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CASE REPORT
Year : 1990  |  Volume : 56  |  Issue : 5  |  Page : 384-386

Dermatofibrosarcoma protuberans




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C Indira


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  Abstract 

A 45-year-old women presented with a large growth in the left groin. She was diagnosed as dermatofibrosarcoma protuberans histopathologically. Surgica resection of the tumour was done and the patient is under regular follow up to note recurrence. The huge size and the unusual location were the interesting features. The relevant differential diagnosis is discussed.


Keywords: Dermatofibrosarcoma protuberans


How to cite this article:
Indira C, Ramesh V, Misra R S. Dermatofibrosarcoma protuberans. Indian J Dermatol Venereol Leprol 1990;56:384-6

How to cite this URL:
Indira C, Ramesh V, Misra R S. Dermatofibrosarcoma protuberans. Indian J Dermatol Venereol Leprol [serial online] 1990 [cited 2020 Sep 24];56:384-6. Available from: http://www.ijdvl.com/text.asp?1990/56/5/384/3580


Dermatofibrosarcoma protuberans is a well differentiated, locally invasive tumour with infrequent metastasis and tendency to recur. We are reporting a woman with dermatofibrosarcoma protuberans in an unusual location which had attained a large size.


  Case report Top


A 45-year-old woman reported with a growth in the inguinal region of 8 months duration associated with discomfort on walking and pain and discharge of recent onset. The lesion which was initially pea sized had gradually increased to attain the present size of 12cms x 5cms. As the tumour increased the patient noticed discomfort while walking. A fortnight before reporting to us, pain and discharge resulted from ulceration on the distal part of the tumour without any history of trauma or surgical procedures. Patient was not a known diabetic or hypertensive and had attained menopause two years ago. On examination, a large multinodular, polypoidal, flesh coloured, nontender, firm growth was present in the left groin [Figure - 1] protruding medially and covering part of vulva. Three small 1 x 1 cms ulcers were present discharging seropurulent material. The tumour was fixed to the skin but was freely mobile over the underlying structures. There was no significant lymphadenopathy. Per vaginal examination revealed a normal sized retroverted uterus with free fornices. Other systems were normal.

Routine blood, urine investigations and chest x-ray were normal. X-ray of the pelvis revealed a large soft tissue mass without any foci of calcification and the bones were normal. Staphylococcus aureus Scientific Name Search  was cultured from the discharging ulcers. After controlling the secondary infection with oral antibacterial therapy, biopsy was performed. Histopathological examination revealed a normal epidermis, and in the dermis large irregularly arranged tumour cells with spindle shaped nuclei interspersed in condensed collagen were seen. Occasionally the cells were arranged in a whorl-like fashion giving the characteristic cart-wheel appearance. Atypical cells, scattered mitotic figures and sporadic giant cells were seen [Figure - 2]. The tumor was infiltrating the dermis and extending deep into the subcutis. These findings were typical of dermatofibrosarcoma. The patient was referred to a surgeon for resection and left inguinal lymphadenectomy. Post-operatively the patient is under regular follow-up for the past seven months without any signs of recurrence.


  Comments Top


Dermatofibrosarcoma protuberans (DFSP) is a rare dermal tumour beginning as a small fibrous nodule on the skin, slow growing, painless and with a tendency to recur. In our patient solitary neurofibroma, lipoma, fibrosarcoma and leiomyosarcoma were considered in the differential diagnosis. The histopathological features have to be differentiated from atypical fibroxanthoma, nodular pseudosarcomatous fascitis and fibrosarcoma. The size of the lesion, presence of normal epidermis, large spindle shaped nuclei with little pleomorphism and deeper infiltration into subcutaneous tissue support the diagnosis of DFSP. Ulceration and bleeding requiring surgical intervention is usually due to trauma, though this was not forthcoming in our patient. There are no well known aetiological risk factors. The role of trauma in initiating the condition is thought to be coincidental[1] but has been implicated in a patient with prolonged arsenic exposure[2]. DFSP can occur anywhere on the skin surface except the palms and soles. It is four times more common in men than women. The usual age of onset is the second or the third decade. The recurrence rate varies between 20%[1] to 49%[3] and generally occurs within the first three years. Both hematogenous and lymphatic spread have been described[3],[4] Our patient had no evidence of metastasis. She underwent surgery as soon as the diagnosis was established. Mac Peak[3] recommends removal of 3 cms of normal appearing skin around the lesion and this may necessitate skin grafting. Prophylactic regional lymphadenectomy is not recommended routinely as the metastatic potential of the tumour is very low. We performed inguinal lymphadenectomy as metastasis was clinically suspected due to the unusually large size of the tumour. However histopathological examination of these lymph nodes revealed only reactive hyperplasia. Moh's surgery (microscopically controlled excision of the tumor)[5],[6] has also been tried and a five year period without any recurrence has been reported.

The origin of the tumour is controversial. Tissue culture studies suggest a histiocytic origin[7]. Identification of the intermediate filaments by immunofluorescent and immunoperoxidase staining suggests a fibroblastic lineage[8]. However electron microscopy demonstrates features compatible with origin from a neural cells[9].

 
  References Top

1.Taylor HB and Helwig : Dermatofibrosarcorna protuberans. A study of 115 cases, Cancer, 1962; 15: 717-725.  Back to cited text no. 1    
2.David Shneidman and Roger Belizaire : Arsenic exposure followed by development of Dermatofibrosarcoma protuberans, Cancer, 1986; 58:1585-1587.  Back to cited text no. 2    
3.Brenner W : Dermatofibrosarcoma protuberans metastatic to a regional lymphnode, Cancer, 1975;36:1897-1902.  Back to cited text no. 3    
4.Mac Peak CJ, Cruz T and Nicastri AD: Dermatofibrosarcoma protuberans. An analysis of 86 cases, five with metastasis, Ann Surg, 1967;166:803-816.  Back to cited text no. 4    
5.June KR : Dermatofibrosarcoma protuberans resected by Moh's surgery. A five year prospective study, J Am Acad Dermatol, 1985;6:1903-1098.  Back to cited text no. 5    
6.Mikhail GR and Lynn BH: Dermatofibrosarcoma protuberans, J Dermatol Surg Oncol, 1978;481-84.  Back to cited text no. 6    
7.Ozello L and Hamels J : The histiocytic nature of Dermatofibrosarcoma protuberans. Tissue culture and EM study, Amer J Clin Pathol, 1976;65:136-148.  Back to cited text no. 7    
8.Alguacil GA, Unni KK and Goeilner Jr: Histiogenesis of Dermatofibrosarcoma protuberans. Ultrastructural study, Amer J Clin Pahtol,1968;69:427-434.  Back to cited text no. 8    
9.Hasimoto K, Brownstein MH and Jacobiek FA: Dermatofibrosarcoma protuberans. A tumour with perineural and endoneural cell features, Arch Dermatol, 1974;110:874-885.  Back to cited text no. 9    


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[Figure - 1], [Figure - 2]



 

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