Elastofibroma: Background, Pathophysiology, Epidemiology

Sections

Overview

Background

Elastofibroma is a rare, benign, slow-growing connective-tissue tumor that occurs most often in the subscapular area in elderly women. Jarvi and Saxen^[1]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. A mechanical strain-dependent reactivation of periostin and tenascin-C expression, along with elastin deposition, may account for the pathogenesis of their neoplasms.^[4]

Elastofibroma may be a reactive process; its etiology remains unknown.^[5]In a 2002 study, chromosomal gains were suggested as a cause for elastofibromas. Nishio et al^[6]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. It was concluded that these chromosomal regions may contain genes involved in the development of at least some elastofibromas. Genomic alterations were documented in 2 cases, with losses detected on 1p, 13q, 19p, and 22q by array-based comparative genomic hybridization (aCGH).^[5]

Coexistence of an elastofibroma with a high-grade spindle cell sarcoma^[7]and a high-grade leiomyosarcoma^[8]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.^[9]

Frequency

Elastofibromas are rare. 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.^[10]

In another study, 1,751 patients were evaluated with (18)fludeoxyglucose (FDG) – positron emission tomography (PET)/CT scanning, which detected 29 cases of elastofibroma dorsi as an incidental finding, giving a prevalence of 1.66%.^[11]

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.

Prognosis

Mortality rates are unknown for elastofibroma. Morbidity is not expected.

