Dermatofibrosarcoma protuberans: what is the best surgical approach? (original) (raw)
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Multidisciplinary approach to the management of dermatofibrosarcoma protuberans
Journal of the American Academy of Dermatology, 2012
Background: Dermatofibrosarcoma protuberans (DFSP) is the most common cutaneous sarcoma. Tentacle-like extensions of neoplastic cells create a high incidence of local recurrence and pose challenges to resection and reconstruction. Objective: Here we present a multidisciplinary approach to the management of DFSP incorporating the expertise of a Mohs micrographic surgeon, surgical oncologist, dermatopathologist, and plastic surgeon. Methods: This was a single-institution, retrospective review of a prospectively maintained database of 19 consecutive patients who underwent resection and reconstruction of a DFSP from 1998 to 2010. All patients underwent Mohs micrographic surgery for mapping of peripheral margins (stage I excision), followed by wide local excision for delineation of the deep margin (stage II excision). Procedures were performed in consultation with a dermatopathologist who confirmed tumor-free margins, and a plastic surgeon who performed immediate reconstruction after the wide local excision (stage II reconstruction). Results: Nineteen patients were included in this study. The average number of Mohs stages required for clearance of peripheral margins was 2.7 6 0.7. The mean time between stage I and II procedures was 16 6 11 days. The average defect size after the stage II operation was 87.3 cm 2 (range, 9-300 cm 2). There were no cases of tumor recurrence. Mean follow-up time was 17 months (range, 1-53 months). Limitations: This is a retrospective review of a single-institution experience. Conclusion: A multidisciplinary approach to the management of DFSP optimizes both oncologic and reconstructive outcomes, minimizing the risk for local recurrence and limiting the functional and cosmetic morbidity associated with surgical resection.
Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans
Journal of the American Academy of Dermatology, 1997
Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft-tissue tumor of the skin; its microscopic extent of invasion beyond the grossly visible tumor is frequently difficult to appreciate. Although wide local excision has been the standard treatment of DFSP, recurrence rates range from 11% to 53%. Because Mohs micrographic surgery allows the extent of excision to be tailored to the microscopic extent of tumor, we evaluated this technique for the treatment of primary and recurrent DFSR Objective: Our purpose was to determine the local recurrence rate and microscopic extent of spread of primary and recurrent DFSP after treatment with Mohs micrographic surgery. Methods: The records of 58 patients with primary and recurrent DFSP treated with Mohs micrographic surgery at three institutions were reviewed and the macroscopic and microscopic extents of tumor were recorded. Results: One patient with a twice-recurrent DFSP had another recurrence after Mohs micrographic surgery, for an overall local recurrence rate of 2% (zero for primary tumors and 4.8% for recurrent tumors). There were no cases of regional or distant metastases. Macroscopic tumor size ranged from 0.3 x 0.6 cm to 30 x 20 cm, whereas microscopic (postoperative) size ranged from 1.8 x 1.0 cm to 35 x 40 cm. We calculated the likelihood that a given width of excision around the macroscopic tumor would clear the entire microscopic extent of tumor. Standard wide excision with a width of 1 cm around the primary tumor would have left microscopic residual tumor in 70.7%; a width of 2 cm, 39.7%; 3 cm, 15.5%; and 5 cm, 5.2%. Even an excision width of 10 cm would not have cleared the microscopic extent of some tumors, despite taking a huge excess of normal tissue. Conclusion: Treatment of primary and recurrent DFSP by Mohs micrographic surgery results in a low recurrence rate because of the ability of the technique to permit the detection and excision of microscopic tumor elements in even the most asymmetric rumors. Whatever type of surgery is chosen to treat DFSR it is necessary to assess the entire perimeter of the tumor for microscopic extension and to achieve tumor-free margins in all directions.
