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ABDOMINAL IMAGING Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 357-358
Dermatofibrosarcoma protuberans (DFSP)-CT findings in DFSP-a rare skin tumour


Mohan Dai Oswal Cancer Hospital, G.T.Road, Sherpur By Pass, Ludhiana, India

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Keywords: Dermatofibrosarcoma, Cutaneous tumor

How to cite this article:
Shrimali R, Garg L, Setia V, Jain S. Dermatofibrosarcoma protuberans (DFSP)-CT findings in DFSP-a rare skin tumour. Indian J Radiol Imaging 2002;12:357-8

How to cite this URL:
Shrimali R, Garg L, Setia V, Jain S. Dermatofibrosarcoma protuberans (DFSP)-CT findings in DFSP-a rare skin tumour. Indian J Radiol Imaging [serial online] 2002 [cited 2019 Oct 21];12:357-8. Available from: http://www.ijri.org/text.asp?2002/12/3/357/28482
DFSP is a rare, slow growing cutaneous tumour located predominantly on the skin of trunk.

It has a tendency to recur locally; distant recurrences are infrequent. We report CT findings in a case of DFSP.


   Case Report Top


A 48 year woman presented with a recurrent lower abdominal swelling which was progressively increasing in size. Clinical examination revealed a firm, 8 x 8cm lump in the infraumbilical and hypogastric area. Excision had been done previously two times (3 yrs and 1 yr back) and biopsy was reported as DFS.

CT examination revealed a large, nodular, well circumscribed, soft tissue attenuation mass, epi-centered in subcutaneous fat of lower anterior abdominal wall in the midline [Figure - 1]. The overlying skin was stretched over the mass and was not separately identifiable from the mass. The lesion caused a prominent bulge in the abdominal contour. Posteriorly the lesion was superficial to the rectus muscles and the intervening fat plane was grossly preserved. On IV contrast administration considerable enhancement as well as non enhancing patchy necrotic areas were seen [Figure - 2]. The CT findings were consistent with the already histopathologically established diagnosis of DFSP.


   Discussion Top


Darrier and Fernand [1] first recognized DFSP as a clinicopathological entity and Hoffman [2] introduced the term "DFSP". This disease is more common in men than in women and has a peak incidence during third decade. It may occur at sites of previous trauma. The tumour is most commonly found in the trunk and proximal extremities [3]. Local recurrences occur in 20-55% of cases [4].

DFSP is thought to be of histiocytic or neurogenic origin. Majority of the tumors are histologically characterised by uniform, slender, spindles shaped, fibroblast like cells, arranged in a typical storiform or cartwheel pattern [5].

The tumour first appears as a single, red to bluish, blanchable, firm, cutaneous nodule. During the late stage, rate of growth accelerates, producing the characteristic protrusion from the skin [6]. The growth rate is variable. Lesions may remain stable for many years or they may grow slowly with periods of accelerated growth [7].

The CT appearance of DFSP is well defined, unmineralized, nodular soft tissue mass involving the skin and subcutaneous adipose tissue. CT scans or MR images are well suited to show this location, the relation of lesion to underlying structures and the distinct lobular or nodular architecture [8]. On CT scanning DFSP may be confused with a dermatofibroma, a neurogenic tumour, a fibrosarcoma or a malignant fibrous histiocytoma as the CT findings of these tumors resemble those of DFSP, DFSP displays an almost pathognomonic protrusion from the skin, a feature which distinguished DFSP from other tumors [6].

Arteriography shows mild to moderate hypervascularity. This hypervascularity is most likely responsible for the moderate enhancement seen on CT scans obtained after injection of contrast material [8].

The tumour must be excised with a 3cm or greater margin of uninvolved skin. CT is helpful in deciding the line of incision and avoiding inadequate excision, which leads to local recurrence or metastasis [6]

 
   References Top

1.Darier J, Ferrand M, Dermatofibrosarcoma progressifs et recidivants on fibrosarcomas de la plan. Ann Dermatol Venereol 1924; 5:545.  Back to cited text no. 1    
2.Hoffman E, Uber das knollentriebende Fibrokom der Haut, Dermat Zeitschr 1925; 43: 1-8  Back to cited text no. 2    
3.Taylar HB, Helwig EB, Dermatofibrosarcoma protuberans - A Study of 115 cases. Cancer 1962; 15:717-725  Back to cited text no. 3    
4.Rockley PF, Robinson JK, Magid M, Goldblatt D, Dermatofibromasarcoma protuberans of the scalp-a series of cases. J Am Acad Dermatol 1989; 21:278-282.  Back to cited text no. 4    
5.Rutgers E, Kroon BBR, Albus-lutter CE, Gortzak E, Dermatofibrosarcoma protuberans: treatment and prognosis, European Journal of Surgical Oncology 1992; 18:241-248.  Back to cited text no. 5    
6.Miyakawa E, Fujimoto H, Miyakawa K, Nemoto K et al, Dermatofibrosarcoma protuberans - CT findings with pathologic correlation in 6 cases. Acta Radiol 1996; 37: 362-365.  Back to cited text no. 6    
7.Phelan JT, Juardo J, Dermatofibrosarcoma Protuberans. AM J Surg 1963; 106: 943-948.  Back to cited text no. 7    
8.Kvransdorf MJ, Meis Kindblom JM, Dermatofibro Sarcoma Protuberans-Radiologic Appearance. AJR 1994; 163: 391-394.  Back to cited text no. 8    

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Correspondence Address:
R Shrimali
Mohan Dai Oswal Cancer Hospital, G.T.Road, Sherpur By Pass, Ludhiana
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2]

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