Dermatofibrosarcoma protuberans, fibrosarcomatous variant: A rare tumor in children

2020 ◽  
Author(s):  
Matthieu Chicaud ◽  
Annonciade Frassati‐Biaggi ◽  
Sophie Kaltenbach ◽  
Marie Karanian ◽  
Daniel Orbach ◽  
...  
Keyword(s):  
Dermatofibrosarcoma Protuberans ◽  
Rare Tumor ◽  
Fibrosarcomatous Variant

scholarly journals Dermatofibrosarcoma protuberans at Niamey National Hospital: About 12 cases

2021 ◽  
Vol 1 (2) ◽  
pp. 49-51
Author(s):  
A Soumaila ◽  
AI Dourahamane ◽  
I Dillé ◽  
OS Galadima ◽  
HM Zaki ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Abdominal Wall ◽  
Recurrence Rate ◽  
Dermatofibrosarcoma Protuberans ◽  
Wide Excision ◽  
Recidivism Rate ◽  
Rare Tumor ◽  
National Hospital ◽  
Histological Evidence

AIM: To report the clinical and therapeutic aspects of dermatofibrosarcoma of Darier and Ferrand (DFS) to the National Hospital of Niamey. MATERIALS AND METHODS: These are 12 patients collected prospectively over three years. RESULTS: It concerns seven men and five women in whom the diagnosis of DFS was made on the basis of histological evidence. The average age was 46.5 years. The tumor was the majority on the trunk (abdominal wall and back) in seven patients or 58.33%. The extension assessment was negative in all patients and all benefited from surgical treatment. This treatment consisted of a wide excision with lateral and deep margins between three and five cm. The recidivism rate is 25% within 17 months. CONCLUSION: DFS is a rare tumor in Niamey. The trunk is its main location. Surgery is the best treatment. And the recurrence rate is still high.

Aspiration Cytology of Fibrosarcomatous Variant of Dermatofibrosarcoma Protuberans with Osteoclastlike Giant Cells in the Chest Wall

2005 ◽  
Vol 49 (6) ◽  
pp. 644-649 ◽  
Author(s):  
Lucia Kim ◽  
In Suh Park ◽  
Jee Young Han ◽  
Joon Mee Kim ◽  
Young Chae Chu
Keyword(s):  
Chest Wall ◽  
Dermatofibrosarcoma Protuberans ◽  
Giant Cells ◽  
Aspiration Cytology ◽  
Fibrosarcomatous Variant

Fibrosarcomatous variant of dermatofibrosarcoma protuberans with pancreatic metastasis

2013 ◽  
Vol 53 (2) ◽  
pp. e140-e142 ◽  
Author(s):  
Hiroshi Murata ◽  
Atsuko Ohashi ◽  
Atsuko Ashida ◽  
Hisashi Uhara ◽  
Ryuhei Okuyama ◽  
...  
Keyword(s):  
Dermatofibrosarcoma Protuberans ◽  
Pancreatic Metastasis ◽  
Fibrosarcomatous Variant

scholarly journals Dermatofibrosarcoma of the scalp

2019 ◽  
Vol 10 ◽  
pp. 221 ◽  
Author(s):  
Marcos Sangrador ◽  
Jimena González Olvera ◽  
Valeria Mendoza Ortiz
Keyword(s):  
Local Recurrence ◽  
Surgical Management ◽  
Dermatofibrosarcoma Protuberans ◽  
Gross Total Resection ◽  
High Grade ◽  
Rare Tumor ◽  
Parietal Region ◽  
Total Resection ◽  
History Of ◽  
The Right

A 25-year-old woman presented with a 4 months history of progressive pain and tumefaction in the right parietal region. Deformity of the scalp was evident, and a biopsy was taken, reporting a high- grade dermatofibrosarcoma. She underwent surgical management, achieving a gross total resection. Dermatofibrosarcoma protuberans is a rare tumor arising from the dermis. It tends to have an indolent course and local recurrence after excision.

scholarly journals Advanced Dermatofibrosarcoma Protuberans Treatment With Imatinib: Experience From a Dedicated Sarcoma Medical Oncology Clinic in India

2018 ◽  
pp. 1-7
Author(s):  
Sameer Rastogi ◽  
Ekta Dhamija ◽  
Adarsh Barwad ◽  
Aditi Aggarwal ◽  
Atul Sharma ◽  
...  
Keyword(s):  
Medical Oncology ◽  
Tertiary Care ◽  
Progression Free Survival ◽  
Dermatofibrosarcoma Protuberans ◽  
Molecular Testing ◽  
Free Survival ◽  
Median Progression Free Survival ◽  
Uncommon Disease ◽  
Tertiary Care Centers ◽  
Fibrosarcomatous Variant

Purpose Advanced dermatofibrosarcoma protuberans (DFSP) is an exceptionally uncommon disease with scarce literature, especially from developing countries. Molecular testing is unfortunately not available in India, and expert diagnosis by a sarcoma pathologist is available only in tertiary care centers. Materials and Methods We retrospectively analyzed consecutive patients with inoperable DFSP (on the basis of expert histopathology only) who presented to our sarcoma medical oncology clinic from January 2016 to July 2017. Results There were a total of seven patients, with median age of 35 years, predominantly males (85.7%). Fibrosarcomatous variant and metastatic disease were present in six (85.7%) patients. Partial response rates were 71.4%, and overall disease control was 85.7%. Median progression-free survival was 14 months. Conclusion DFSP diagnosis on the basis of expert histopathology in the absence of translocation can help out in targeted therapy–based treatment until translocation testing becomes available. The fibrosarcomatous variant has poor outcome, and further research is needed to help this group of patients.

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