Gastrointestinal Stromal Tumour

        

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               Path Quiz Case-33

      Diagnosis:  Atypical Fibroxanthoma

            Dr Sampurna Roy  MD

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 August 2009

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               Case 33:Case history and images:

                    

Elastofibroma is a benign slowly progressive reactive lesion involving abnormal elastogenesis.

It was first reported by Jarvi and Saxen in 1959 and published in 1961.

The lesion usually occurs in elderly patients and is relatively more common in females. Most cases are unilateral.

Bilateral involvement is present in about 10% of all cases.

The patient usually gives long history of manual labour.

Elastofibroma is usually located in the lower subscapular area and is probably caused by friction between the inferior edge of the scapula and the underlying chest wall.

Cases have also been reported  in the deltoid muscle, hip, thigh and stomach.

Histologically, the lesion is characterized by an admixture of collagenous fibrous bands and elastic fibres. Elastin stains reveal branched or unbranched fibres with irregular serrated margins or fibres arranged in globoid aggregates. 

Electron microscopic studies revealed that the fibres consisted of granular aggregations of electron dense material along a central core surrounded by an amorphous matrix containing microfibrils. The collagen deposited in this disease is a mixture of Type I, II and III.

According to some authors it has been assumed there is an underlying genetic disposition or inherent enzymatic defect.

This benign reactive lesion has no tendency to recur and is usually treated by surgical excision.

Image Link1 ; Image Link2 ; Image Link3 ; Image Link4

                    

Further reading:

Elastofibroma Dorsi- Acta Pathol Microbiol Scand 51:83, 1961

Elastofibroma. A correlated light and electron microscopic study. Virchows arch (Pathol Anat) 369 (2):127-140, 1982 

Elastofibromatous lesion of the stomach in a patient with elastofibroma dorsi.Am J Surg Pathol 9(3) : 233-237, 1985

Elastofibroma in Okinawa.A clinicopathologic study of 170 cases. Cancer 50(9):1794-1805,1982 

Elastofibroma. Disturbed elastic fibrillogenesis by periosteal-derived cells ? An immunoelectron microscopic and in-situ hybridization study. Hum Pathol 22: 1017-1029, 1991

"Aberrant elastic" in elastofibroma. An immunohistochemical and ultrastructural study.  Ultrastruct pathol 19: 45-50, 1995 

Elastofibroma of the stomach. Pathol Res Pract. 199 (9) : 637-639, 2003

Elastofibroma associated with high grade leiomyosarcoma of the soft tissue ;  a light and ultrastructural study of one case . J  Submicrosc Cytol  Pathol .35 (1) : 43-8,  2003

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