BACKGROUND



The Use of Wide Local Excision and Temporary Wound-Vac® Dressing in Treating Two Cases of DFSP

Stephen H Pearson, MD and James K Amsberry, MD

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous malignancy. The Department of Plastic Surgery at the Naval Medical Center San Diego has played a primary role in the surgical treatment of patients diagnosed with this condition. We present two recent cases in which wide local excision (WLE) and temporary negative pressure wound dressing (Wound-Vac®) were used to care for patients with biopsy-proven DFSP.

CASE1: A 50-year-old female presented to her PCM in February 2006 with a chief complaint of a “lump” on her sacrum. The lesion had been present for approximately one year and had recently grown in size. She was diagnosed with a lipoma and referred to the general surgery department for definitive therapy. At this time, it was noted that the lesion was likely not a lipoma, but rather an epidermal inclusion cyst. The patient underwent excisional biopsy under local anesthesia without complication; the results revealed DFSP and positive margins. After evaluation by a general dermatologist, she was seen by the Mohs surgeon in the department. Ultimately, in April, the patient was referred to the Department of Plastic Surgery for wide local excision, margin control by the Department of Pathology and definitive wound management by the Wound Care Clinic. The patient underwent successful wide local excision in the main operating room three weeks later. The resulting defect measured 14cm x 4cm x 3cm. A Wound-Vac® dressing was applied at the time of surgery, and the patient was discharged home on the same day. Initial dressing change occurred on POD 4, and the resulting pathology report, available on POD 7, revealed tumor-free margins. Wound management was continued with dressing changes every 3 to 4 days until POD 18 when she underwent delayed primary closure in the minor operating room. She had an uncomplicated postoperative course, and short-term follow-up to date has demonstrated no evidence of recurrent tumor.

CASE 2: A 31-year-old male pilot presented to his squadron flight surgeon (PCM) in February 2006 with a chief complaint of a 3-4 year history of a slowly enlarging, firm, scar-like lesion on his upper left chest (Figure 1). His initial diagnosis was hypertrophic scar. He was subsequently referred for dermatologic evaluation and underwent a 5mm punch biopsy. Biopsy results revealed DFSP with positive margins. After evaluation by the Mohs surgeon, he was referred to the Department of Plastic Surgery. Following a pre-operative evaluation, the patient underwent wide local excision and Wound-Vac( dressing placement in the main operating room. The resulting defect measured 12cm x 11cm x 2cm (Figures 2,3). Initial dressing change occurred on POD 3. The pathology report, available on POD 6, revealed tumor present within one millimeter of the deep central margin. The patient underwent re-excision on POD 12. The pathologic results revealed margins free from tumor. Eight days following re-excision, the patient underwent a split-thickness skin graft from the left thigh for definitive wound management. The open defect was managed in the time spanning the initial operation, re-excision and skin graft using the Wound-Vac®. Dressing changes occurred every 3-5 days during that time. The patient’s postoperative course was uncomplicated, with 100% take of the skin graft and return to full duty in May 2006. Short-term follow-up has been negative for local recurrence.

DISCUSSION: A dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor, notorious for its propensity to recur locally. Most DFSPs are considered low-grade sarcomas; however, approximately 10-15% have a high-grade sarcomatous component (DFSP-FS) and are considered intermediate grade sarcomas (1). DFSPs are locally aggressive tumors that arise from the dermis and invade deeper subcutaneous tissues. Although they metastasize in less than 5% of cases, local recurrence as high as 20% has been reported in the literature (2).

The incidence of DFSP is estimated to be 0.8-2 cases per 1 million people per year. DFSPs account for < 0.1% of all malignant neoplasms and make up approximately 1% of all soft tissue sarcomas. A pigmented variant called the Bednar tumor, notable for its dispersal of melanin-containing in an otherwise classic DFSP, is extremely rare, constituting only 5% of all DFSPs (3). DFSPs emerge most commonly in the fourth decade; nonetheless, cases have been reported among infants and the elderly (4). Patients with DFSP-FS have a median age of 56 years in one study (5). DFSP is known to occur in all races without racial predilection, with the exception of the Bednar variant, which is found more regularly in African-American patients. Most research supports a nearly equal sex distribution or a slight male predominance.

The cellular origin of DFSP is still under debate. Historically, most theories corroborate a fibroblastic origin, since DFSP cells stain evenly with vimentin and contain active endoplasmic reticulum that readily synthesizes collagen (6). Recent studies have raised the possibility that dendritic cells derived from hematopoietic progenitor cells may be the cell of origin. This is likely due to the finding of CD34 positivity in DFSP tumors. CD34-positive cells in normal skin are a separate collection of spindle-shaped cells found in a perifollicular, peri-sweat gland and perivascular distribution, as well as on subcutaneous dendritic and interstitial dermal cells. The assumption is that these dendritic cells may arise from circulating hematopoietic progenitor cells that have become skin-associated, and they correspond to cell of origin of DFSP (7). This is an area of active research on DFSP.

The clinical history and presentation of DFSP tumors are inconsistent. Initially, DFSP lesions are often ignored. Early lesions are commonly small papules or plaques with violacious or blue borders. In rare cases, these tumors can arise from a preexisting scar (8). As the tumor enlarges, it may become a lumpy nodule or continue to develop into an atrophic or sclerotic plaque; the resulting lesion is characteristically fixed to the dermis, but freely movable over deeper tissues. Lesions fixed to deeper structures are typical of advanced disease. The vast majority of tumors are superficial and less than 5 cm in diameter. Advanced, untreated DFSPs are capable of achieving substantial dimensions, resulting in bulky “protuberant” nodules, for which the tumor is named.

