[Fibrosarcomatous dermatofibrosarcoma protuberans: a clinicopathological analysis of 12 cases].
OBJECTIVE
To investigate the clinical pathological features of fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP).


METHODS
The clinical history, histopathological features and immunohistochemical characteristics were analyzed in twelve cases of FS-DFSP from January 1997 to February 2011, and related literature were reviewed.


RESULTS
Age of the patients (2 females, 10 males) at diagnosis ranged from 41 to 70 years (mean 53 years). Among the 12 cases of FS-DFSP, 9 cases aroused in recurrent ordinary DFSP. Histologically, FS areas in FS-DFSP were characterized by a fascicular and highly cellular histology, frequently showing a characteristic herringbone pattern. FS-DFSP showed diminishment of CD34 staining in FS areas. The labeling index of Ki-67 was much higher in the FS areas (10%-40%) than that in the conventional DFSP areas (2%-5%). All the patients were treated by operation with local excision or wide excision. Postoperative radiotherapy and chemotherapy was administered in two cases respectively. Follow-up information in 9 of 12 patients (9 to 86 months) revealed local recurrence in 6 patients. Distant metastases were seen in two patients. One patient was died in the follow up period.


CONCLUSIONS
FS-DFSP is a rare and unique subtype of DFSP and is associated with significant elevated risk of both local and distance metastasis, usually followed by poor outcome. Compared to ordinary DFSP as a borderline neoplasm, FS-DFSP should be considered as a malignant tumor.
Department of Pathology, Jinling Hospital, Nanjing 210002, China.
Jin
J
Zhang
Department of Pathology, Jinling Hospital, Nanjing 210002, China.
Nan
N
Wu
Department of Pathology, Jinling Hospital, Nanjing 210002, China.
Chun
C
Xia
Department of Pathology, Jinling Hospital, Nanjing 210002, China.
Xue
X
Wei
Department of Pathology, Jinling Hospital, Nanjing 210002, China.
Qun-li
QL
Shi
Department of Pathology, Jinling Hospital, Nanjing 210002, China.
Xiao-jun
XJ
Zhou
Department of Pathology, Jinling Hospital, Nanjing 210002, China. E-mail:maggie-maj@163.com.
Jie
J
Ma
D000328:Adult; D000368:Aged; D018952:Antigens, CD34 / Q000378:metabolism; D017024:Chemotherapy, Adjuvant; D018223:Dermatofibrosarcoma / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D005350:Fibroma / Q000473:pathology; D005354:Fibrosarcoma / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D005500:Follow-Up Studies; D018219:Histiocytoma, Benign Fibrous / Q000378:metabolism / Q000473:pathology; D051677:Histiocytoma, Malignant Fibrous / Q000473:pathology; D006801:Humans; D019394:Ki-67 Antigen / Q000378:metabolism; D008175:Lung Neoplasms / Q000556:secondary; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy
D018952:Antigens, CD34; D019394:Ki-67 Antigen