Clinical Presentation

Jarvi O, Saxen E. Elastofibroma dorse. Acta Pathol Microbiol Scand Suppl. 1961. 51(Suppl 144):83-4. [Medline].
Hisaoka M, Hashimoto H. Elastofibroma: clonal fibrous proliferation with predominant CD34-positive cells. Virchows Arch. 2006 Feb. 448(2):195-9. [Medline].
Shimizu S, Yasui C, Tateno M, et al. Multiple elastofibromas. J Am Acad Dermatol. 2004 Jan. 50(1):126-9. [Medline].
Di Vito A, Scali E, Ferraro G, Mignogna C, Presta I, Camastra C, et al. Elastofibroma dorsi: a histochemical and immunohistochemical study. Eur J Histochem. 2015 Feb 19. 59 (1):2459. [Medline].
Hernandez JL, Rodriguez-Parets JO, Valero JM, et al. High-resolution genome-wide analysis of chromosomal alterations in elastofibroma. Virchows Arch. 2010 Apr 27. [Medline].
Nishio JN, Iwasaki H, Ohjimi Y, et al. Gain of Xq detected by comparative genomic hybridization in elastofibroma. Int J Mol Med. 2002 Sep. 10(3):277-80. [Medline].
Alberghini M, Bacchini P, Pignatti G, et al. Histochemical and ultrastructural study of an elastofibroma dorsi coexisting with a high grade spindle cell sarcoma. Eur J Histochem. 2004 Apr-Jun. 48(2):173-8. [Medline].
Alberghini M, Pignatti G, Zanella L, et al. Elastofibroma associated with high grade leiomyosarcoma of the soft tissues: a light and ultrastructural study of one case. J Submicrosc Cytol Pathol. 2003 Jan. 35(1):43-8. [Medline].
Gun BD, Bahadir B, Behzatoglu K, Gun MO, Ozdamar SO. Elastofibroma: a clinicopathologic and immunohistochemical study of seven cases and literature review. APMIS. 2007 Feb. 115(2):115-9. [Medline].
Chandrasekar CR, Grimer RJ, Carter SR, et al. Elastofibroma dorsi: an uncommon benign pseudotumour. Sarcoma. 2008. 2008:756565. [Medline].
Blumenkrantz Y, Bruno GL, Gonzalez CJ, et al. Characterization of Elastofibroma Dorsi with (18)FDG PET/CT: a retrospective study. Rev Esp Med Nucl. 2011 Apr 3. [Medline].
Hocar O, Buffard V, Ortonne N, Bagot M. [Bilateral elastofibroma dorsi]. Ann Dermatol Venereol. 2012 Jan. 139(1):41-5. [Medline].
Lococo F, Cesario A, Mattei F, Petrone G, Vita LM, Petracca-Ciavarella L, et al. Elastofibroma dorsi: clinicopathological analysis of 71 cases. Thorac Cardiovasc Surg. 2013 Apr. 61(3):215-22. [Medline].
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Nonaka CF, Rego DM, Miguel MC, de Souza LB, Pinto LP. Elastofibromatous change of the oral mucosa: case report and literature review. J Cutan Pathol. 2010 Oct. 37(10):1067-71. [Medline].
Schwarz T, Oppolzer G, Duschet P, Bruckler B, Gschnait F. Ulcerating elastofibroma dorsi. J Am Acad Dermatol. 1989 Nov. 21(5 Pt 2):1142-4. [Medline].
Daigeler A, Vogt PM, Busch K, et al. Elastofibroma dorsi--differential diagnosis in chest wall tumours. World J Surg Oncol. 2007 Feb 5. 5:15. [Medline].
Cavallasca JA, Sohn DI, Borgia AR, Maliandi MD, Musuruana JL. Elastofibroma dorsi: Review of 4 cases. Reumatol Clin. 2012 Mar 19. [Medline].
Mirra JM, Straub LR, Jarvi OH. Elastofibroma of the deltoid. A case report. Cancer. 1974 Jan. 33(1):234-8. [Medline].
De Nictolis M, Goteri G, Campanati G, Prat J. Elastofibrolipoma of the mediastinum. A previously undescribed benign tumor containing abnormal elastic fibers. Am J Surg Pathol. 1995 Mar. 19(3):364-7. [Medline].
Manchandu R, Foote J, Alawi F. Elastofibroma presenting as an oral soft tissue mass. J Oral Pathol Med. 2008 Feb. 37(2):125-6. [Medline].
Majo J, Gracia I, Doncel A, Valera M, Nunez A, Guix M. Elastofibroma dorsi as a cause of shoulder pain or snapping scapula. Clin Orthop Relat Res. 2001 Jul. 200-4. [Medline].
Potter TJ, Summerlin DJ, Rodgers SF. Elastofibroma: the initial report in the oral mucosa. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2004 Jan. 97(1):64-7. [Medline].
Hsu JK, Cavanagh HD, Green WR. An unusual case of elastofibroma oculi. Cornea. 1997 Jan. 16(1):112-9. [Medline].
Smith HG, Hannay J, Thway K, Messiou C, Smith M, Strauss DC, et al. Elastofibroma dorsi: The clunking tumour that need not cause alarm. Ann R Coll Surg Engl. 2016 Mar. 98 (3):208-11. [Medline].
Erkilic S, Kocer NE, Sivrikoz C. Subscapular elastofibroma intermingled with adipose tissue: variant type of elastofibroma or lipoma?. Ann Diagn Pathol. 2005 Dec. 9(6):327-9. [Medline].
Bianchi S, Martinoli C, Abdelwahab IF, Gandolfo N, Derchi LE, Damiani S. Elastofibroma dorsi: sonographic findings. AJR Am J Roentgenol. 1997 Oct. 169(4):1113-5. [Medline].
Solivetti FM, Bacaro D, Di Luca Sidozzi A, Cecconi P. Elastofibroma dorsi: ultrasound pattern in three patients. J Exp Clin Cancer Res. 2003 Dec. 22(4):565-9. [Medline].
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Naylor MF, Nascimento AG, Sherrick AD, McLeod RA. Elastofibroma dorsi: radiologic findings in 12 patients. AJR Am J Roentgenol. 1996 Sep. 167(3):683-7. [Medline].
Valls R, Melloni P, Darnell A, Sanchez-Flo R. Elastofibroma dorsi: a chest wall pseudotumor. Computed tomography and magnetic resonance imaging diagnosis. Acta Orthop Belg. 1996 Jun. 62(2):103-6. [Medline].
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Oueslati S, Douira-Khomsi W, Bouaziz MC, Zaouia K. Elastofibroma dorsi: A report on 6 cases. Acta Orthop Belg. 2006 Apr. 72(2):237-42. [Medline].
Alouini R, Allani M, Harzallah L, Bahri M, Kraiem C, Tlili-Graies K. [Elastofibroma: imaging features]. J Radiol. 2005 Nov. 86(11):1712-5. [Medline].
Yanez S, Val-Bernal JF, Echevarria MA, Landeras R, Izquierdo J, Gallardo E. [Retrospective analysis of 6 cases of elastofibroma dorsi]. Actas Dermosifiliogr. 2008 Oct. 99(8):644-7. [Medline].
Tamimi Mariño I, Sesma Solis P, Pérez Lara A, Martinez Malo J, Vazquez ML, Tamimi F. Sensitivity and positive predictive value of magnetic resonance imaging in the diagnosis of elastofibroma dorsi: review of fourteen cases. J Shoulder Elbow Surg. 2013 Jan. 22(1):57-63. [Medline].
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Wasyliw CW, Caride VJ. Incidental detection of bilateral elastofibroma dorsi with F-18 FDG PET/CT. Clin Nucl Med. 2005 Oct. 30(10):700-1. [Medline].
Kai K, Kusano K, Sakai M, et al. Active neovascularization and possible vascular-centric development of gastric and periscapular elastofibromas. Virchows Arch. 2009 Feb. 454(2):181-8. [Medline].
Kayaselcuk F, Demirhan B, Kayaselcuk U, Ozerdem OR, Tuncer I. Vimentin, smooth muscle actin, desmin, S-100 protein, p53, and estrogen receptor expression in elastofibroma and nodular fasciitis. Ann Diagn Pathol. 2002 Apr. 6(2):94-9. [Medline].
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Media Gallery

Elastofibroma. Hematoxylin and eosin stain. Intermediate magnification. Courtesy of Soca1zim, via Wikimedia Commons.

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Sonja Stander, MD Staff Physician, Department of Dermatology, University-Hospital Muenster, Germany

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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: Women's Dermatologic Society, American Contact Dermatitis Society, Medical Dermatology Society, Dermatology Foundation, Alpha Omega Alpha, American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

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.

Elastofibroma

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