Cancer, 2004
BACKGROUND. Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor of the skin. Clinically, it often masquerades as a benign, indolent tumor on the trunk and extremities. Microscopically, it extends far beyond assessed clinical margins, spreading locally in the dermis, subcutaneous tissue, and muscle. The local recurrence rate in patients with DFSP who undergo wide local excision ranges from 0% to 21%. Recent preliminary reports indicate more consistently favorable cure rates with Mohs micrographic surgery (MMS). However, to date only a few scattered reports have documented long-term 5-year follow-up. The authors present data on 29 patients with DFSP who underwent MMS. In addition, they reviewed the medical literature to summarize the accumulated experience of MMS treatment in the management of DFSP. METHODS. The authors conducted a retrospective review of a series of 40 consecutive patients with DFSP who underwent MMS over the last 20 years. Of these, there were 29 patients with Ͼ 5 years of follow-up who formed the basis of the current review. The literature also was searched for patients with DFSP who underwent MMS with Ͼ 5 years of follow-up RESULTS. At the University of Wisconsin Mohs Surgery Clinic, 29 patients with Ͼ 5 years of follow-up were treated. There were 16 women and 13 men. Eight patients developed recurrent disease after previous non-Mohs treatment. Site distribution was 45% head and neck and 55% trunk and extremities. In the current series, there were no local recurrences, with a local 5-year cure rate of 100%. In the literature review, which included the current series, there were 136 patients with DFSP who underwent Mohs surgery with Ͼ 5 years of follow-up. Nine patients in the current series developed local recurrences, including five patients who underwent a second Mohs procedure. The local cure rates after the first and second Mohs surgeries were 93.4% and 98.5%, respectively. The rate (percent) and time to local recurrence was 50% at 3 years and 75% at 5 years. However, 25% of local recurrences appeared late, after the usual 5-year recommendation. CONCLUSIONS. In a series of 29 patients with of DFSP and in an accompanying update of the medical literature, 136 patients with DFSP underwent MMS with Ͼ 5 years of follow-up. There were no regional and/or distant metastases. However, late recurrences beyond the usual recommended 5-year follow-up may occur. Therefore, all patients with DFSP, especially those with recurrent tumors, should be followed for an extended period. The accumulated data continue to confirm that, when DFSP is discovered early and is accessible readily to excision by MMS, a favorable outcome can be expected with minimal trauma or sacrifice of adjacent normal structures and with a low recurrence rate.
Surgical Treatment of Dermatofibrosarcoma Protuberans
Plastic & Reconstructive Surgery, 1997
Dermatofibrosarcoma protuberans is a rare dermal tumor that recurs after inadequate primary treatment. In a retrospective study, we analyzed the outcomes of 117 patients (mean age 39 years) treated surgically for a dermatofibrosarcoma protuberans. In most cases (107 patients), surgery was performed according to a protocol of taking wide peripheral resection margins of 5 cm and by resecting a disease-free anatomic zone deep to the lesion. The mean follow-up was 61 months. The results suggest a difference in prognosis between patients treated primarily with wide initial resection and those referred secondarily with recurrent disease following previous treatment by narrow resection margins. There was no recurrent disease in the 66 patients treated primarily by wide peripheral (5 cm) and deep resection of the tumor. Of the 41 patients referred secondarily at the time of recurrence, 2 developed further local disease within a year, despite equally aggressive local treatments. Both patients eventually died of metastatic fibrosarcoma. We emphasize the value of a highly aggressive local resection in the primary treatment of dermatofibrosarcoma protuberans to minimize local recurrence and potential malignant transformation. Modern reconstructive techniques provide satisfactory solutions for defects of almost any size and composition. Our findings suggest that radical primary resection of dermatofibrosarcoma protuberans in conjunction with immediate reconstruction of the primary defect provides the best outcome for the patient.
