scholarly journals Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years

2018 ◽  
Vol 6 (1) ◽  
pp. 35-37
Author(s):  
Uwe Wollina ◽  
Dana Langner ◽  
Jacqueline Schönlebe ◽  
Katlein França ◽  
Torello Lotti ◽  
...  

Dermatofibrosarcoma protuberans (DFSP) is rare mesenchymal neoplasia with a high risk of local recurrence but a low risk of metastatic spread. Tumor cells express CD34 and show a characteristic translocation t(17;22)(q22;q13). We analysed the documented cases at the Department of Dermatology and Allergology between 08/2001 and 08/2017. The diagnosis had been confirmed by histology and immunohistology in all cases. We identified four adults and a pediatric patient with DFSP. All patients were treated by wide surgical excision and controlled by three-dimensional histologic margin control. We observed no recurrence and no metastatic spread. We discuss prognostic factors and emerging treatments.

1994 ◽  
Vol 20 (10) ◽  
pp. 687-689 ◽  
Author(s):  
FRANCISCO JAVIER JIMENEZ ◽  
JAMES MICHAEL GRICHNIK ◽  
MARK DEWAINE BUCHANAN ◽  
ROBERT EARL CLARK

2020 ◽  
Vol 13 (1) ◽  
pp. e232614 ◽  
Author(s):  
Durga Sowgandhi Chilukuri ◽  
Prithviraj Premkumar ◽  
Balasubramanian Venkitaraman ◽  
Jagadesh Chandra Bose Soundararajan

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue malignancy which is locally aggressive, slow growing. It has a very low metastatic potential and has high risk of local recurrence. We report a 65-year-old man with recurrent DFSP of thigh with pancreatic metastasis. Apart from our patient, only four other cases of pancreatic metastasis of DFSP have been reported. Our patient had a solitary metastasis to pancreas and was treated with distal pancreaticosplenectomy. Outcome of the patient was good. We present this case report to emphasise that resection may be considered for solitary metastasis of DFSP and can be managed successfully.


1995 ◽  
Vol 31 (4) ◽  
pp. 321-326 ◽  
Author(s):  
AS Hammer ◽  
FR Weeren ◽  
SE Weisbrode ◽  
SL Padgett

The medical records of 45 dogs with histopathologically confirmed osteosarcomas arising from flat or irregular bones were reviewed. Thirty-five percent of the dogs eventually developed metastases. Telangectatic tumors and tumors arising from the rib and scapula had the highest prevalence of metastases. Survival times were short, with an overall median survival time of 120 days. Anatomic site, body weight, and completeness of surgical excision were found to be prognostic factors. Dogs with mandibular osteosarcomas in this study had shorter survival times than those times recently reported. 21 Local recurrence was the most common cause of death or euthanasia in this population of dogs.


2017 ◽  
Author(s):  
Benjamin Deschner ◽  
Jeffrey Wayne

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant cutaneous tumor with an annual incidence of 4.2 per million. The tumor typically grows slowly and has a proclivity toward local invasiveness and away from distant metastasis. The mainstay of treatment is complete surgical resection, which is curative in almost all cases. The tumor’s characteristics make this difficult, however, and reexcision to negative margins can be challenging. DFSP can also present with or develop fibrosarcomatous change (DFSP-FS), a more aggressive variant. Thus, these tumors require thoughtful management. In this review, we discuss the biologic background of DFSP, describe the tumor’s behavior and presentation, and discuss current concepts in management.   This review contains 5 figures, 4 tables and 28 references Key words: dermatofibrosarcoma protuberans, fibrosarcomatous change, imatinib, local recurrence, micrographic surgery, nonmelanoma, radiation, sarcoma, skin cancer, surgical excision


2017 ◽  
Author(s):  
Benjamin Deschner ◽  
Jeffrey Wayne

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant cutaneous tumor with an annual incidence of 4.2 per million. The tumor typically grows slowly and has a proclivity toward local invasiveness and away from distant metastasis. The mainstay of treatment is complete surgical resection, which is curative in almost all cases. The tumor’s characteristics make this difficult, however, and reexcision to negative margins can be challenging. DFSP can also present with or develop fibrosarcomatous change (DFSP-FS), a more aggressive variant. Thus, these tumors require thoughtful management. In this review, we discuss the biologic background of DFSP, describe the tumor’s behavior and presentation, and discuss current concepts in management.   This review contains 5 figures, 4 tables and 28 references Key words: dermatofibrosarcoma protuberans, fibrosarcomatous change, imatinib, local recurrence, micrographic surgery, nonmelanoma, radiation, sarcoma, skin cancer, surgical excision


Sarcoma ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-10 ◽  
Author(s):  
J. Haniball ◽  
V. P. Sumathi ◽  
L.-G. Kindblom ◽  
A. Abudu ◽  
S. R. Carter ◽  
...  

Background. This study aimed to investigate prognostic factors for patients with myxoid/round-cell liposarcoma (MRCLS), in particular the significance of the round cell component, and to identify metastatic patterns as well as possibly suggest a suitable strategy for followup.Methods. Clinical, morphologic, and follow-up data from 160 patients with MRCLS was reviewed and statistically analysed.Results. Of 130 tumours with the round cell component evaluated, 61 had no round cell component, 27 had <5% round cell component, and 42 had >5%. All patients underwent surgical excision, 15 requiring amputation. 107 patients received adjuvant radiotherapy. Local recurrence occurred in 19 patients (12%), predominantly in patients with marginal or intralesional margins and a round cell component. Overall disease specific survival was 75% at 5 years and 56% at 10 years and was related to the proportion of round cell component. Of 52 patients who developed metastases, 38 (73%) had purely extrapulmonary metastases. We could not identify any factors predicting the site of metastases developing.Conclusions. The occurrence of any round cell component is the most important adverse prognostic factor for patients with MRCLS; patients with >5% round cell component are at higher risk of local recurrence, metastasis and tumour-related death and should be considered for adjuvant radiotherapy and possibly chemotherapy. The best method of monitoring extrapulmonary metastases remains to be established.


2018 ◽  
Vol 24 ◽  
pp. 249
Author(s):  
David Broome ◽  
Gauri Bhuchar ◽  
Ehsan Fayazzadeh ◽  
James Bena ◽  
Christian Nasr

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