Elastofibroma

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List of terms related to Elastofibroma

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

Pathophysiology

• Elastofibroma, also called elastofibroma dorsi, is an ill-defined fibroelastic tumor-like condition made up of enlarged and irregular elastic fibers.
• 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.
• 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:

• Spindle cell lipoma
• nuchal-type 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.
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].

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

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 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

• [Disease name] may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

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].