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Year : 2001  |  Volume : 49  |  Issue : 1  |  Page : 81-3

Dermatofibrosarcoma protuberans of scalp : a case report.

Department of Neurosurgery, S.M.S. Medical College, Jaipur - 302017, India.

Correspondence Address:
Department of Neurosurgery, S.M.S. Medical College, Jaipur - 302017, India.

  »  Abstract

A case of dermatofibrosarcoma protuberans of scalp involving the underlying bone, operated after recurrence by taking safety margin of 3 cm and skin deficit covered by transposition flap, is being reported. Modality of treatment has been discussed.

How to cite this article:
Sinha V D, Dharker S R, Kalra G S. Dermatofibrosarcoma protuberans of scalp : a case report. Neurol India 2001;49:81

How to cite this URL:
Sinha V D, Dharker S R, Kalra G S. Dermatofibrosarcoma protuberans of scalp : a case report. Neurol India [serial online] 2001 [cited 2023 Feb 3];49:81. Available from: https://www.neurologyindia.com/text.asp?2001/49/1/81/1294

   »   Introduction Top

Dermatofibrosarcoma protuberans is a rare skin tumour with a pronounced tendency to local recurrence.[1] It is slow growing, locally aggressive fibrous tumour, rarely metastasising to regional lymph node or distant sites.[2] It seems that the relatively infrequent occurrence of dermato-fibrosarcoma protuberans lessens their clinical awareness. The condition is mostly diagnosed on microscopic examination.[3] Recognition of this tumour is important because of the excellent prognosis after adequate surgical excision, ensuring adequate margin of 3 to 5 cm. We are reporting a case of dermatofibrosarcoma along with modality of treatment.

   »   Case report Top

A 45 year old male presented with a large gradually increasing swelling in parietal region of one year duration. The swelling was non-tender, firm, non transilluminant and non pulsatile. Impulse on cough was absent. Skull X-ray did not reveal any bony involvement. CT Scan showed soft tissue density mass in parietal region extending on either side with enhancing peripheral and central necrotic component. However, the underlying cranium was uninvolved [Figure - 1]. It was a pseudo-capsulated, firm, vascular mass, extending on either side, in parietal region. Total gross excision of the tumour was done. Biopsy report was suggestive of dermatofibrosarcoma protuberans. Microscopically, there were fascicles of spindle shaped cells (fibroblast) arranged in storiform pattern [Figure - 2]. Patient was again admitted after 6 months, with fast growing swelling in the same area. Mass was similar to the primary swelling but was larger in size. The overlying skin was stretched with vessels shining through it. CT scan head showed swelling in parietal region extending on either side with erosion of the outer table of skull without intracranial extension.Tumour was excised taking 3 cm of normal skin margin. The underlying bone was also removed along with margin of 3 cm. Primary cranioplasty was not done. Two transposition flaps from fronto temporal area were elevated to cover skin defect. Skin grafting was done over donor area. There was no evidence of regrowth on follow up after four months.

