A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

Wang, Yihua; Wang, Yu; Chen, Rui; Tang, Zhenrong; Liu, Shengchun

doi:10.1155/2020/8852182

Cited by 10 publications

(19 citation statements)

References 58 publications

(73 reference statements)

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“…Interestingly for DFSP of the breast, there are no case reports that describe use of MMS for resection, very few case reports that describe reconstruction and its complications following resection, and no studies that compare complications between primary closure and reconstruction [ 8 ]. In addition to primary closure, reconstruction techniques cited in the literature include pedicled latissimus dorsi flap, rotation flap, reverse abdominoplasty, pectoralis flap, and reduction mammoplasty to provide wound coverage and to preserve breast shape [ 25 - 30 ].…”

Section: Discussionmentioning

confidence: 99%

“…An additional challenge is balancing adequate resection for margin control and the aesthetic goals of symmetry, especially with breast lesions. The present literature on DFSP of the breast is scarce, with single institution case series describing 1–6 patients resected by WLE [ 8 ]. Although there are no established guidelines for breast reconstruction following DFSP resection, one case report of reconstruction following WLE recommended delaying reconstruction at least 2 years as it may prevent detection of local recurrence and will result in better aesthetic outcomes [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

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Wide local excision, Mohs micrographic surgery, and reconstructive options for treatment of dermatofibrosarcoma protuberans of the breast: A retrospective case series from Mayo Clinic

et al. 2023

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Background Dermatofibrosarcoma protuberans (DFSP) of the breast is a dermal fibroblastic neoplasm requiring wide excisional margins due to recurrence rates ranging from 26 to 60%. The current literature on reconstructive options and utility of Mohs micrographic surgery for DFSP of the breast is scarce. We describe surgical management of DFSP of the breast at our institution with the largest case series reported to date. Methods A retrospective review was performed of women who underwent surgery for DFSP of the breast at our institution between 1990 and 2019. Continuous data was summarized using mean, median, and range; categorical data was summarized with frequency count and percentage. Preoperative lesion size and postoperative defect size were evaluated using 2-sided Fisher exact test, and p-values < 0.05 were considered statistically significant. Results Nine patients underwent wide local excision (WLE) with reconstruction including pedicled latissimus dorsi flaps (n = 2), local flap advancement (n = 2), mastectomy with implant (n = 1), oncoplastic breast reduction (n = 1), and skin grafts (n = 3). Nine underwent Mohs micrographic surgery (MMS) with complex primary closure. Mean postoperative maximum wound defect size for WLE was 10.8 cm versus 7.0 cm for MMS with no statistical significance (p = 0.77). Mean preoperative maximum lesion size for WLE was 6.4 cm versus 3.3 cm for MMS with no statistical significance (p = 0.07). Complications with WLE included wound dehiscence in three patients and seroma in one patient. No complications were reported with MMS and primary closure. Recurrence was reported in one WLE patient, which was successfully detected despite flap coverage and resected without complications. Median follow-up for the patients without recurrence was 5.0 years, with two patients in MMS cohort lost to follow-up. Five-year overall survival was 100%. Conclusions MMS and WLE are both viable surgical options for managing DFSP of the breast. MMS could potentially minimize reconstructive needs due to smaller average defect size and result in fewer complications but may also result in asymmetry. Immediate flap reconstruction, especially in larger defects, can achieve excellent aesthetic outcomes for patients with DFSP of the breast without compromising detection of disease recurrence.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Wide local excision, Mohs micrographic surgery, and reconstructive options for treatment of dermatofibrosarcoma protuberans of the breast: A retrospective case series from Mayo Clinic

et al. 2023

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“…The patients were operated on with a margin of 2-4 cm and postoperative RT was applied to 2 patients. 17 In the case presented by Salim Al-Rahbi et al, firstly, an excisional biopsy was performed and wide local excision was performed after positive surgical margins. Although negative surgical margins were obtained, postoperative RT was applied to the case.…”