Dermatofibrosarcoma protuberans: wide and deep block excision including underlying muscle
European Journal of Plastic Surgery, 1996
Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of the skin, which has a propensity for local recurrence. Surgery is the only option of treatment and resection with wide surgical margins including the underlying fascia has become the standard treatment. Two cases of DFSP located on the trunk and forearm are presented who were managed with 3 cm tumor free lateral margins and in depth resection including underlying muscles. The patients were found to have no tumors after 3 and 4 years follow-up. This aggressive approach including excision of underlying muscles is advocated for DFSP located on the trunk or extremities to prevent local recurrence.
Dermatofibrosarcoma protuberans: A 10-year experience
Formosan Journal of Surgery, 2015
Background: Providing soft-tissue coverage after wide excision of dermatofibrosarcoma protuberans (DFSP) is always challenging; according to the literature, a skin graft is often chosen as the first option. However, possible suboptimal functional and cosmetic results have been noted. Aims and objectives: We present our 10-year experience using pedicled or free flaps for reconstruction after a wide excision of DFSP to provide adequate soft-tissue augmentation and enhanced esthetic results. Materials and methods: This was a retrospective study comprising 14 DFSP patients who were treated between February 2003 and December 2013. All patients underwent a wide excision with a 3-cm safe margin with immediate reconstruction, and a negative deep margin confirmed with a frozen section. The reconstruction method included nine pedicled perforator flaps and five free perforator flaps. All patients received adjuvant radiotherapy after surgery. Results: The peak incidence that occurred in this series was the highest in patients younger than 30 years. None of the 14 patients exhibited recurrence, and the mean follow-up time was 30 months. Half of the patients exhibited DFSP distributed over the trunk; the patients in the series were predominantly male. A total of 13 flaps were successful except for one pedicled flap that failed from venous congestion; we used it in a salvage procedure by using full-thickness skin graft for coverage. Conclusion: A wide excision with a 3-cm margin of safety with immediate reconstruction is a reliable method for DFSP resection. Initiating adjuvant radiotherapy might reduce the chance of local recurrence. To minimize the complications of skin graft, pedicled, or free flaps provide superior functional outcome, soft-tissue augmentation, and esthetic results.
Journal of the European Academy of Dermatology and Venereology, 2013
BackgroundDermatofibrosarcoma protuberans (DFSP) is characterized by unpredictable subclinical extension, meaning that positive margins are frequently detected following conventional surgical excision.ObjectiveTo study the presence or absence of residual tumour in DFSP with positive margins after conventional surgery and identify possible predictors of residual tumour or clear margins following a single Mohs micrographic surgery (MMS) stage.MethodsA retrospective study of patients with DFSP and positive margins following conventional excision referred for MMS was performed. We studied gender, age, tumour site, time from presentation to diagnosis, and affected margins.ResultsWe studied 58 cases, 35 (60.3%) of which had histological evidence of residual tumour. Tumours of the head and neck were significantly associated with the persistence of tumour. A single MMS stage was sufficient to achieve clearance in the majority of cases (n = 46). All tumours with lateral involvement only were...
Dermatofibrosarcoma protuberans: Role of wide local excision
South Asian Journal of Cancer, 2013
Objectives: The main objective of the present study was to study the outcome of surgical treatment of dermatofibrosarcoma protuberans. Materials and Methods: This study included 45 patients both retrospective and prospective from December 1995 to December 2010. Results: Out of 45 patients, 30 were males and 15 females with the male to female ratio of 2:1. Mean age of presentation was 38.4 + 13.2 years. Commonest mode of presentation was raised firm multinodular lesion with fixity to overlying skin. Site distribution was 42.22% trunk, 57.88% extremities and head and neck. None of the patients had lymph node involvement All patients underwent wide local excision. On histological examination, 8 patients had positive margins. Overall recurrence rate was 22.22%. (please clarify what is the difference between the rate of recurrence following surgery and the overall recurrence rate) Only 2 patients developed metastasis to lungs in the course of their follow‑up. Out of 45 patients, 35 remai...