PMID- 36377270
OWN - NLM
STAT- MEDLINE
DCOM- 20221121
LR  - 20221121
IS  - 1610-0387 (Electronic)
IS  - 1610-0379 (Linking)
VI  - 20
IP  - 11
DP  - 2022 Nov
TI  - Dermatofibrosarcoma protuberans of the face: A clinicopathologic and molecular 
      study of 34 cases.
PG  - 1463-1473
LID - 10.1111/ddg.14882 [doi]
AB  - BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is one of the most common 
      dermal sarcomas, but facial DFSP is rare. PATIENTS AND METHODS: The 
      clinicopathological characteristics of 34 facial DFSPs were reviewed. Additional 
      immunostaining (CD34) and PDGFB/COL1A1-PDGFB fluorescence in situ hybridization 
      (FISH) detection were performed. RESULTS: Patients were aged from 24 to 64 years 
      (mean 42.9 years), with a male-to-female ratio of 4.7 : 1. Morphologically, 
      classic DFSP (25/34, 73.5 %), pigmented DFSP (2/34, 5.9 %), DFSP with myoid 
      differentiation (1/34, 2.9 %) and fibrosarcomatous DFSP (FS-DFSP) (6/34, 17.6 %) 
      were found. Moreover, myxoid degeneration was observed in three FS-DFSP cases 
      (3/6, 50.0 %). All 29 cases that underwent CD34 immunohistochemistry exhibited 
      positive staining (100 %). Genetically, PDGFB rearrangement/COL1A1-PDGFB fusion 
      was detected in 94.1 % (16/17) of patients. Regarding prognosis, the recurrence 
      (83.3 % vs. 59.1 %) and metastasis (33.3 % vs. 0 %) rates were higher in FS-DFSPs 
      than that in ordinary DFSPs. All available surgical margins were positive before 
      DFSPs recurrence. All patients with negative excision or re-excision margins were 
      alive without evidence of disease (mean, 81.8 months; median, 81 months). 
      CONCLUSIONS: Facial DFSP occurs predominantly in males, while FS-DFSPs are more 
      likely to exhibit myxoid degeneration and a worse prognosis. Notably, negative 
      surgical margin status determined a satisfactory prognosis.
CI  - (c) 2022 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & 
      Sons Ltd.
FAU - Han, Qianqian
AU  - Han Q
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, 
      Sichuan, China.
FAU - Chen, Min
AU  - Chen M
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, 
      Sichuan, China.
FAU - Yang, Jieliang
AU  - Yang J
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, 
      Sichuan, China.
FAU - Du, Tianhai
AU  - Du T
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, 
      Sichuan, China.
FAU - Peng, Heng
AU  - Peng H
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, 
      Sichuan, China.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20221114
PL  - Germany
TA  - J Dtsch Dermatol Ges
JT  - Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German 
      Society of Dermatology : JDDG
JID - 101164708
RN  - 0 (Antigens, CD34)
RN  - 0 (Proto-Oncogene Proteins c-sis)
SB  - IM
MH  - Female
MH  - Humans
MH  - Male
MH  - Antigens, CD34
MH  - *Dermatofibrosarcoma/diagnosis/genetics/surgery
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Proto-Oncogene Proteins c-sis/genetics
MH  - *Skin Neoplasms/genetics/surgery/diagnosis
MH  - Adult
MH  - Middle Aged
EDAT- 2022/11/16 06:00
MHDA- 2022/11/19 06:00
CRDT- 2022/11/15 02:13
PHST- 2022/04/12 00:00 [received]
PHST- 2022/07/25 00:00 [accepted]
PHST- 2022/11/16 06:00 [pubmed]
PHST- 2022/11/19 06:00 [medline]
PHST- 2022/11/15 02:13 [entrez]
AID - 10.1111/ddg.14882 [doi]
PST - ppublish
SO  - J Dtsch Dermatol Ges. 2022 Nov;20(11):1463-1473. doi: 10.1111/ddg.14882. Epub 
      2022 Nov 14.