Several factors together often result in a delay in diagnosis of DFSP tumors. First, DFSPs are slow growing. Second, these tumors are relatively uncommon in the general practice setting. Finally, DFSP tumors have a variable presentation. In most cases, lesions are present for several years prior to definitive diagnosis. In one study of 35 patients with DFSP, symptom duration ranged from six months to 30 years, with a mean of 6.4 years (9). DFSP tumors occur most commonly on the trunk (47%), followed by lower extremity (20%), upper extremity (18%) and the head and neck (14%) (10). Definitive diagnosis is only obtained by means of sufficient tissue, usually by core needle or incisional biopsy. FNA is occasionally valuable in diagnosing recurrent disease in a previously treated patient (11).

While surgical resection is the treatment of choice for DFSP tumors, the most appropriate procedure is determined by tumor location, and to a lesser extent, tumor size. Simple excision is extremely ineffective with recurrence rates as high as 50% (12). Wide local excision (WLE) is a common and acceptable surgical approach to DFSP tumors. The ideal width of resection margin is still debated. Tentacle-like tumor projections have been found to frequently extend beyond 3cm, which has resulted in some recommendations calling for 5cm margins (13,14). Such wide margins are usually clinically unrealistic given the resulting aesthetic and functional outcome. The most common recommendation is for resection of at least 3 cm of uninvolved normal tissue, down to and including fascia (15). Despite adequate therapy with WLE, local recurrence rates range from 4-12% (16). Several relatively recent studies have demonstrated a benefit in using Mohs micrographic surgery (MMS) compared to WLE. A survey of 221 reported cases of DFSP treated with MMS reported a 2.3% overall recurrence rate (17). In addition to a lower recurrence rate, the primary benefit of MMS is the ability to conserve tissue in surgical resection, thereby aiding a more cosmetically appealing result. However, according to guidelines published by the National Comprehensive Cancer Network, wide local excision with negative margins is still within the standard of care. Post-treatment follow-up for DFSP patients includes life-long surveillance. Approximately 50% of local recurrences will arise within three years of treatment, and approximately 25% are detected after five years (18).

CONCLUSIONS: While MMS has increased in popularity, it is not without its drawbacks. The time and monetary disbursement required to adequately treat a DFSP tumor is substantial. Additionally, Mohs-trained surgeons are not as widely available as, for example, a general surgeon who could perform a wide local excision. We believe our cases demonstrate the successful use of WLE and a temporary negative-pressure wound dressing (Wound-Vac®) while waiting for final permanent pathology when treating a DFSP tumor. The benefits of this approach include: (1) a more reliable pathologic specimen, as opposed to frozen section specimens, (2) decreased wound care and dressing changes, as compared to traditional dressings, and (3) an optimal wound bed for definitive wound management. Consequently, we believe the use of WLE and temporary Wound-Vac® dressing is a suitable alternative to Mohs micrographic surgery in the treatment of DFSP tumors.

REFERENCES:

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2. Gloster HM Jr; Harris KR; Roenigk RK. A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol 1996 Jul;35(1):82-7.

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6. Allan AE; Tsou HC; Harrington A; Stasko T; Lee X; Si SP; Grande DJ; Peacocke M. Clonal origin of dermatofibrosarcoma protuberans. J Invest Dermatol 1993 Feb;100(2):99-102.

7. Nickoloff BJ. The human progenitor cell antigen (CD34) is localized on endothelial cells, dermal dendritic cells, and perifollicular cells in formalin-fixed normal skin, and on proliferating endothelial cells and stromal spindle-shaped cells in Kaposi's sarcoma. Arch Dermatol 1991 Apr;127(4):523-9.

8. Green JJ; Heymann WR. Dermatofibrosarcoma protuberans occurring in a smallpox vaccination scar. J Am Acad Dermatol 2003 May;48(5 Suppl):S54-5.

9. Lindner NJ; Scarborough MT; Powell GJ; Spanier S; Enneking WF. Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. Eur J Surg Oncol 1999 Aug;25(4):392-7.

10. Enzinger, FM, Weiss, SW. Fibrohistiocytic tumors of intermediate malignancy. In: Stamatis, G. Soft Tissue Tumors. Mosby, St Louis, MO 1988. p.252.

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15. Roses DF; Valensi Q; LaTrenta G; Harris MN. Surgical treatment of dermatofibrosarcoma protuberans. Surg Gynecol Obstet 1986 May;162(5):449-52.

16. Gloster HM Jr; Harris KR; Roenigk RK. A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol 1996 Jul;35(1):82-7.

17. Nouri K, Lodha R, Jimenez G, Robins P. Mohs micrographic surgery for dermatobibrosarcoma protuberans: University of Miami and NYU experience. Dermatol Surg 2002; 28: 1060.

18. Snow SN, Gordon EM, Larson PO, et al. Dermatobibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. Cancer 2004; 101:28.

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Figure 1. Case 2 pre-op

Figure 2. Case 2 surgical defect

Figure 3. Case 2 with Wound-Vac(

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