Actins ; metabolism ; Dermatofibrosarcoma ; classification ; pathology ; Desmin ; metabolism ; Head and Neck Neoplasms ; metabolism ; pathology ; Histiocytoma, Benign Fibrous ; classification ; metabolism ; pathology ; Histiocytoma, Malignant Fibrous ; classification ; metabolism ; pathology ; Humans ; Oncogene Proteins, Fusion ; metabolism ; Skin Neoplasms ; metabolism ; pathology ; Vimentin ; metabolism ; Xanthomatosis ; pathology

Actins ; metabolism ; Adult ; Desmin ; metabolism ; Diagnosis, Differential ; Histiocytoma, Malignant Fibrous ; pathology ; Humans ; Immunohistochemistry ; Leiomyoma ; pathology ; Leiomyosarcoma ; metabolism ; pathology ; surgery ; Male ; Mediastinal Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Aged ; Female ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Spleen ; chemistry ; pathology ; surgery ; Splenectomy ; Splenic Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Carcinosarcoma ; metabolism ; pathology ; surgery ; Esophageal Neoplasms ; metabolism ; pathology ; surgery ; Esophagectomy ; methods ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; surgery ; Humans ; Keratins ; metabolism ; Male ; Middle Aged ; Mucin-1 ; metabolism ; Vimentin ; metabolism

Abdominal Neoplasms ; metabolism ; pathology ; Adolescent ; Diagnosis, Differential ; Fibroblasts ; metabolism ; pathology ; Groin ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Humans ; Keratins ; metabolism ; Lymph Nodes ; metabolism ; pathology ; Lymphoma ; metabolism ; pathology ; Male ; Melanoma ; metabolism ; pathology ; Vimentin ; metabolism

Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Breast Neoplasms ; metabolism ; pathology ; surgery ; Carcinoma, Lobular ; metabolism ; pathology ; surgery ; Female ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Middle Aged ; Receptors, Progesterone ; metabolism ; Retroperitoneal Neoplasms ; metabolism ; pathology ; surgery

OBJECTIVETo study the clinicopathologic features, immunophenotype and differential diagnosis of angiomatoid fibrous histiocytoma (AFH).

METHODSThe clinicopathologic features of 5 cases of AFH were analyzed. Immunohistochemical study was carried out and the literature was reviewed.

RESULTSThere were a total of 3 males and 2 females. The average age of patients was 21.4 years old. The average duration of symptoms was 13 months. The patients primarily presented with a slowly enlarging painless deep dermal or subcutaneous mass. The mass was located in the head and neck region in 3 cases, elbow in 1 case and foot in 1 case. The patients underwent complete resection of the tumor, with no adjuvant chemotherapy and/or radiotherapy given. During a period of follow up for 10 to 29 months, all of them had no recurrence or distant metastasis. Gross examination showed that the tumor was well-circumscribed and had a grey-colored cut surface, with focal hemorrhagic cystic changes. The average tumor dimension was 1.9 cm. Histologically, the tumor was composed of histiocytoid or spindly cells arranged in nodular pattern. Fibrillary neuropil-type intercellular material was identified in all cases and a fibrous pseudocapsule surrounded by lymphocytes and plasma cells was demonstrated in 3 cases. Immunohistochemical study showed that all of them were positive for vimentin and negative for S-100 protein, pan-cytokeratin, CD34 and CD31. Three of the cases expressed desmin and CD68. Two cases were epithelial membrane antigen and CD99-positive.

CONCLUSIONSAFH is a rare tumor of intermediate malignant potential. Definitive diagnosis requires thorough histologic examination and clinical correlation. Immunohistochemistry is also helpful for diagnosis and differential diagnosis. Wide local excision with post-operative follow up is the main modality of treatment.

Adolescent ; Adult ; Aneurysm ; metabolism ; pathology ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Chemotherapy, Adjuvant ; Child ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; surgery ; Histiocytoma, Malignant Fibrous ; pathology ; Humans ; Male ; Radiotherapy, Adjuvant ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Young Adult

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

Adult ; Diagnosis, Differential ; Histiocytoma, Malignant Fibrous ; pathology ; Humans ; Laryngeal Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Laryngectomy ; Leiomyosarcoma ; pathology ; Male ; Rhabdomyosarcoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Tomography, X-Ray Computed ; Vimentin ; metabolism

BACKGROUND: We investigated the expression of matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) in malignant fibrous histiocytoma (MFH), and determined whether these could be useful as prognostic factors. METHODS: Among patients treated from 1993 to 2007, 30 cases of MFH were evaluated. Immunohistochemical staining was performed for MMP-2, MMP-9, TIMP-1, and TIMP-2 using paraffin wax-embedded blocks of MFH tissues. Reverse transcriptase polymerase chain reaction (RT-PCR) and Western blot and zymography were performed using fresh tissues obtained from 17 of the 30 cases. The levels of MMP and TIMP expression were compared between the MFH and normal control groups, and between non-metastatic and metastatic MFH groups. RESULTS: Expression levels of MMP-2, MMP-9, TIMP-1, and TIMP-2 were higher in the MFH group than the control group by RT-PCR, Western blotting, and zymography. Immunohistochemical staining revealed that MMP-2 and MMP-9 protein expression was higher in the metastatic than in the non-metastatic group. The expression levels of MMP-2 and TIMP-1 were significantly higher in the metastatic than in the non-metastatic group (p < 0.05) by RT-PCR. By Western blot analysis, the expression levels of MMP-2, TIMP-1, and TIMP-2 were higher in the metastatic group (p < 0.05), but MMP-9 showed only a slight increase in the metastatic group compared with the non-metastatic group (p > 0.05). Finally, gelatin zymography analysis showed that the expression levels of the pro- and active forms of MMP-2 were significantly higher in the metastatic group (p < 0.05), but the expression of the pro- and active forms of MMP-9 showed a slight decrease in the metastatic group (p > 0.05). CONCLUSIONS: These results suggest that MMP-2, MMP-9, TIMP-1, and TIMP-2 may have important roles in the development and progression of MFH, and that the degree of expression of these metalloproteinases and their inhibitors, especially MMP-2, could be useful as prognostic factors related to metastasis in MFH.
Adult ; