eMedicine Specialties > Dermatology > Benign Neoplasms

Elastofibroma

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

Updated: Jul 21, 2009

Introduction

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, elastofibroma, in 1961. Elastofibroma is characterized by accumulated abnormal elastic fibers and is generally regarded as a reactive process, an unusual fibroblastic pseudotumor.2

Pathophysiology

The etiology of elastofibroma 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 for elastofibroma. Morbidity is not expected.

Sex

Of persons affected by elastofibromas, 93% are female.

Age

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

Clinical

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 with elastofibroma dorsi present with a long history of swelling and, occasionally, pain and discomfort.8

Physical

Clinically, patients with elastofibromas 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.10

Uncommon locations include the deltoid muscle,11 ischial tuberosity, greater trochanter, olecranon, thoracic wall, foot, stomach, mediastinum,12 orbita, cornea, and oral mucosa.13 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.14 Potter et al reported an elastofibroma presenting in the oral cavity,15 and Hsu et al reported a case of elastofibroma oculi.16

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.

More on Elastofibroma

Overview: Elastofibroma
Differential Diagnoses & Workup: Elastofibroma
Treatment & Medication: Elastofibroma
Follow-up: Elastofibroma
References
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References

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Further Reading

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Keywords

elastofibroma, elastofibroma dorsi, connective-tissue tumor, connective tissue tumor, elastogenesis

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Sonja Stander, MD, Staff Physician, Department of Dermatology, University-Hospital Muenster, Germany
Disclosure: Nothing to disclose.

Thomas Schwarz, MD, Vice Chairman, Professor, Department of Dermatology, University of Kiel, Germany
Thomas Schwarz, MD is a member of the following medical societies: American Academy of Dermatology, American Association of Immunologists, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Medical Editor

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
Kathryn Schwarzenberger, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, American Dermatological Association, Dermatology Foundation, Medical Dermatology Society, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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