Elastofibroma: Difference between revisions

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==Differentiating Elastofibroma from other Diseases==
==Differentiating Elastofibroma from other Diseases==
*Elastofibroma must be differentiated from other diseases that cause soft tissue tumor such as:
*Elastofibroma must be differentiated from other diseases that cause soft tissue tumor such as:
:*Spindle cell lipoma
:*Spindle cell [[lipoma]]
:*Nuchal fibroma|nuchal-type fibroma
:*Nuchal fibroma
:*Fibromatosis colli
:*Fibromatosis colli



Revision as of 19:17, 25 April 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Synonyms and keywords: Elastofibroma dorsi

Overview

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.

Causes

Differentiating Elastofibroma from other Diseases

  • Elastofibroma must be differentiated from other diseases that cause soft tissue tumor such as:
  • Spindle cell lipoma
  • Nuchal fibroma
  • Fibromatosis colli

Epidemiology and Demographics

  • Elastoblastoma is a very rare disease.

Age

  • 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

Treatment

Surgery

  • Surgery is the mainstay of therapy for elastofibroma.

References

  1. 1.0 1.1 Chandrasekar, C. R.; Grimer, R. J.; Carter, S. R.; Tillman, R. M.; Abudu, A.; Davies, A. M.; Sumathi, V. P. (2008). "Elastofibroma Dorsi: An Uncommon Benign Pseudotumour". Sarcoma. 2008: 1. doi:10.1155/2008/756565. PMC 2276598. PMID 18382611.
  2. 2.0 2.1 Briccoli, 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. PMID 10664338.
  3. 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. PMID 17462431.
  4. Nakamura, Y.; Ohta, Y.; Itoh, S.; Haratake, A.; Nakano, Y.; Umeda, A.; Shima, H.; Tomoda, N. (1992). "Elastofibroma dorsi. Cytologic, histologic, immunohistochemical and ultrastructural studies". Acta cytologica. 36 (4): 559–562. PMID 1636353.