   »   Discussion Top

Hoffmann,[4] first named this tumour as dermatofibrosarcoma protuberance. Other terms used to describe this neoplasm are hypertrophic morphea, progressive and recurrent dermatofibroma, fibrosarcoma of skin and sarcomatous tumour resembling keloid.[2],[3],[5] Age at onset of the disease ranged from 6-65 years[3] while the maximum incidence is in 2nd and 3rd decade. The majority of patients are under 40 years of age at the time of diagnosis.[1] The tumour has marked predilection for the trunk, although, no anatomical region is spared except for hands.[3] Head and neck regions are rarely involved. Burkhardt[5] reported six cases involving the scalp out of 56 cases of dermatofibrosarcoma protuberance. Tamoney,[6] in review of cases of dermatofibrosarcoma, found head and neck involvement is 40 out of 261 cases. Mbonde[2] however, reported higher incidence of the tumour in head and neck region and lower limbs, implicating trauma as the probable cause.
The tumour tends to appear first as a single firm, fibrous nodule in skin, with a pink or violaceous hue, which is freely mobile on deeper structures. It enlarges by expansile growth, while the periphery of tumour infiltrates the adjacent skin and subcutaneous tissue.[3] After few years, overlying skin may ulcerate because of stretching and atrophic changes, which increases the susceptibility to trauma, resulting in superficial ulceration and bleedings.
Mc Peak[3] reported two patients with disease of scalp, who succumbed to direct invasion of skull and brain following incomplete removal of the tumour. Taylor and Helwing[7] emphasised an arrangement of tumour cells in a cartwheel pattern, which they believed to be histologically characteristic. Microscopic examination of tumour revealed a subepidermal fibrotic plaque to be present in 71%.[5] The tumour was composed of uniform spindle cells, variable number of vascular spaces and collagen, giving the appearance of spiral nebular or so called 'spokes wheel'. A high mitotic rate is an ominous finding in dermatofibrosarcoma protuberans, but a low mitotic rate does not preclude the possibility of metastasis.[1]
Taylor[7] reported 49% local recurrence rate following simple excisions. Mc Peak[3] reported recurrences in 80% cases within first three years. In our case, the patient first underwent simple excision of the lesion with recurrence within 6 months. The local recurrence of the tumour stems from its infiltrative capability, that is not widely appreciated. Condensation of connective tissue at the periphery may give a false appearance of encapsulation but actually, tumour may extend well beyond margins in fine microscopic projection.[3] The tumour remains unchanged over long period of time. It is possible that any manipulation in the form of inadequate excision, which cuts through the tumour and simultaneously opens vascular channels, may be essential for vascular or lymphatic spread. Distant metastasis reported by Burkhardt[5] and Mc Peak[3] by haematogenous route are seen in 4-6% of cases, while Przybora[8] reported metastasis in 10% of cases, through lymphatic route. Taylor[7] could not find even a single case of distant metastasis in his large series of 115 cases. Brenner et al[1] reviewed the world literature and found the incidence of metastasis to be 5.7%. 4.2% were haematogenous and 1.4% were lymphatic in origin.
In the opinion of all reviewers, the treatment of choice is wide surgical excision ensuring adequate margins of 3 to 5 cms, followed by immediate recons-truction.[9],[10] Lobay[9] recommended single stage radical excision of scalp and underlying bone and immediate reconstruction with cranioplasty and free skin flap coverage. Mohs micrographic surgery may be the treatment of choice for facial and scalp dermatofibrosarcoma protuberans since this technique enhances reconstruction options and decreases the chance of recurrence of this uncommon tumour.[11] Koshima et al[12] described wide excision of dermatofibrosarcoma protuberans and reconstruction of the defect with scarpas adipofacial flap.
The ability to provide single stage reconstructions of large defects of scalp and underlying cranium represent a considerable advance in cranioplasty. It has resulted from the development of microsurgical techniques and the cooperation of plastic surgical and neurosurgical teams.[9] Immediate reconstruction of scalp and underlying occipital bone using an acrylic cranioplasty and free omental transfer recommended by Harashina et al[13] was not successful. Dermatofibrosarcoma protuberans is not radiosensitive.
Lymph node metastasis reported is not a justification for elective prophylactic lymph node dissection in all the cases. Lymph nodes however, require examination during a protracted period of follow up. When regional lymph node metastasis is discovered in the absence of systemic metastasis, radical regional lymph node dissection should be done.[1] We also treated recurrence of tumour by wide excision and skin flap rotational technique with the help of plastic surgeon. Patient did not show any evidence of recurrence in the follow up of 4 months later.


  »   References Top

1.Brenner W, Schaefler K, Chhabra H et al : Dermatofibrosarcoma protuberans metastatic to a regional lymph node. Report of a case and review. Cancer1975; 36 : 1897-1902.   Back to cited text no. 1    
2.Mbonde MP, Amir H, Kitinya JN : Dermatofibrosarcoma protuberans : a clinicopathological study in an African population (review ). East Afr Med J 1996; 73 : 410-413.   Back to cited text no. 2    
3.Mc Peak CJ, Cruz T, Nicastri AD : Dermatofibrosarcoma protuberans - An analysis of 86 cases - Five with metastasis. Ann Surg 1967; 166 : 803-816.   Back to cited text no. 3    
4.Hoffmann E : Uber das knollentreibende fibrosarkom der Haut (Dermatofibrosarkoma protuberans). Dermatol Z 1925; 43 : 1-28.   Back to cited text no. 4    
5.Burkhardt BR, Soule EH, Winkelmann RK et al : Dermatofibrosarcoma protuberans. Study of fifty six cases. Am J Surg 1966; 111 : 638-644.   Back to cited text no. 5    
6.Tamoney HJ Jr : Dermatofibrosarcoma protuberans in review of twelve cases. Conn Med 1971; 35 : 674-679.   Back to cited text no. 6    
7.Taylor HH, Helwing EB : Dermatofibrosarcoma protuberans. Cancer1962; 15 : 717-725.   Back to cited text no. 7    
8.Przybora LA, Wojnerowicz C : Malignancy of Dermatofibrosarcoma protuberans and report of two cases with lymph gland metastases. Oncologia1959; 12 : 236.   Back to cited text no. 8    
9.Lobay GW, Bryce Weir, Robert Carter : DFP of the scalp treated by radical excision, emmediate cranioplasty, and free groin flap : case report. J Neurosurg 1981; 55 : 640-642.   Back to cited text no. 9    
10.Parker TL, Zitelli JA : Surgical margins for excision of dermatofibrosarcoma protuberans . J Am Acad Dermatol 1995; 32 : 233-236.   Back to cited text no. 10    
11.King M, Ocheitree I : Dermatofibrosarcoma protuberans treatment and reconstruction : a case study. Plasticsurgical Nursing 1996; 16 : 77-82.   Back to cited text no. 11    
12.Koshima I, Inagawa K, Jitsuiki Y et al : Scarpa's adipofascial flap for repair of wide scalp defects. Ann Plast Surg 1996; 36 : 88-92.   Back to cited text no. 12    
13.Harashina T, Nakejima T, Maruyama Y : Delto vertibral flap to cover a large scalp defect : case report. Plast Reconstr Surg 1997; 59 : 851-854.   Back to cited text no. 13    


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