Subcutaneous dermatofibrosarcoma protuberans, a rare subtype with predilection for the head: A retrospective series of 18 cases

Llombart, B.; Serra‐Guillén, C.; Rubio, Luis; Nagore, Eduardo; Requena, Celia; Traves, Víctor; Calomarde, L.; Bancalari, Bernardo; Löpez‐Guerrero, José Antonio; Guillén-Barona, C.; Sanmartín, O.

doi:10.1016/j.jaad.2017.02.046

Cited by 24 publications

(25 citation statements)

References 20 publications

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“…Histologically, DFSP shows diffuse infiltration of the dermis and subcutaneous adipose tissue with tightly packed, cytologically rather uniform spindle‐shaped CD34‐positive neoplastic cells, arranged in a characteristic storiform (reminiscent of a straw mat or cartwheel) pattern. Purely subcutaneous DFSP variants are rare and usually found on the head . One feature typical of DFSP is that the tumor cells spread along the septa of the subcutaneous adipose tissue with subsequent fat entrapment.…”

Section: Diagnosismentioning

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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Section: Diagnosismentioning

confidence: 99%

S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

Ugurel

Kortmann

Mohr

et al. 2019

J Deutsche Derma Gesell

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“…It was first described by Darier and Ferrand however Hoffman officially coined the term dermatofibrosarcoma protuberans [4]. DFSP, described as a slow-growing low-grade cutaneous sarcoma [5] that often presents or has a predilection on the following sites: trunk and proximal extremities [6], less frequently on the head and neck regions [2,7,8]. DFSP has a very low potential to metastasize but with significant subclinical tentacular extensions and great capacity for local destruction as evidenced by Acosta et al [9].…”

Section: Introductionmentioning

confidence: 99%

“…This rare cutaneous tumor, constitutes of not more than 0.1% of all malignancies and approximately 1% of all soft-tissue sarcomatous tumors [8,13]. It is a locally aggressive sarcoma of intermediate malignancy that favors young to middle-aged individuals of all sexes [14].…”

Section: Introductionmentioning

confidence: 99%

The efficacy of Mohs micrographic surgery over the traditional wide local excision surgery in the cure of dermatofibrosarcoma protuberans

2019

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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 of localized disease. DFSP rarely exhibits any lymphatic or hematogenous dissemination. It is because of its high recurrence rate associated with Wide 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 based 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 were 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 and 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.

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“…Our patient was initially diagnosed with a DFSP, for which she presented to our clinic for surgical management. While the most common location of DFSP is the trunk, a recent report found that 61% of deep, or subcutaneous DFSPs are located on the head . On review of the patient's biopsy specimen, the spindle cells were seen in an alternating hypo‐ and hypercellular pattern, which raised suspicion for an SFT.…”

Section: Discussionmentioning

confidence: 99%