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Background: retrospective study to elucidate the epidemio-clinical and pathological characteristics, management and evolutional aspects of DFS with comparison with the data of the literature. Procedure: a thirty seven-year study of all cases of DFS. Results: Forty-nine cases of DFS (22 males and 27 females) were diagnosed. Mean age at diagnosis was 48.2 years. The tumor appeared before the age of 15 years in five patients (10%). This was a recurrence in 12% of cases. Five patients reported a history of trauma. The average delay before consultation was 5.6 years. A solitary nodule was the predominant clinical aspect of DFS (59.2%). Mean size was 4.7 cm. The tumor was mainly located on the trunk. The diagnosis of dermatofibrosarcoma protuberans was histologically confirmed in all cases. Immunohistochemical study was achieved in 34 cases and showed positive staining for CD34. The treatment consisted of large surgical excision in all patients. Four patients had revision surgery because of tumor margins. Eight cases (17.4%) presented a local recurrence after a mean delay of 12.5 months. Conclusion: DFS is a low-malignancy potential skin tumor. Treatment of choice is surgery. The main risk is tumor relapse. A long follow-up is then very important to detect it. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a rare slow growing skin neoplasm which is characterized by a low potential of malignancy. It rarely shows metastasis to local lymph nodes or distant sites. However, it appears locally aggressive with a tendency to peripheral tumor extensions and recurrence. While DFSP can occur in all ages, it affects in the majority of cases the patients between the third and the fifth decades of age. A high index of suspicion should be taken when facing unusual lesion with a history of slow but persistent growth. DFSP involves most commonly the trunk and extremities but can also be located at any site such as scalp, head, neck, breast, pubic area and vulva. Here, we present a case of recurrent DFSP in a 52-year-old woman with a slow enlarging mass in the upper of the thorax, between the two breasts. She was previously managed by surgery several years before for a DFSP developed at the same site. Complete resection including wide surgical margins of at least 3 cm followed by immediate reconstruction is the treatment of choice to minimize recurrence rate. Marked propensity of DFSP to recur necessitates referral to a specialized center to achieve optimal oncological resection and avoid unnecessary re-operation. However, the minimum resection margin required remains undefined. Radiation and chemotherapy have been tried with limited success, although adjuvant radiotherapy may be used in advanced cases where surgery is no longer feasible. Imatinib can contribute to disease control in patients with locally advanced or metastatic DFSP.

Résumé Objectifs L’objectif principal de cette étude est de déterminer les marges de résection nécessaires pour réaliser une exérèse complète (R0) des dermatofibrosarcomes protuberans (DFSP) cervico-faciaux par technique de Slow-Mohs en 1 re intention. L’objectif secondaire est d’étudier le taux de récidive de ces tumeurs. Matériel et méthodes La technique de Slow-Mohs en galette était utilisée sur les patients inclus de 2005 à 2015 du CHU de Bordeaux. L’âge, le sexe, la localisation tumorale, les marges initiales, les marges avant R0, la survenue d’une récidive locale, locorégionale, métastatique ou de décès, la durée de suivi ont été étudiés. L’exérèse était réalisée sous anesthésie locale. La reconstruction, par autogreffe de peau ou lambeau local, était réalisée lors un 2 e temps chirurgical. Résultats Vingt patients ont été inclus. Les marges initiales étaient de 10 mm (9 patients) et 15 mm (11 patients). L’exérèse était complète dès le premier temps chirurgical pour quinze patients (75 %). Les marges d’exérèse moyennes permettant une résection R0 était de 15,25 ± 5,7 mm (10–25). Aucun cas de récidive n’a été constaté. La durée de suivi moyenne était de 38 mois. Conclusion La technique de Slow-Mohs permet de réaliser une exérèse R0 dans 75 % des cas pour le traitement des DFSP cervico-faciaux en 1 re intention, avec des marges réduites de 12,75 ± 2,55 mm. Elle permet, au prix d’un délai d’analyse histologique supérieur, un meilleur contrôle au niveau local tout en réduisant les marges d’exérèse R0. Cette préservation des tissus mous est un atout esthétique et fonctionnel majeur dans la chirurgie cervico-faciale. Summary Objectives The main objective of this study is to determine the necessary surgical margins to obtain a complete R0 resection for head and neck dermatofibrosarcoma protuberans (DFSP) using Slow-Mohs micrographic surgery. The secondary objective is to study the recurrence rate of these tumors. Patients and methods Slow-Mohs micrographic surgery was used for patients included between 2005 and 2015 at Bordeaux universitary hospital. For each patient the age, the sex and death occurrence, the initial surgical margins, the surgical margins for complete R0 resection, the occurrence of local or general recurrence during follow-up were reported. Surgery was realized under local anesthesia. The closure of the tumor site was realized secondarily using a skin graft or local flap. Results Twenty patients were included in the study. Initial surgical margins were 10 mm (9 patients) or 15 mm (11 patients). Complete resection was obtained from the first surgery for fifteen patients (75%). The average surgical margin for a complete R0 resection was 15,25 ± 5,7 mm (10–25). None of the patients presented recurrences during the entire follow-up (38 months) Conclusion A complete R0 resection of head and neck DFSP is obtained from the first surgery in 75% of the cases, with minimum surgical margins (12,75 ± 2,55 mm) using the Slow-Mohs micrographic surgery. This allows a reduction of surgical margins and local recurrences. This technique provides a preservation of soft-tissues, which plays a key role for head and neck surgery. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFS) is a rare malignant skin tumor, characterized by local aggressiveness, significant potential for recurrence and a low risk of distant metastasis. The location under the eyes, not yet reported in the literature may involve the functional and aesthetic prognosis. We report a case. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma is a rare low-grade sarcoma characterized in more than 90% of cases by (17q22; 22q13) translocation leading to a fusion transcript COL1A1-PDGFB. Surgical therapy is the standard of care; however, tyrosine-kinase inhibitors targeting PDGFR are of interest in unresectable or rare metastatic tumors. Imatinib mesylate has been approved in this indication and research is going on with other inhibitors. [ABSTRACT FROM AUTHOR]

