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CASE REPORTS
Dermatofibrosarcoma Protuberans
Arshad S Khan, Girish D Bakhshi, Vaibhav A Thakare, Nitin S Gothwal, Lata D Bhandarkar
Dermatofibrosarcoma protuberans (DFSP) is a rare variety of mesenchymal tumour arising from dermis. It is locally aggressive and highly recurrent malignant neoplasm. We present a case of 51 year old female diagnosed to have DFSP over interscapular area. Patient was treated surgically by doing wide excision with primary skin grafting. Post-operative follow-up of 1 year has shown her to be clinically disease free with no evidence of recurrence.
INTRODUCTION
Primary mesenchymal tumours with localization outside the skeleton, parenchymatous organs or hollow viscera are generally referred to as soft tissue sarcomas. Dermatofibrosarcoma protuberans (DFSP) is a relatively unusual, highly invasive locally aggressive cutaneous tumour of intermediate malignancy. DFSP was first described by Darier and Ferrand as a distinct clinical entity in 1924. However, Hoffman officially coined the term dermatofibrosarcoma protuberans in 1925.1
In constitutes less than 0.1% of all cutaneous malignancies and 6% of all soft tissue sarcomas. The incidence has been estimated at 0.8 cases per million persons per year.2 DFSP most commonly occurs in early to middle adult life between 20 and 50 years of age, although its range of occurrence is from birth through the 80’s.2 Although metastasis rarely is seen, DFSP is a locally aggressive tumour with a high recurrence rate.3 We present a case of 51 year female with DFSP with modality of treatment.
CASE REPORT
We present a case of 51 year old female who presented with a swelling in interscapular region since 2 years. Swelling had progressively increased in size and had ulcerated resulting in pain and bleeding. Clinical examination revealed it to be tender, firm, nodular swelling measuring 8 cms X 5 cms with areas of skin ulceration (Fig. 1). Swelling was mobile and not fixed to the underlying muscles. Biopsy done in a private hospital showed it to be DFSP. Patient was worked up for surgery and wide excision with primary skin grafting was done. Postoperatively patient recovered uneventfully. Histopathology of the excised specimen showed plump fibroblasts arranged in a storiform pattern (Fig. 2). The individual cells were spindle to oval in shape with oval nuclei showing nuclear pleomorphism and low mitotic activity which confirmed the diagnosis of DFSP. Both radial and deep margins of resection were free of tumour cells. Post-operative follow-up period of 1 year has shown her to be clinically disease free with no evidence of recurrence.
DISCUSSION
Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumour with a pronounced tendency to local recurrence.3 It is slow growing, locally aggressive fibrous tumour, rarely metastasizing to regional lymph nodes or distant sites.4 Though, DFSP was first described by Darier and Ferrand as a distinct clinical entity in 1924, it was Hoffmann who first coined the term DFSP.1 Other terms used to describe this neoplasm are hypertrophic morphea, progressive and recurrent dermatofibroma, fibrosarcoma of skin and sarcomatous tumour resembling keloid.2,4,5 The incidence has been estimated at 0.8 cases per million persons per year. Age at onset of the disease ranges from 6-65 years with maximum incidence in 2nd and 3rd decade.2 The majority of patients are under 40 years of age at the time of diagnosis.3 Our patient presented to us at 51 years of age.
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.2 After few years, overlying skin may ulcerate because of stretching and atrophic changes, which increases the susceptibility to trauma, resulting in superficial ulceration and bleeding. Metastases have also been described, but they are rare and may occur after the repeated recurrences.6 The differential diagnosis in the early phase includes a dermatofibroma and keloid. In the advanced phase the tumour’s slow growth rate, a characteristic colour and irregular margins suggest the diagnosis of dermatofibrosarcoma.6 Our patient was 51 year old female who had an interscapular swelling since 2 year which had progressively increased and had ulcerated causing pain. In our case patient presented late with classical features of DFSP.
Histologically, the neoplasm is composed of densely packed. Monomorphous spindle cells arranged in a storiform pattern.5 It has been classified as fibrohistiocytic tumour of intermediate malignancy. Routine histology and immunohistochemistry can usually distinguish dermatofibroma (DF) and dermatofibrosarcoma protuberans (DFSP). DF generally expresses factor XIIIa whereas DFSP generally expresses CD34.7 In our case tumour showed classified storiform pattern of spindle cells with low mitotic activity. Both radial and deep resection margins were free of tumour.
In general, wide surgical excision of followed by immediate reconstruction is considered necessary to avoid reappearance of the tumour.8 Recurrence rate using undefined surgical margins is estimated from 49 to 53%. It drops to 10-20% when wide surgical margins (3 cm of clinically uninvolved tissue) reaching down to the superficial fascia, are used. In our case wide resection margin was taken to prevent recurrence which was beneficial.
One of the surgical techniques used to prevent recurrence is Mohs method. The Mohs micrographic surgery is an excisional technique with complete histological margins’ control. This technique allows the complete removal of the neoplasm, examining all its margins, while conserving as much uninvolved tissue as possible.9 Radiotherapy has a limited role; it may however be useful, combined with surgery when margins are positive or even alone in cases of non-operable tumours.10 Chemotherapy is not indicated being reserved for metastatic disease.6
Our case confirms that surgical excision of this locally malignant tumour can prevent recurrence provided wide excision margins are taken while doing surgery. Knowledge of the condition, its clinical appearance, course and histopathology puts the practitioner in a better position to answer questions from concerned patients and primary caregivers, and optimize management strategies.
ACKNOWLEDGEMENT
We would like to thank Dean and Dr GB Daver Head of the Department of Surgery, Grant Medical College and JJ Group of Hospitals for granting us permission to publish this case report.
REFERENCES
1. Hoffmann E. Uber das knollentreibende fibrosarkom der Haut (Dermatofibro sarkoma protuberans). Dermatol Z 1925; 43 : 1-28.
2. Mc Peak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans - An analysis of 86 cases- five with metastases. Ann Surg 1967; 166 : 803-16.
3. Brenner W, Schaefler K, Chhabra H, et al. Dermatofibrosarcoma protuberans metastatic to a regional lymph node. Report of a case an review. Cancer 1975; 36 : 1897-1902.
4. Mbonde MP, Amir H, Kitinya JN. Dermatofibrosarcoma protuberans : a clinicopathological study in an African population (review). East Afr Med J 1996; 73 : 410-3.
5. Burchardt BR, Soule EH, Winkelmann RK, et al. Dermatofibrosarcoma Protuberans. Study of fifty six cases. Am J Surg 1966; 111 : 638-44.
6. Garcia C. Dermatofibrosarcoma protuberans. Int J Dermatol 1996; 35 : 867-71.
7. Marcelo GH, Victor GP, Nuckols JD, Burchette JL, Shea CR. Intermediate fibrohistiocytic lesions of the skin. Is there spectrum between Dermatofibroma and dermatofibrosarcoma protuberans? Am J Surg Pathol 2000; 24 : 996-1003.
8. Parker TL, Zitelli JA. Surgical margins for excision of dermatofibrosarcoma protuberans. J Am Acad Dermatol 1995; 32 : 233-6.
9. Ratner D. Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol 1997; 37 : 600-13.
10. Hass RL, Keus RB, Loftus BM, Rutgers EJ. The role of radiotherapy in the local management of dermatofibrosarcoma protuberans. Soft Tissue Tumours Working Group. Eur J Cancer 1997; 33 : 1055-60.
AFFORDABLE MALARIA TREATMENT
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The developmet of this new antimalarial, show that it is possible to run a low-cost development programme for affordable.
Lancet Infect Dis 2003; 3 : 162-68.
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