OBJECTIVETo investigate the clinical pathological features of fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP).

METHODSThe 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.

RESULTSAge 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.

CONCLUSIONSFS-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.

Adult ; Aged ; Antigens, CD34 ; metabolism ; Chemotherapy, Adjuvant ; Dermatofibrosarcoma ; metabolism ; pathology ; therapy ; Diagnosis, Differential ; Female ; Fibroma ; pathology ; Fibrosarcoma ; metabolism ; pathology ; therapy ; Follow-Up Studies ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; Histiocytoma, Malignant Fibrous ; pathology ; Humans ; Ki-67 Antigen ; metabolism ; Lung Neoplasms ; secondary ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Radiotherapy, Adjuvant ; Retrospective Studies ; Skin Neoplasms ; metabolism ; pathology ; therapy

Angiofibroma ; metabolism ; pathology ; Biomarkers, Tumor ; metabolism ; Diagnosis, Differential ; Epithelioid Cells ; Extremities ; Fibroma ; metabolism ; pathology ; Fingers ; Hemangioblastoma ; metabolism ; pathology ; Hemangioendothelioma ; metabolism ; pathology ; Humans ; Rhabdomyosarcoma ; metabolism ; pathology ; Sclerosis ; Soft Tissue Neoplasms ; classification ; metabolism ; pathology

Adult ; Antigens, CD ; metabolism ; Antigens, CD34 ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Dermatofibrosarcoma ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Fibroma ; metabolism ; pathology ; Humans ; Lipoma ; pathology ; Neurofibroma ; metabolism ; pathology ; Receptors, Cell Surface ; metabolism ; Skin Neoplasms ; metabolism ; pathology ; surgery

Actins ; immunology ; metabolism ; Antibodies, Monoclonal ; metabolism ; Child ; Diagnosis, Differential ; Fibroma ; metabolism ; pathology ; surgery ; Follow-Up Studies ; Gastrointestinal Neoplasms ; metabolism ; pathology ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Humans ; Leiomyoma ; metabolism ; pathology ; Male ; Pyloric Antrum ; pathology ; Stomach Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Actins ; metabolism ; Adult ; Breast Diseases ; metabolism ; pathology ; surgery ; Breast Neoplasms ; pathology ; Calcium-Binding Proteins ; metabolism ; Carcinoma ; pathology ; Diagnosis, Differential ; Fasciitis ; metabolism ; pathology ; surgery ; Female ; Fibroma ; pathology ; Fibrosarcoma ; pathology ; Follow-Up Studies ; Humans ; Microfilament Proteins ; metabolism ; Vimentin ; metabolism ; Young Adult

Calcium-Binding Proteins ; metabolism ; Diagnosis, Differential ; Female ; Fibroma ; metabolism ; pathology ; surgery ; Humans ; Inclusion Bodies ; pathology ; Infant ; Microfilament Proteins ; metabolism ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Soft Tissue Neoplasms ; pathology ; Tendons ; pathology ; Toes ; Vimentin ; metabolism

Actins ; metabolism ; Adult ; Diagnosis, Differential ; Female ; Fibroma ; pathology ; Fibrosarcoma ; pathology ; Follow-Up Studies ; Humans ; Hysterectomy ; Inflammation ; Keratins ; metabolism ; Leiomyoma ; pathology ; Neoplasm Recurrence, Local ; Neoplasms, Muscle Tissue ; drug therapy ; metabolism ; pathology ; surgery ; Receptor Protein-Tyrosine Kinases ; metabolism ; Uterine Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Young Adult

Adult ; Diagnosis, Differential ; Female ; Fibroma ; metabolism ; pathology ; Fingers ; Giant Cell Tumors ; metabolism ; pathology ; Humans ; Mucin-1 ; metabolism ; Nerve Sheath Neoplasms ; metabolism ; pathology ; surgery ; Neurilemmoma ; metabolism ; pathology ; Sclerosis ; metabolism ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Tendons ; Young Adult

Diagnosis, Differential ; Fetus ; Fibroma ; metabolism ; pathology ; Fibrosarcoma ; congenital ; genetics ; metabolism ; secondary ; Hemangiopericytoma ; metabolism ; pathology ; Humans ; Kidney Neoplasms ; pathology ; secondary ; Liver Neoplasms ; pathology ; secondary ; Lung Neoplasms ; pathology ; secondary ; Male ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Rhabdomyosarcoma, Embryonal ; metabolism ; pathology ; Soft Tissue Neoplasms ; congenital ; genetics ; metabolism ; pathology ; Vimentin ; metabolism

Actins ; metabolism ; Aged ; Breast Neoplasms ; metabolism ; pathology ; surgery ; Carcinoma ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Fasciitis ; metabolism ; pathology ; Female ; Fibroma ; metabolism ; pathology ; surgery ; Humans ; Keratin-5 ; metabolism ; Mastectomy, Modified Radical ; Neoplasms, Muscle Tissue ; metabolism ; pathology