Relapse in dermatofibrosarcoma protuberans: A histological and molecular analysis

Molina, Angie; Neto, João Pedreira Duprat; Bertolli, Eduardo; Cunha, Isabela Werneck da; Fregnani, José Humberto Tavares Guerreiro; Figueiredo, Pedro H M; Soares, Fernando Augusto; Macêdo, Mariana Petaccia de; Lopes, Clovis A. Pinto; Filho, Ivan Dunshee de Abranches Oliveira Santos

doi:10.1002/jso.25039

Cited by 14 publications

(9 citation statements)

References 41 publications

(64 reference statements)

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“…DFSP is a slowly growing mesenchymal neoplasm, characterized by high morbidity due to its local invasion and high recurrence rate after surgical excision [12,13]. The peak age at onset is between 20 and 50 years, and incidence among women is 1.14 times higher than in men [14].…”

Section: Discussionmentioning

confidence: 99%

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Dermoscopy of Dermatofibrosarcoma Protuberans: What Do We Know?

Escobar

Ribeiro

Leite

et al. 2019

Dermatol Pract Concept

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Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon mesenchymal tumor of intermediate malignancy. Its rarity and slow progression often imply a delayed diagnosis. There are few previous reports of dermoscopic features of DFSP and most are single case descriptions. Objectives: To report 2 cases of DFSP and their dermoscopic features, as well as conduct a review of all previous cases published addressing the use of dermoscopy in this tumor. Methods: We conducted a literature search for all dermoscopic cases of DFSP. In addition, we presented 2 additional cases and compared them to the earlier findings. Results: We summarized the main dermoscopic findings of DFSP based on analysis from 32 patients. The most common features of this tumor are the presence of vessels (81%), followed by a pigmented network (78%) and a pinkish background (66%). Conclusions: DFSP can mimic benign lesions and the diagnosis may be challenging. Dermoscopy is an important tool that may enhance clinical suspicion toward the diagnosis of DFSP.

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Section: Discussionmentioning

confidence: 99%

“…Surgical excision remains the mainstay treatment for DFSP. Because of its infiltrating growth pattern, a wide excision with margins of at least 3 cm of clinically uninvolved skin is strongly advised for the treatment of DFSP [13,21,22].…”

Section: Discussionmentioning

confidence: 99%

Dermoscopy of Dermatofibrosarcoma Protuberans: What Do We Know?

Escobar

Ribeiro

Leite

et al. 2019

Dermatol Pract Concept

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“…In case of regular histopathological processing (conventional histological margin control), wider surgical margins of at least 2 cm are recommended. In a retrospective analysis of 61 DFSP patients, surgical margins of less than 2 cm were associated with a significantly increased risk of recurrence . A systematic review of 23 nonrandomized trials comparing recurrence rates in patients treated with micrographic surgery versus wide local excision (combined with conventional histological examination of margins) revealed the former to be superior (recurrence rate 1.11 % versus 6.32 %) [9].…”

Section: Treatmentmentioning

confidence: 99%

S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

Ugurel

Kortmann

Mohr

et al. 2019

J Deutsche Derma Gesell

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Summary While dermatofibrosarcoma protuberans (DFSP) is a rare cancer entity overall, it is nevertheless the most common type of cutaneous sarcoma. The tumor is of fibroblastic origin and characterized by slow, undermining and locally destructive growth. Metastatic spread is very rare. Given its nonspecific clinical appearance, diagnosis is frequently delayed. Biopsy and subsequent histopathology are key diagnostic tools. Standard treatment for primary tumors consists of complete excision with surgical margins of 1 to 2 cm. Smaller margins are associated with high local recurrence rates. Inoperable and metastatic DFSP may be treated with radiation therapy. Approximately 80–90 % of DFSP lesions harbor a fusion gene that results in continuous activation of the PDGF‐β signaling pathway. Consequently, molecular targeted therapy inhibiting PDGF‐β is an effective option for advanced (inoperable) and metastatic DFSP. The first agent to be approved for systemic treatment of DFSP is the multikinase inhibitor imatinib, showing objective response rates of about 50 % in clinical trials.

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“…Patients without this fusion had a high risk of recurrence in our series, which might be of limited significance due to the very small cohort ( n = 2). Molina et al 39 reported a similar result in adult DFSP, though. As the fusion turns on the PDGF signaling, the PDGF inhibitor imatinib was recommended as an alternative treatment for unresectable, metastatic or recurrent DFSP.…”

Section: Discussionmentioning

confidence: 75%

Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease—Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)

Krewer

Rolle

Kościelniak

et al. 2020

Journal of Surgical Oncology

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Background Dermatofibrosarcoma protuberans (DFSP) is a rare low‐grade tumor. Little is known about best treatment of primary and relapsed disease (RD). Methods Treatment and outcome of 40 patients with DFSP prospectively registered within the CWS‐96 and ‐2002P trials and the registry SoTiSaR (1996‐2016) were analysed. Results Median age was 8 years (range, 0.64‐17.77). Fluorescence in situ hybridization analysis to detect COL1A1‐PDGFB fusion genes was positive in 86% (12/14) of evaluated patients. Primary resection was performed in all patients. Patients had IRS group I (n = 28), II (n = 9), and III (n = 2); not available (n = 1). To achieve complete remission (CR), a secondary resection was performed in 18 patients resulting in microscopically complete (R0, n = 34/40) and microscopically incomplete (R1, n = 5/40) resection. All patients achieved CR. The 5‐year event‐free survival (EFS) and overall survival was 86% (±12; CI, 95%) and 100% (±0; CI, 95%), respectively. R0 resection/IRS I was significantly favorable for the 5‐year EFS. Local relapse occurred after a median time of 1.1 years (range, 0.04‐5.1) in 15% (6/40) after CR. All patients with RD underwent resection and achieved CR. Three patients had fibrosarcomatous DFSP, two were alive after R0 resection. Conclusion Complete surgical resection is mandatory to prevent relapse of DFSP.

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Relapse in dermatofibrosarcoma protuberans: A histological and molecular analysis

Cited by 14 publications

References 41 publications

Dermoscopy of Dermatofibrosarcoma Protuberans: What Do We Know?

Dermoscopy of Dermatofibrosarcoma Protuberans: What Do We Know?

S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease—Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)

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