Elastofibroma: Difference between revisions

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:*Slow growing, deep-seated, firm mass, often presenting bilaterally
:*Slow growing, deep-seated, firm mass, often presenting bilaterally
:*Tenderness
:*Tenderness
=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].
*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Imaging Findings===
*On CT, elastofibroma is characterized bypoorly circumscribed, heterogeneous soft tissue mass, with a signal intensity similar to skeletal muscle.
* On [[medical ultrasonography|US]], elastofibroma is characterized by multilayered pattern of hypoechoic linear areas of fat deposition intermixed with echogenic fibroelastic tissue.
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Elastofibroma may also be diagnosed using biopsy and histochemistry.
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
====Histochemistry====
[[File:Elastofibroma_stained_with_elastic_stain_LDRT.tif|thumb|right|An 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. <ref name="Nakamura">{{Cite journal | last1 = Nakamura | first1 = Y. | last2 = Ohta | first2 = Y. | last3 = Itoh | first3 = S. | last4 = Haratake | first4 = A. | last5 = Nakano | first5 = Y. | last6 = Umeda | first6 = A. | last7 = Shima | first7 = H. | last8 = Tomoda | first8 = N. | title = Elastofibroma dorsi. Cytologic, histologic, immunohistochemical and ultrastructural studies | journal = Acta cytologica | volume = 36 | issue = 4 | pages = 559–562 | year = 1992 | pmid = 1636353}}</ref>


== Treatment ==
== Treatment ==

Revision as of 20:24, 11 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: Synonym 1; Synonym 2; Synonym 3

Overview

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, sometimes even 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. Because of degeneration, the elastic fibers will appear as globules with a serrated or "prickled" edge.

Causes

  • Elastofibroma may be caused by either trauma, genetic mutation, or systemic enzyme defects.

Differentiating [disease name] from other Diseases

  • Elastofibroma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

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

File:Elastofibroma stained with elastic stain LDRT.tif
An 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

Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1], [test 2], and [test 3].

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.