Elastofibroma D23.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Elastofibroma cutis; elastofibroma dorsi; Elastofibroma of the back

History
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Jarvi and Saxen, 1961

Definition
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Rare, benign, subcutaneously palpable, clinically symptom-free, solid tumour (node), predominantly located in the scapular region.

Occurrence/Epidemiology
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About 300 cases have been described worldwide. About 2/3 of all published cases come from a family line in southern Japan (Okinawa).

Etiopathogenesis
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Degenerative processes and repeated microtraumas under mechanical stress are discussed. Genetic disposition (familial occurrence possible). Possible genetic defect on chromosome Xq12-q22.

Manifestation
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Occurs at an advanced age, usually after the 35th year of life (average 7th decade). Women are affected much more often than men.

Localization
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Mainly scapula area located below the fascia (> 80% of cases), also lumbar region, upper arm, hip and foot.

Clinical features
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Predominantly isolated, asymptomatic, rarely slightly painful, 4-12 cm large, skin-coloured, not very conspicuous lump, which is fused with the covering skin. In about 10% of patients there is bilateral, mostly asynchronous growth. Adhesions with deeper structures (muscles) are possible. In these cases the nodules are considered pathological.

Histology
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Tumorous structural change of the collagenous connective tissue localized in the dermis and spreading to the subcutaneous fatty tissue. There are broad, cell-poor, lumpy collagenous ligaments with a staining behaviour like elastotic material. These are partly wavy, partly fragmented and fluoresce greenish. Isolated islands of fatty tissue.

Immunohistochemistry: Numerous myofibroblasts of the VAD-type, some CD34- and S100-positive cells.

Differential diagnosis
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Therapy
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If symptomatic, excision without greater safety margin (no evidence of malignant degeneration).

Progression/forecast
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Excision is curative. Recurrences have so far only been proven by casuistry (probably after incomplete excision!).

Literature
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  1. Alberghini M et al (2003) Elastofibroma associated with high grade leiomyosarcoma of the soft tissues: a light and ultrastructural study of one case. J Submicrosc Cytol Pathol 35: 43-48
  2. Alberghini M et al (2004) Histochemical and ultrastructural study of an elastofibroma dorsi coexisting with a high grade spindle cell sarcoma. Eur J Histochem 48: 173-178
  3. Dalal A (2003) Sonographic detection of elastofibroma dorsi. J Clin Ultrasound 31: 375-378
  4. Guha AR et al (2004) Elastofibroma dorsi--a case report and review of literature. Int J Clin Pract 58: 218-220
  5. Jarvi OH, Saxen AE (1961) Elastofibroma dorsi. Acta Pathol Microbiol Scand 144: 83-84
  6. Kara M (2002) Bilateral elastofibroma dorsi: proper positioning for an accurate diagnosis. Eur J Cardiothorac Surgery 22: 839-841
  7. Muller LP et al (1999) Bilateral elastofibroma dorsi. Surgeon 70: 1357-1360
  8. Nishio JN et al (2002) Gain of Xq detected by comparative genomic hybridization in elastofibroma. Int J Mol Med 10: 277-280
  9. Nishida A et al (2003) Bilateral elastofibroma of the thighs with concomitant subscapular lesions. Skeletal radiol 32: 116-118

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Last updated on: 29.10.2020