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AUTHOR AND EDITOR INFORMATION

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Author: Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School

Robert A Schwartz is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Coauthor(s): Sonja Stander, MD, Staff Physician, Department of Dermatology, University-Hospital Muenster, Germany; Thomas Schwarz, MD, Vice Chairman, Professor, Department of Dermatology, University of Kiel, Germany

Editors: Kathryn Schwarzenberger, MD, Associate Professor of Medicine, Division of Dermatology, University of Vermont College of Medicine; Consulting Staff, Division of Dermatology, Fletcher Allen Health Care; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center

Author and Editor Disclosure

Synonyms and related keywords: elastofibroma dorsi, connective-tissue tumor, connective tissue tumor, elastogenesis

INTRODUCTION

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Background

Elastofibroma is a rare, benign, slow-growing connective-tissue tumor that occurs most often in the subscapular area in elderly women. Jarvi and Saxen1 first described this rare entity in 1961. It is characterized by accumulated abnormal elastic fibers and is generally regarded as a reactive process, an unusual fibroblastic pseudotumor.2

Pathophysiology

The etiology of this tumor remains unclear, although prevalence is increased in persons who perform manual labor involving the shoulder girdle. Thus, repeated trauma due to mechanical friction of the scapula against the ribs has been suggested to induce this process. This theory provides an explanation for the right-sided preponderance; however, in up to 66% of cases, the tumor is bilateral. Rarely, elastofibromas are multiple in the same individual.3 In up to one third of cases, the patient has a family history of the tumor, suggesting a nontraumatic genetic origin.

In a 2002 study, chromosomal gains have been speculated as a cause for the development of elastofibromas. Nishio et al4 detected DNA copy number changes involving 1 or 2 chromosomes in 33% of 27 patients. The most common recurrent gains were at bands Xq12-q22 and 19. High-level amplifications and recurrent losses were not observed. No correlation was found between DNA copy number changes and elastofibroma size. The authors concluded that these chromosomal regions may contain genes involved in the development of at least some elastofibromas.

Coexistence of an elastofibroma with a high-grade spindle cell sarcoma5 and a high-grade leiomyosarcoma6 has been reported. Moreover, because a number of other entities, including lipomas, metastases, sarcomas, and extra-abdominal fibromatoses and hemangiomas, may occur on the back and in the subscapular site, the diagnosis must be confirmed with biopsy.

Cytogenetic chromosomal instability and some recurrent or clonal chromosomal changes have raised the possibility that the lesion represents a neoplastic process.2 Recent findings suggest that CD34-positive mesenchymal cells are an integral component of elastofibroma, presumably representing a clonal fibrous proliferation.7

Frequency

United States

Elastofibromas are rare.

International

Elastofibromas are often mistaken for malignant tumors because of their size and location deep to the periscapular muscles. An orthopedic oncology database of 17,500 patients showed 15 patients with elastofibroma dorsi, 12 men and 3 women, with a mean age at diagnosis of 68.4 years and a range of 51-79 years.8

Mortality/Morbidity

Mortality rates are unknown. Morbidity is not expected.

Sex

Of persons affected, 93% are female.

Age

Elastofibromas occur most often in elderly women but have been reported in persons aged 35-94 years.


CLINICAL

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History

Elastofibroma dorsi is an uncommon benign soft tissue pseudotumor usually located at the lower pole of the scapula, deep to the serratus anterior, and often attached to the periosteum of the ribs; patients present with a long history of swelling and, occasionally, pain and discomfort.8

Physical

Clinically, patients usually present with a large, well-circumscribed tumor that most often does not adhere to the overlying skin. One case of ulceration has been described.9 In 99% of cases, elastofibromas are located in the periscapular area, in relation to the latissimus dorsi, rhomboid, and serratus anterior muscles.

Uncommon locations include the deltoid muscle, ischial tuberosity, greater trochanter, olecranon, thoracic wall, foot, stomach, mediastinum,10 orbita, cornea, and oral mucosa.11 Occasionally, the tumor invades the surrounding tissues and becomes fixed to the underlying periosteum. Usually, the lesions are prominent and measure several centimeters in length; however, a small elastofibroma may be overlooked unless the patient is asked to move his or her arm laterally or anteriorly. Most patients with elastofibromas are asymptomatic. Only rarely do they report stiff shoulders, local pain with arm movement, and/or an annoying click with shoulder motion occur.

