Elastofibroma was first discovered by Jarvi and Saxen, in 1961.[1]
Classification
There is no established system for the classification of elastofibroma.
Pathophysiology
Elastofibroma dorsi is a rare, slow growing, ill-defined soft tissue mass of the chest wall. It occurs most in the periscapular region.It is commonly located beneath latissimus dorsi and rhomboid major muscles near to the inferior angle of the scapula. It is a benign neoplasm with clinical appearence of a malignant tumor. [2]
The exact pathogenesis of elastofibroma dorsi is not fully understood. It is thought that elastofibroma dorsi is the result of subclinical microtrauma, reactive hyperplasia of elastic fibres and increased production of fibrous tissue. [3]
Elastofibroma often has bilateral location in the thoracic wall.[4]
On gross pathology, characteristic findings of elastofibroma include:[5]
A solitary, poorly circumscribed, heterogeneous, soft-tissue mass
Cut section are firm with grayish-white areas
On microscopic histopathological analysis, characteristic findings of Elastofibroma include: [6]
Eosinophilic, beaded elastic fibers with Verhoeff's elastic stain
Many fragmented fibers with appearance of beads on a string
Causes
The cause of elastofibroma dorsi has not been identified. Subclinical microtrauma could be one of the reasons. [3]
Differentiating Elastofibroma dorsi from Other Diseases
Elastofibroma dorsi must be differentiated from desmoid tumours, neurofibroma and liposarcoma. [7]
Epidemiology and Demographics
The prevalence of elastofibroma is approximately 11200 in men and 24400 in women per 100,000 individuals in each gender autopsies. [8]
Elastofibroma commonly affects females ranging from 35-94 years. [10]
Female are more commonly affected by elastofibroma than men. The female to male ratio is approximately 2.1
The majority of elastofibroma cases are reported in Japan. [11]
Risk Factors
There are no established risk factors for elastofibroma.
Screening
There is insufficient evidence to recommend routine screening for elastofibroma.
Natural History, Complications, and Prognosis
Prognosis is generally excellent.
Diagnosis
Diagnostic Study of Choice
There are no established criteria for the diagnosis of elastofibroma.
History and Symptoms
The majority of patients with elastofibroma dorsi are asymptomatic. Elastofibroma may present with:[12]
Painless swelling
Pain ( less than 10% of patients)
Scapular snapping
Limitation of motion,
Clunking sensation in the shoulder adduction-abduction movement[13]
Physical Examination
Physical examination findings of Elastofibroma can include limited range of motion and swelling [13]
Laboratory Findings
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
OR
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
OR
[Test] is usually normal among patients with [disease name].
OR
Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
OR
There are no diagnostic laboratory findings associated with [disease name].
Electrocardiogram
There are no ECG findings associated with [disease name].
OR
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
X-ray
An x-ray may be helpful in the diagnosis of elastofibroma. Findings on an x-ray suggestive of elastofibroma include soft tissue density in the periscapular region. X-ray may be normal. [14]
Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with [disease name].
OR
Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
CT scan
CT scan may be helpful in the diagnosis of elastofibroma dorsi. Findings on CT scan suggestive of elastofibroma dorsi include a heterogenous soft tissue mass with poorly defined margins. [15]
MRI
Magnetic resonance imaging is the most useful diagnostic tool for diagnosis of elastofibroma dorsi. [16]
Findings on MRI suggestive of elastofibroma include solitary heterogeneous, poorly circumscribed, soft-tissue mass.[17]
The lesion is isointense to muscle in T1 and T2WI images
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Other Imaging Findings
Positron emission tomography/computed tomography (PET/CT) may be helpful in the diagnosis of elastofibroma. Findings on PET/CT suggestive of elastofibroma include low to moderate metabolic activity in these patients. PET/CT shows low-grade diffuse 18F fluorodeoxyglucose uptake. [18]
Other Diagnostic Studies
Needle aspiration biopsy is helpful in the diagnosis of elastofibroma and exclude sarcoma. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
OR
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
OR
The majority of cases of [disease name] are self-limited and require only supportive care.
OR
[Disease name] is a medical emergency and requires prompt treatment.
OR
The mainstay of treatment for [disease name] is [therapy].
OR
The optimal therapy for [malignancy name] depends on the stage at diagnosis.
OR
[Therapy] is recommended among all patients who develop [disease name].
OR
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
OR
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
OR
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
OR
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
Surgery
Surgical resection is considered only in symptomatic cases. [19]
Surgical intervention is not recommended for the management of [disease name].
OR
Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for symptomatic patients.[20]
OR
The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].
OR
The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
OR
Surgery is the mainstay of treatment for [disease or malignancy].
Primary Prevention
There are no established measures for the primary prevention of [disease name].
OR
There are no available vaccines against [disease name].
OR
Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
OR
[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].
Secondary Prevention
There are no established measures for the secondary prevention of [disease name].
OR
Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].
References
↑JARVI O, SAXEN E (1961). "Elastofibroma dorse". Acta Pathol Microbiol Scand Suppl. 51(Suppl 144): 83–4. PMID13789598.
↑Freixinet J, Rodríguez P, Hussein M, Sanromán B, Herrero J, Gil R (August 2008). "Elastofibroma of the thoracic wall". Interact Cardiovasc Thorac Surg. 7 (4): 626–8. doi:10.1510/icvts.2007.174722. PMID18407963.
↑Järvi OH, Länsimies PH (January 1975). "Subclinical elastofibromas in the scapular region in an autopsy series". Acta Pathol Microbiol Scand A. 83 (1): 87–108. PMID1124654.
↑Nagamine N, Nohara Y, Ito E (November 1982). "Elastofibroma in Okinawa. A clinicopathologic study of 170 cases". Cancer. 50 (9): 1794–805. PMID7116305.
↑Greenberg JA, Lockwood RC (March 1989). "Elastofibroma dorsi. A case report and review of the literature". Orthop Rev. 18 (3): 329–33. PMID2652048.
↑Hoffman JK, Klein MH, McInerney VK (April 1996). "Bilateral elastofibroma: a case report and review of the literature". Clin. Orthop. Relat. Res. (325): 245–50. PMID8998883.
↑Domanski HA, Carlén B, Sloth M, Rydholm A (December 2003). "Elastofibroma dorsi has distinct cytomorphologic features, making diagnostic surgical biopsy unnecessary: cytomorphologic study with clinical, radiologic, and electron microscopic correlations". Diagn. Cytopathol. 29 (6): 327–33. doi:10.1002/dc.10381. PMID14648789.
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 prickled 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:
↑ 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.
