An unusual variant of atrophic dermatofibrosarcoma protuberans

Aragão, Simone Silva da Costa; Cardoso, Alberto Eduardo Cox; Leite, Everson José Dos Santos; Houly, Ricardo Luiz Simoes

doi:10.1590/abd1806-4841.20187049

Cited by 9 publications

(13 citation statements)

References 8 publications

(10 reference statements)

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“…Dermatofibrosarcoma protuberans (DFSP) is one of the rare, indolent, spindle cell mesenchymal sarcoma or malignancy of low-grade aggressiveness, that arises in the dermis and can extend to the deep subcutaneous tissue and can also affect other under lying structures like muscles and bones in un intervened cases and often stains positive for CD34 [1-3]. It was first described by Darier and Ferrand however Hoffman officially coined the term dermatofibrosarcoma protuberans [4].…”

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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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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“…La diferencia por sexo es controversial y algunos estudios sugieren predominio masculino (11), mientras que otros sugieren predominio femenino (8,12) y otros no muestran diferencias (13,14). Algunas variantes clínicopatológicas del tumor pueden tener particularidades en su epidemiología, como por ejemplo: el tumor de Bednar que se encuentra más comúnmente en niños y afrodescendientes (12,13), el fibroblastoma de células gigantes que es un tumor de la edad pediátrica(15) y la variante atrófica que es más frecuente en personas jóvenes (16).…”

Section: Epidemiologíaunclassified

“…Cuando las lesiones inician pueden tener las características clásicas o carecer del típico crecimiento exofítico; en este último caso el 29% de ellos inician simulando una morfea, el 42% simulando un angioma y 19% simulando atrofodermia (10). Con el tiempo es usual que las lesiones adquieran el crecimiento exofítico evidente, esto puede tardar en promedio 7,6 años (10) y sin embargo algunas lesiones se mantienen sin protruir (16). Además del DFSP clásico existen otras formas clínicas de la enfermedad, como: la variante atrófica (27), la variante pigmentada o el tumor de Bednar, la variante fibrosarcomatosa y el fibroblastoma de células gigantes (15).…”

Section: Clínicaunclassified

“…La localización en genitales se considera poco frecuente (29). Algunas variantes podrían tener una distribución diferente; el DFSP mixoide tiene predilección por las extremidades (30) y la variante atrófica puede verse más comúnmente en el dorso (16). Los niños tienen particularidades en la localización; son frecuentes en ellos las lesiones acrales, que pueden verse en el 15,3% de los casos (28).…”

Section: Clínicaunclassified

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Dermatofibrosarcoma protuberans

Barrera¹,

Acosta

Ramirez

2019

Rev. colomb. cancerol.

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El dermatofibrosarcoma protuberans (DFSP) es un sarcoma cutáneo de baja incidencia y lento crecimiento que afecta principalmente personas de los 20 a los 39 años y es más frecuente en afrodescendientes. La localización anatómica más común es el tronco, seguido de las extremidades superiores. La etiología no está clara, pero la mayoría de los casos presentan rearreglos genéticos que involucran los genes COL1A1 y PDGFB. El riesgo de metástasis es bajo, pero tiene tendencia a la recurrencia local, especialmente en los tumores resecados con márgenes insuficientes, en los tumores profundos y los que presentan cambio fibrosarcomatoso. La primera línea de manejo para la enfermedad local es la cirugía; resección local amplia o cirugía micrográfica de Mohs (CMM). Esta última técnica ha mostrado recurrencias consistentemente bajas. La radioterapia se ha usado para tumores irresecables o como adyuvancia. El imatinib es de utilidad para enfermedad metastásica.

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“…DFSP is a low‐grade slow‐growing malignancy with relative aggressiveness that mostly invades locally to deep subcutaneous tissues or in case of intervention to underlying bone or muscles . Distant metastases are an unusual event but not impossible …”

Section: Introductionmentioning

confidence: 99%

An experience of slow‐Mohs micrographic surgery for the treatment of Dermatofibrosarcoma protuberans: A long‐term cohort study

Asilian

Honarjou

Faghihi

et al. 2020

J of Cosmetic Dermatology

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Background Dermatofibrosarcoma protuberans (DFSP) is a rare dermal mesenchymal tumor known as a low‐grade, slow‐growing malignancy. The local invasion and high rate of recurrence following surgical treatment are the main concerns to plan the best surgical approach of treatment. Aims In the current study, it is aimed to provide an experience of slow‐Mohs surgery for the treatment of patients with DSFP. Patients/Methods Number of 25 patients with the diagnosis of DFSP through histological and immunostaining study was included. The slow‐Mohs was performed by excision of the tumor with margins accounting for 1–2 cm from both the tumor margins and three‐dimensional thickness. The obtained tissue margins were horizontally, and if any of the specimens was not margin‐free, the procedure was repeated. The patients' opinion about the procedure was assessed using Patient‐Observer Scar Assessment Scale (POSAS). Results Number of 25 patients were included and followed for a median of 46.9 months. The median of the area of excision was 35.56 cm2, and the median clinical excision margins were 19 mm (tumor excision margins + thickness of the three‐dimensional excision). The surgery was performed once for 16 (64%), and postoperative skin closure within 5–7 days after the procedure was performed for 19 (76%) patients. None of the patients represented any recurrence. The patients' overall opinion and satisfaction POSAS score accounted for 2.3 ± 1.65 and 1.6 ± 0.59, respectively. Conclusion The findings of the current study are in favor of slow‐Mohs surgery for the management of DFSP, while more extensive studies are strongly recommended for generalization of this procedure.

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An unusual variant of atrophic dermatofibrosarcoma protuberans

Cited by 9 publications

References 8 publications

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

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

Dermatofibrosarcoma protuberans

An experience of slow‐Mohs micrographic surgery for the treatment of Dermatofibrosarcoma protuberans: A long‐term cohort study

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