Causes

The histogenesis of the morphologically unique elastic fibers is controversial. Note particularly that the elastinophilic structure may result from elastotic degeneration of collagen. Recent immunohistochemical, ultrastructural, and biochemical investigations have shown that the elastinophilic material is composed of elastic fibers with only a slight difference in the amino acid composition. This elastic material possibly derives from an abnormal process of elastogenesis rather than representing degenerative changes in collagen or elastic fibers themselves.


DIFFERENTIALS

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Lipomas

Other Problems to be Considered

Extra-abdominal fibromatosis
Sarcoma
Subcutaneous metastasis


WORKUP

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Imaging Studies

  • Elastofibromas have a typical sonographic appearance consisting of arrays of linear strands against an echogenic background.12 However, in some cases, the ultrasound pattern of an elastofibroma dorsi may be very similar to the surrounding muscular tissue, and neither a clear cleavage surface nor a specific vascular pattern can be seen. In these cases, the elastofibroma may be very difficult to distinguish from surrounding tissue.13
  • Chest wall radiographic findings are usually normal; however, elevation of the scapula from the chest wall has been detected in a few cases.
  • CT scanning and MRI reveal a lenticular, unencapsulated, soft tissue mass with skeletal muscle attenuation interspersed with strands of fat attenuation.14 Small elastofibromas may be difficult to visualize on CT scans or MRIs, but they can be enhanced by the use of gadolinium. Its characteristic location (periscapular region) and specific imaging appearance on ultrasound images, CT scans, and MRIs facilitates accurate diagnosis.15
  • Incidental detection of bilateral elastofibroma dorsi with F-18 fluorodeoxyglucose positron emission computed tomography scanning has been described.16

Procedures

  • A biopsy specimen should be obtained from the affected area. The surgical excision should be large and should include skin, subcutaneous fatty tissue, and, if necessary, deeper tissue. A shave or punch biopsy is not sufficient.

Histologic Findings

The tumor grossly appears as an ill-defined mass with a white or gray-white, glistening surface.

Upon light microscopy, elastofibromas are dermal unencapsulated tumors composed of branched and unbranched elastic fibers, eosinophilic collagen bundles, and scattered fatty tissue. The elastic fibers have a degenerated, beaded appearance or are fragmented into small globules or droplets arranged in a linear pattern. The epidermis is usually unaffected. The interspersed spindle or stellate cells show a fibroblastlike appearance and were almost consistently positive for vimentin and frequently positive for CD34 and lysozyme immunohistochemically.2

At an ultrastructural level, the elastic fibers appear as an irregular granular or fibrillary aggregation of electron-dense, amorphous material surrounded by microfibrils and collagen fibers. Collagen fibers are commonly incorporated within the elastic material.

Immunohistochemically, elastofibromas stain positively for vimentin but negatively for smooth muscle actin, S-100, desmin, and p53.

Because of their densely fibrous nature, hypocellularity may be observed in fine-needle aspiration biopsy specimens of elastofibromas; thus, diagnostic material may be overlooked. The smears show evidence of mature adipocytes, fibroblasts, collagen fibers, globular bodies, and characteristic braidlike or fernlike structures, revealing degenerative elastic fibers. Careful evaluation of the background of the smears coupled with full knowledge of the clinical and radiological findings, including those from MRIs, is required to establish the correct diagnosis; therefore, a skin biopsy is preferable.


TREATMENT

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Surgical Care

Complete surgical excision is the treatment of choice in symptomatic patients. Recurrence after surgery is unusual and has been reported in only one case. In another case, spontaneous regression was observed without treatment.


FOLLOW-UP

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Further Outpatient Care

Because elastofibromas are benign neoplasms, no long-term follow-up care is necessary.


MISCELLANEOUS

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Medical/Legal Pitfalls

If patients do not report stiff shoulders or pain, consider differential diagnoses such as lipoma, extra-abdominal fibromatosis, sarcoma, and subcutaneous metastasis.

The eMedicine article Fibroma may be of interest, as may the Medscape CME course Stay Connected: Update on the Management of Sarcoma (Slides With Transcript)


REFERENCES

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Elastofibroma excerpt

Article Last Updated: May 15, 2008