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Case Reports 2018 Medical Case Reports
ISSN: 2471-8041 Page 54
May 28-29, 2018
London, UK
8th Edition of International Conference on
Clinical and Medical Case Reports
Introduction: Darier and Ferrand dermatofibrosarcoma is a rare
soft tissue sarcoma characterized by a slow evolution with a major
risk of recurrence in case of non-cancerous resection. Although
large surgical excision remains the standard treatment, imatinib
has a place in locally advanced and metastatic forms The objective
of this study is to determine the epidemiological, diagnostic,
therapeutic and evolutionary characteristics collected from the
clinical records of patients for dermatofibrosarcoma of Darier and
Ferrand.
Materials and methods: This is a retrospective study conducted
fromJanuary 2013 to December 2016 in the department of medical
oncology at CHU Hassan II in Fez, including patients with Darier
and Ferrand dermatofibrosarcoma locally advanced or metastatic.
Results: 7 cases were reported, 3 women and 4 men, the mean
age was 46.2 years (range: 33-65). The tumor was located in the
scalp in 3 cases, 1 case in the peri-umbilical, 1 case at the level
of the chest wall and 1 case at the level of the scapular region.
In all cases, there was histological evidence of the diagnosis of
dermatofibrosarcoma. Immunohistochemical examination with
CD34 antibody was performed in all cases showing positivity with
cytoplasmic and membrane CD34 labeling. The tumor stage was
locally advanced in 5 cases and metastatic at the pulmonary level
in 2 cases. Five patients had local recurrence in the initial site of
resection. Imatinib was administered at a dose of 400mgx2 / day in
all patients. The outcome was marked by stability in 4 patients and
a partial clinical response in 2 patients after an average duration of
11 months, while 1 patient was lost sight of. Regarding tolerance
to imatinib, 3 patients presented with a hand-grade I syndrome,
2 patients with thrombocytopenia and 1 patient with severe
neutropenia, which required stopping treatment temporarily with
reintroduction after resolution of neutropenia.
Conclusion: The dermatofibrosarcoma of Darier and Ferrand is a
rare cutaneous tumor with slow local evolution taking place over
several years. It is distinguished by its diagnostic difficulty, its
tendency to recurrence and the rarity of its metastases which are
essentially pulmonary. Imatinib is an effective therapeutic option
with a good safety profile in the treatment of locally advanced or
metastatic dermatofibrosarcoma.
Biography
Nisrine Acharfi is currently affiliated to the department of Medical Oncology at
the Hassan II Hospital University in Fez, Morocco.
Dermatofibrosarcoma of Darier and Ferrand locally advanced
and metastatic: About 7 cases.
N. Acharfi1, R.Boujarnija2, M .Benhami3,L.Amaadour 4, K. Oualla5 , Z. Benbrahim6,
FZ Elmrabet7 , S. Arifi, N. Mellas8
1Hassan II Hospital University, Morocco
N. Acharfi et al., Med Case Rep. 2018, Volume 4
DOI:10.21767/2471-8041-C1-003