Surgical Management Of A Dermatofibrosarcoma Protuberance Of The Face
A Agarwal, S Gupta, G Guha
Citation
A Agarwal, S Gupta, G Guha. Surgical Management Of A Dermatofibrosarcoma Protuberance Of The Face. The Internet Journal of Plastic Surgery. 2006 Volume 3 Number 1.
Abstract
Background: Dermatofibrosarcoma Protuberance(DFSP) is a rare cutaneous tumor of unknown etiology. The lesion frequently develops on the extremities (mostly the lower legs) trunk, and rarely above neck. It is usually asymptomatic, although pruritus and tenderness are not uncommon. DFSP is a cutaneous malignancy that arises from the dermis and invades deeper subcutaneous tissue (eg, fat, fascia, muscle, bone).
Two cases of dermatofibroma of the face presented to us of which is a pigmented variety also called bednar [6] . WE report these cases as these tumors are rare and though an entity known for about 9 decades still much is research is to be done regarding the management.
Materials & Methods : Two cases of Dermatofibrosarcoma Protuberance of face of which one was pigmented variety, presented to Plastic Surgery OPD between July 2003 to December 2005.
These cases were managed by wide excision and reconstruction by local rotational flaps to achieve cosmetically good results.
Introduction
Primary mesenchymal tumours with localization outside the skeleton, parenchymatous organs or hollow viscera are generally referred to as soft tissue sarcomas. These are slow growing tumors Dermatofibrosarcoma protuberans (DFSP) is a relatively unusual, highly invasive locally aggressive cutaneous tumour of intermediate malignancy.
Usually this is reported to occur in limbs and trunks, incidence on face is very rare. Moreover the pigmented variety is still a rarer entity. [7]
The age of patients vary between second to forth decade, but may present as late as 80 years. These tumors are locally invasive, but recurrence rate is found to be very high.
Surgery forms the mainstay of management, though radiotherapy has got some role incases of involved margins. Chemotherapy has no role in management.
History
Two female patients with exfoliative growth at face presented to us between July 2003 to December 2005.
On histopatholgy these lesions came out to be pigmented dermatofibrosarcoma protuberance a locally invasive malignant tumor which was cosmetically unacceptable in these patients.
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Tissue histopathology was done and margins were free of tumor.
Discussion
Dermatofibrosarcoma protuberans (DFSP) is a rare variety of mesenchymal tumour arising from dermis. It is locally aggressive and highly recurrent malignant neoplasm.
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.
DFSP usually presents as a large indurated plaque several centimeters in diameter. It is composed of firm, irregular nodules varying in color from flesh to reddish brown. Sometimes, it may present as a morphea-like, atrophic, sclerotic, violaceous plaque without nodularity that may ulcerate as it slowly increases in size .[2]
Histologic Findings
In the plaque type of DFSP, slender tumor cells with large, spindle-shaped nuclei are embedded fairly uniformly in the collagen stroma, parallel to the skin surface. Mitotic figures are sparse. The more characteristic findings are seen in the nodular type. These findings include the high cellularity and irregular, short, intersecting bands of tumor cells forming a storiform pattern. Also typical are cells radiating from a central hub of fibrous tissue forming a cartwheel pattern. The degree of cellular atypia is higher in nodular lesions than in plaque lesions. Occasionally, DFSP may show focal fibrosarcomatous changes with a characteristic herringbone pattern. The cellular atypia is then even more prominent with hyperchromatic nuclei and more mitotic figures.[2]
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. Chemotherapy is not indicated being reserved for metastatic disease
Ours case was managed by wide local excision with a single stage reconstruction using local rotational flaps, and has yielded good results so far with no recurrences.
Thus we can conclude that though a rare and locally invasive entity, these lesions, by knowledge of the condition, its clinical appearance, course and histopathology can be dealt with by optimized management strategies.