Outcomes in 11 patients with dermatofibrosarcoma protuberans treated with Mohs micrographic surgery (original) (raw)
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The Pan African medical journal, 2019
Usually most patients with dermatofibrosarcoma protuberans (DFSP) may present rather late when the tumor is in protuberant phase due to its rarity and indolent onset. It has a high propensity for local recurrence and destructive nature. Management of DFSP requires a biopsychosocial and Multidisplinary approach regardless of the clinical or immunohistochemical variant. Surgery is the Gold standard management o f localized disease. DFSP rarely exhibits any lymphatic or hematogenous dissemination. It is because of its high recurrence rate associated with W ide Local Excision (WLE), the introduction of Mohs micrographic surgery (MMS) has really helped in reducing the rates of recurrence of DFSP. Thus, the aim of this meta-analysis and systemic review is to advocate for MMS over WLE for DFSP and other cutaneous malignancies using DFSP as a prototype. The objective of this study were to conduct a meta-analysis on comparative surgical methods used in the cure of DFSP with regards to WLE verses MMS, to evaluate the cure rates with relation to recurrence rates, offer a recommendation on the various treatment modalities base d on the location of lesion, and use of adjuvant therapy in different clinical-medical setups. A comprehensive retrospective analysis search in EMBASE, Google Scholar and Medline (PubMed) for studies published from 2008 to 2018 containing the surgical management of DFSP with WLE verses MMS w ere reviewed. Five studies of moderate-quality evidence (level B) with a pooled patient load of 684 was analyzed and found for recurrence of DFSP after WLE and MMS to be 9.10% and 2.72% respectively after an average follow-up time for both groups of 5.32 years with a female predominance of 1.58. The trunk is the commonest site for the DFSP lesion which was at 52.80% then the upper and lower extremities zones and the head a nd neck zones at 31.75% and 15.45% respectively. The pooled adjusted odds ratio (OR) analysis indicated that there was a direct relationship with regards the reduced recurrence rate of DFSP in the MMS group compared to the WLE group (OR:0.31;95%; CI :0.17-0.56). Furthermore, there was significant association between the reduced recurrence rate with the MMS in DFSP patients with a statistical P-value of 0.0001 at 95% CI. The expected increased recurrence rate by zones was in WLE head and neck zone at 38.19% then trunk and extremities zone at 13.34%. In the MMS group it was at of 23.4% as compared to 16.0% in the head and neck zone. Mohs Micrographic Surgery (MMS) is more efficacious in the cure rate and recurrence reduction of DFSP and should be advocated for as first line therapy especially in high recurrence prone zones.
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.
Outcomes of surgery for dermatofibrosarcoma protuberans
Ejso, 2004
Background. Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma usually presenting as nodular cutaneous mass on the trunk and proximal extremity. The tumour grows slowly, typically over years. The standard treatment is wide local excision with at least a 3-cm margin. The local regional recurrence is up to 50%, emphasizing the need for wide margins for local control. A small fraction of DFSP may metastasize, but on histological examination such tumours have features of fibrosarcomas rather than DFSP.
