Elastofibroma

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Elastofibroma dorsi Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]Ammu Susheela, M.D. [3]

Synonyms and Keywords: elastofibroma dorsi

Overview

Historical Perspective

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 elderly female. [9]

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

Patients with elastofibroma usually appear normal.

Physical examination findings of Elastofibroma can include limited range of motion and swelling [13]

Laboratory Findings

There are no diagnostic laboratory findings associated with elastofibroma.

Electrocardiogram

There are no ECG findings associated with elastofibroma.

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 elastofibroma.

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

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. [14]

Treatment

Surgery

Surgical resection is considered only in symptomatic cases. [19]


Primary Prevention

There are no established measures for the primary prevention of elastofibroma.


Secondary Prevention

There are no established measures for the secondary prevention of elastofibroma.

References

  1. JARVI O, SAXEN E (1961). "Elastofibroma dorse". Acta Pathol Microbiol Scand Suppl. 51(Suppl 144): 83–4. PMID 13789598.
  2. 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. PMID 18407963.
  3. 3.0 3.1 Machens HG, Mechtersheimer R, Göhring U, Schlag PN (October 1992). "Bilateral elastofibroma dorsi". Ann. Thorac. Surg. 54 (4): 774–6. PMID 1417241.
  4. Jena, Amitabh; Patnayak, Rashmi; Settipalli, Sarla; Nagesh, N (2016). "Elastofibroma: An uncommon tumor revisited". Journal of Cutaneous and Aesthetic Surgery. 9 (1): 34. doi:10.4103/0974-2077.178543. ISSN 0974-2077.
  5. Jena, Amitabh; Patnayak, Rashmi; Settipalli, Sarla; Nagesh, N (2016). "Elastofibroma: An uncommon tumor revisited". Journal of Cutaneous and Aesthetic Surgery. 9 (1): 34. doi:10.4103/0974-2077.178543. ISSN 0974-2077.
  6. Jena, Amitabh; Patnayak, Rashmi; Settipalli, Sarla; Nagesh, N (2016). "Elastofibroma: An uncommon tumor revisited". Journal of Cutaneous and Aesthetic Surgery. 9 (1): 34. doi:10.4103/0974-2077.178543. ISSN 0974-2077.
  7. Tetikkurt C, Tetikkurt S, Bayar N (2008). "Diagnosis of elastofibroma". Can. Respir. J. 15 (4): 217–8. doi:10.1155/2008/638624. PMC 2677955. PMID 18551204.
  8. 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. PMID 1124654.
  9. Patnayak R, Jena A, Settipalli S, Nagesh N (2016). "Elastofibroma: An Uncommon Tumor Revisited". J Cutan Aesthet Surg. 9 (1): 34–7. doi:10.4103/0974-2077.178543. PMC 4812887. PMID 27081248.
  10. Patnayak R, Jena A, Settipalli S, Nagesh N (2016). "Elastofibroma: An Uncommon Tumor Revisited". J Cutan Aesthet Surg. 9 (1): 34–7. doi:10.4103/0974-2077.178543. PMC 4812887. PMID 27081248.
  11. Nagamine N, Nohara Y, Ito E (November 1982). "Elastofibroma in Okinawa. A clinicopathologic study of 170 cases". Cancer. 50 (9): 1794–805. PMID 7116305.
  12. Greenberg JA, Lockwood RC (March 1989). "Elastofibroma dorsi. A case report and review of the literature". Orthop Rev. 18 (3): 329–33. PMID 2652048.
  13. 13.0 13.1 Sarici IS, Basbay E, Mustu M, Eskut B, Kala F, Agcaoglu O, Akici M, Ozkurt E (2014). "Bilateral elastofibroma dorsi: A case report". Int J Surg Case Rep. 5 (12): 1139–41. doi:10.1016/j.ijscr.2014.10.032. PMC 4275815. PMID 25437657.
  14. 14.0 14.1 Tetikkurt C, Tetikkurt S, Bayar N (2008). "Diagnosis of elastofibroma". Can. Respir. J. 15 (4): 217–8. doi:10.1155/2008/638624. PMC 2677955. PMID 18551204.
  15. 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. PMID 8998883.
  16. 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. PMID 14648789.
  17. Go PH, Meadows MC, Deleon EM, Chamberlain RS (October 2010). "Elastofibroma dorsi: A soft tissue masquerade". Int J Shoulder Surg. 4 (4): 97–101. doi:10.4103/0973-6042.79797. PMC 3100815. PMID 21655005.
  18. Patrikeos A, Breidahl W, Robins P (September 2005). "F-18 FDG uptake associated with Elastofibroma dorsi". Clin Nucl Med. 30 (9): 617–8. PMID 16100483.
  19. Jena, Amitabh; Patnayak, Rashmi; Settipalli, Sarla; Nagesh, N (2016). "Elastofibroma: An uncommon tumor revisited". Journal of Cutaneous and Aesthetic Surgery. 9 (1): 34. doi:10.4103/0974-2077.178543. ISSN 0974-2077.





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