Section: Discussionmentioning

confidence: 99%

Dermatofibrosarcoma Protuberans and Adjuvant Radiotherapy: A Case Report with Uncertain Surgical Margins in The Breast

et al. 2021

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Background: Dermatofibrosarcoma Protuberans (DFSP) is a rare, locally aggressive superficial soft tissue tumor that can occur in many parts of the body. Surgical resection with a wide margin of safety is the main treatment modality of this rare tumor of the breast. According to the postoperative pathology report, the patient can be followed up or adjuvant radiotherapy (RT) can be added. Case presentation: A 22-year-old woman presented with a mass filling the lower inner quadrant of her right breast. Tru-cut biopsy revealed a mesenchymal tumor, but excision was recommended for definitive diagnosis. A right breast quadrantectomy was performed. The result came as DFSP. Tumor diameter was 10x9x6.5 cm and the tumor was positive in most of the surgical margins. The patient underwent re-resection and a residual tumor with a diameter of 0.2 cm was detected at a distance of 3.3 cm from the surgical margin. Although the surgical margins were negative, the distance of the posterior surgical margin, in particular, could not be assured. Because of the uncertainty of surgical margins, 60 Gy RT was planned. Conclusion: The localization of DFSP in the breast is extremely rare and surgery is the primary treatment. RT should be added as an adjuvant when safe surgical margins cannot be obtained.

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“…Outcomes are significantly worse when DFSP undergoes fibrosarcomatous (FS) transformation (FS-DFSP), which is associated with an increased risk of metastasis, reduced CD34 staining, and a herringbone pattern with increased atypia and mitoses. 3,4 A systematic review of outcomes for FS-DFSP and DFSP showed that the risk of local recurrence, metastasis, and death from disease in FS-DFSP was significantly higher as compared to DFSP (local recurrence 29.8% vs 13.7%, risk ratio 2.2, 95% confidence interval [CI] 1.7-2.9; metastasis 14.4% vs 1.1%, risk ratio 5.5, 95% CI 4.3-7.0; and death from disease 14.7% vs 0.8%, risk ratio 6.2, 95% CI 5.0-7.8). 4 In the United States, the overall annual incidence of DFSP was 4.1 persons per million person-years in 2000 to 2010.…”

Section: Introductionmentioning

confidence: 99%

Platelet-Derived Growth Factor-D Fusion-Positive Dermatofibrosarcoma Protuberans: Case Report of an Atypical Breast Mass and Literature Review

Chandler¹,

Jing²,

David³

et al. 2023

Int J Surg Pathol

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Dermatofibrosarcoma protuberans (DFSP) is a rare, CD34+ mesenchymal neoplasm that classically involves the dermis. A COL1A1::PDGFB t(17;22) translocation is present in 91.4% to 96% of cases, resulting in aberrant proliferation due to tyrosine kinase hyperactivity. Here, we present a postmenopausal woman with a CD34-positive spindle cell neoplasm of the breast without cutaneous involvement, lacking muscle marker expression, STAT6 expression, and 13q14 deletion by fluorescence in situ hybridization (FISH). Although the classic PDGFB translocation was not detected by FISH, the overall features were highly suspicious for DFSP. Subsequent RNA-based next-generation sequencing revealed an EMILIN2::PDGFD fusion. A literature review showed that PDGFD fusions can be detected in up to 55% PDGFB FISH negative cases, with EMILIN2::PDGFD fusion highly associated with fibrosarcomatous transformation. This holds important diagnostic and prognostic information as fibrosarcomatous-DFSP is associated with higher recurrence and metastatic potential. The tumor was completely resected with clear margins, showed no fibrosarcomatous areas, and no evidence of recurrence is documented 2 years since resection. This review and case report adds to the literature regarding PDGFD-translocation positive DFSP as a differential diagnosis of CD34-positive spindle cell tumors of the breast, while emphasizing the prognostic importance of EMILIN2::PDGFD fusions.

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A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

Cited by 10 publications

References 58 publications

Wide local excision, Mohs micrographic surgery, and reconstructive options for treatment of dermatofibrosarcoma protuberans of the breast: A retrospective case series from Mayo Clinic

Wide local excision, Mohs micrographic surgery, and reconstructive options for treatment of dermatofibrosarcoma protuberans of the breast: A retrospective case series from Mayo Clinic

Dermatofibrosarcoma Protuberans and Adjuvant Radiotherapy: A Case Report with Uncertain Surgical Margins in The Breast

Platelet-Derived Growth Factor-D Fusion-Positive Dermatofibrosarcoma Protuberans: Case Report of an Atypical Breast Mass and Literature Review

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