Low recurrence rate after surgery for dermatofibrosarcoma protuberans
Cancer, 2004
BACKGROUND. Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma with a propensity for local recurrence. Treatments with wide excision, Mohs surgery, and other approaches have been reported with widely variable local control rates. The objective of this study was to review the experience with a multidisciplinary approach employing wide excision and Mohs surgery selectively in the treatment of patients with DFSP at a single academic institution over the past 10 years. METHODS. The records of 62 patients with 63 DFSP tumors who underwent wide excision, Mohs surgery, or a multidisciplinary combination approach from January 1991 to December 2000 were reviewed retrospectively. Primary endpoints included the ability to extirpate the DFSP lesion completely, the tumor recurrence rate, and the need for skin grafts or local tissue flaps. Additional objectives included defining surgical practice patterns at the authors' institution. RESULTS. Sixty-three DFSP lesions were removed from 62 patients. At a median follow-up of 4.4 years, no local or distant recurrences were detected in any patient. Forty-three lesions were treated with wide local excision, 11 lesions were treated with Mohs surgery, and 9 lesions were treated with a combination approach. Ninety-five percent of lesions that were approached initially with wide local excision were cleared histologically. Two patients (5%) received postoperative radiation for positive margins after undergoing maximal excision. Eighty-five percent of lesions that were approached initially with Mohs surgery were cleared histologically. The remaining 15% of lesions subsequently were cleared surgically with a wide excision. DFSP lesions that were approached initially with Mohs surgery tended to be smaller. Patients with head and neck lesions most often underwent Mohs surgery or were treated with a multidisciplinary combination approach (87%). CONCLUSIONS. Wide local excision with careful pathologic analysis of margins was found to have a very low recurrence rate and was used for the majority of patients with DFSP lesions at the authors' institution. Wide local excision, Mohs surgery, and a multidisciplinary combination approach, selected based on both tumor and patient factors, were capable of achieving very high local control rates in the treatment of DFSP. The evolution of a multidisciplinary approach has provided a level of expertise that no single individual could achieve for the treatment of the full spectrum of DFSP lesions at the authors' institution. Cancer 2004;100:
Annals of Surgical Oncology, 2004
Background: Dermatofibrosarcoma protuberans (DFSP) is a very rare, low-grade malignant tumor. Local recurrence is frequent after incomplete resection either because of false diagnosis or inadequate standard surgical excision. This study evaluated the clinical outcome of micrographic surgery with paraffin sectioning in the therapy of DFSP. Methods: Medical charts of 22 patients with histologically confirmed DFSP treated at our institution between 1987 and 2002 were reviewed. Results: For 13 patients with primary and 9 with recurrent disease, tumor-free excision margins could be achieved, and all patients remained free of local recurrence during a mean follow-up of 54 months. Conclusions: The results of our study and a review of the literature confirm the successful and recurrence-free treatment of DFSP with micrographic surgery as the treatment of choice for this locally invasive tumor. Particularly in recurrent lesions, we recommend the use of paraffin sectioning and three-dimensional histological evaluation as an accurate additional tool for treatment optimization.
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.
Dermatofibrosarcoma protuberans: Wide local excision vs. Mohs micrographic surgery
Cancer Treatment Reviews, 2008
Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor of the skin with high rates of local recurrence. It is debated whether Mohs micrographic surgery (MMS) involves lower recurrence rates than wide local excision (WLE). Recent preliminary reports indicate more consistently favorable cure rates with MMS. We report comparative observational data on 41 patients who underwent MMS and 38 who underwent WLE. Their data were then pooled with those available in the medical literature to obtain more precise estimates of recurrence rates with MMS and WLE. Methods: The records of 79 patients with DFSP who underwent WLE (n = 38) or MMS (n = 41) in 1990-2005 were reviewed retrospectively. The primary endpoint was tumor recurrence rate. The PubMed database was searched for DFSP case series treated with WLE or MMS, and the recurrence proportions reported for the two separate procedures were pooled. Results: Five of the 38 WLE patients (follow-up = 4.8 years) had recurrences (13.2%, 95% CI 4.4-28.1%) as opposed to none (95% CI 0-8.6%) of the 41 MMS patients (follow-up = 5.4 years). Pooling of these data with those from the literature yielded 6/463 recurrences for MMS (1.3%, 95% CI 0.5-2.8%) and 288/1394 recurrences for WLE (20.7%, 95% CI 18.6-22.9%). The relative risk of recurrence for WLE vs. MMS patients was 15.9 (95% CI 7.2-35.5). Conclusions: Significantly lower recurrence rates were recorded in our patients subjected to MMS compared with those treated with WLE. The pooled data also indicated a clear advantage 0305-7372/$ -see front matter c
Efficacy of Mohs Micrographic Surgery for the Treatment of Dermatofibrosarcoma Protuberans
Archives of Dermatology, 2012
The authors concluded they can weakly recommend Mohs micrographic surgery or similar techniques with meticulous histologic evaluation of all margins being the first-line therapy for dermatofibrosarcoma protuberans, particularly in recurrence-prone regions. The authors' conclusion reflects the evidence presented and is appropriately cautious given the small sample size and poor quality evidence available. Authors' objectives To evaluate the recurrence of dermatofibrosarcoma protuberans (DFSP) following Mohs micrographic surgery.