the surrounding tissue. MRI is the best non-invasive diagnostic modality for ED, and the lesion is well visualized on both T1- and T2-weighted images [7-9]. The diagnosis of ED can be confidently established based on its characteristic chest MRI findings; however, differential diagnosis with other soft tissue masses must be considered. Fine needle biopsy is not recommended due to the tumor’s hypocellular nature; instead, open biopsy or core needle biopsy is required to obtain sufficient tissue for diagnosis [9].

Complete surgical excision of the mass serves both diagnostic and therapeutic purposes. Surgical excision is recommended for symptomatic patients when ED causes pain, limitation of motion, compression symptoms, cosmetic disfigurement, or if the lesion size is ≥5 cm [1-4]. Postoperative seroma and hematoma formation are common complications, as they tend to accumulate in the dead space left after ED removal. Several methods have been reported to prevent postoperative seroma, including the application of a compressive bandage, limiting shoulder movements, and avoiding heavy activities. Most postoperative seromas can be managed conservatively, while fine needle aspiration is reserved for larger seromas [10]. Marginal excision of the mass has been recommended, as it is associated with a low risk of local recurrence, and no cases of malignant transformation or metastasis have been reported [1-4]. Our patient was followed for 18 months, with no signs of local recurrence or seroma.

In conclusion, ED is a rare, benign, slowly growing neoplasm located deep to the inferior scapular angle that should be differentiated from other soft-tissue tumors. ED is usually diagnosed based on its characteristic clinical and radiological findings. Surgical intervention is recommended for symptomatic patients, and careful wound management with suction drainage and a compressive bandage is essential to reduce the incidence of postoperative seroma.

Funding Statement

Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.