Elastofibroma is an ill-defined fibroelastic tumor-like condition made up of enlarged and irregular elastic fibers. On gross pathology, ill defined, nonencapsulated, rubbery, and firm, white lesion with interspersed fat are characteristic findings of elastofibroma. On microscopic histopathological analysis, heavy dense bands of collagenous tissue dissected by fat and abnormal elastic fibers are characteristic findings of elastofibroma . The elastic fibers are usually quite large and are easily identified. The elastic fibers are coarse, thick, and darkly eosinophilic, often fragmented into globules, creating a "string of pearls" or "pipe cleaner" appearance. Degeneration will cause the elastic fibers to appear as globules with a serrated or pricked edge. Elastofibroma may be caused by either trauma, genetic mutation, or systemic enzyme defects. Elastofibroma must be differentiated from other diseases that cause soft tissue tumor such as: spindle cell lipoma, nuchal-type fibroma, and fibromatosis colli. Elastofibroma may also be diagnosed using biopsy and histochemistry. Surgery is the mainstay of therapy for elastofibroma.
Pathophysiology
Elastofibroma, also called elastofibroma dorsi, is an ill-defined fibroelastic tumor-like condition made up of enlarged and irregular elastic fibers. [1][2]
The tumor develops very specifically in the subscapular or infrascapular area, deep to the muscle, and can be attached to periosteum of ribs. It is usually between the shoulder blade and the lower neck, with rare tumors reported in the chest wall. [1][3][2]
The genetic mutation in has been associated alterations of short arm of chromosome 1 with the development of elastofibroma.
On gross pathology, ill defined, nonencapsulated, rubbery, and firm, white lesion with interspersed fat are characteristic findings of elastofibroma.
On microscopic histopathological analysis, heavy dense bands of collagenous tissue dissected by fat and abnormal elastic fibers are characteristic findings of elastofibroma. The elastic fibers are often quite large and are easily identified. The elastic fibers are coarse, thick, and darkly eosinophilic, often fragmented into globules, creating a "string of pearls" or "pipe cleaner" appearance. Degeneration will cause the elastic fibers to appear as globules with a serrated or prickled edge.
Elastofibroma
Elastofibroma
Papillary Fibroelastoma: When located on the mitral valve, these tumors are usually on the anterior leaflet of the atrial surface.
Causes
Elastofibroma may be caused by either trauma, genetic mutation, or systemic enzyme defects.
Differentiating Elastofibroma from other Diseases
Elastofibroma must be differentiated from other diseases that cause soft tissue tumor such as:
Elastofibroma is more commonly observed among patients aged more than 50 years old.
Gender
Females are more commonly affected with elastofibroma than male.
The female to male ratio is approximately 5:1.
Race
Elastofibroma usually reported more in individuals of the Japanese race.
Risk Factors
Common risk factor in the development of elastofibroma is trauma.
Diagnosis
Symptoms
Symptoms of elastofibroma may include the following:
Swelling
Pain
Physical Examination
Patients with elastofibroma usually appear normal.
Physical examination may be remarkable for:
Slow growing, deep-seated, firm mass, often presenting bilaterally
Tenderness
Other Diagnostic Studies
Elastofibroma may also be diagnosed using biopsy and histochemistry.
Histochemistry
File:Elastofibroma stained with elastic stain LDRT.tifAn elastic stain photomicrograph at high power highlighting the elastic fibers in black. The elastic fibers will be highlighted by a Weigert or von Gieson elastic stains. [4]
Treatment
Surgery
Surgery is the mainstay of therapy for elastofibroma.
↑ 2.02.1Briccoli, A.; Casadei, R.; Di Renzo, M.; Favale, L.; Bacchini, P.; Bertoni, F. (2000). "Elastofibroma dorsi". Surgery today. 30 (2): 147–152. doi:10.1007/pl00010063. PMID10664338.
↑Mortman, K. D.; Hochheiser, G. M.; Giblin, E. M.; Manon-Matos, Y.; Frankel, K. M. (2007). "Elastofibroma Dorsi: Clinicopathologic Review of 6 Cases". The Annals of Thoracic Surgery. 83 (5): 1894–1897. doi:10.1016/j.athoracsur.2006.11.050. PMID17462431.