Summary: Dermatofibrosarcoma protuberans is a malignant and locally invasive tumor. It generally affects young adults. However, in rare but not exceptional cases, children can suffer from this disease. In the literature, there are only few studies on this pathology occurring in children. The main treatment is surgical excision with large margins. We studied in our series 15 cases of dermatofibrosarcoma in children between 1995 and 2008. The data were retrospectively collected. The aim of our study is to underline the best support for this pathology in children. The mean age at the moment of diagnosis was 13years old. None of our patients underwent surgery for dermatofibrosarcoma removal before. The diagnosis was confirmed by biopsy and immunology by FISH method. In 60% of the cases, the lesion, which is very polymorphic, was on the trunk. In three cases, a skin trauma was noted in the patient''s history. Surgical management consisted in tumor''s excision with a 3cm margin laterally associated with the removal of the first unaffected anatomical layer in depth. Reconstruction was adapted to the defect''s width, depth and topography. The mean follow-up was 7.8years. There were no relapses reported in a 3-year period at least. Complications were two scars dehiscences which needed skin graft for repair and a scarring alopecia reconstructed secondarily by a skin expansion protocol. No functional sequelae were reported in our series. As dermatofibrosarcoma in children presents the same clinical aspect and evolution as in adults, we treated it the same way. This diagnosis should not be ignored in children, and should be made early to give the patient the most effective support. [Copyright &y& Elsevier]

Abstract: Introduction. – The dermatofibrosarcoma protuberans (DFSP) is a rare but not exceptional tumour. Surgical treatment should contain a wide excision to avoid local recurrence. Materiel and method. – We report a retrospective study of 34 cases treated by the same team from 1994 to 1999. In this series, only 4 cases (12%) presented recurrences after previous treatment by the other teams. In all cases, surgical resection was performed with 3 cm lateral margin and a disease free anatomic layer removed with the tumour. Results. – The mean of follow up was 60 months. There was no recurrence case in our 34 patients during this period. We compare our results with those, from others teams described in international literature since 1951. Margins and results are similar with some teams who practice the Mohs surgery. Conclusion. – These data are in favour of a reduction of the lateral margins in classical surgical procedure of DFSP. They should be consolidated by a follow-up in longer term with a prospective study. [Copyright &y& Elsevier]

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Introduction: Dermatomyofibroma (DMF) is a rare, benign tumour that is little-known among clinicians. However, it has typical clinical, histological and immunohistochemical features that distinguish it from other fibrous tumours., Method: We report herein on the clinical, histological and immunohistochemical aspects of eight cases of DMF identified between 2008 and 2019 at the dermatopathology laboratory of Strasbourg., Results: Five men and three women of average age at diagnosis of 21 years and 9 months (range: 9 to 54 years) were included. Lesions ranged in size from 1 to 11cm. Most cases involved the upper body (6 cases), with one case on the abdomen and one on the side. The lesions presented as a solitary asymptomatic red or reddish brown nodule or plaque that gradually developed. The plaques were hard and caused functional discomfort on movement of the neck. Well-circumscribed spindle cell proliferation was noted in the reticular dermis parallel to the epidermis, without mitotic figures or cytological atypia. The subcutis was infiltrated in 5 cases. Expression of calponin was positive in all cases but one, while that of caldesmon, PS100 and desmin was negative. Expression of smooth muscle actin was positive in 2 cases, and both cases were also positive for stromylesin-3. CD34 was positive in 2 cases., Discussion: DMF is an extensive tumour capable of attaining large diameters and must be completely excised. The main differential diagnoses of DMF are dermatofibrosarcoma protuberans, dermatofibroma, fibrous hamartoma, myofibromatosis and cheloid. It can be identified based on various factors, whether clinical (young age, extensive lesion), histological (horizontal proliferation in the reticular dermis) or immunohistochemical (positive expression of calponin)., (Copyright © 2020. Published by Elsevier Masson SAS.)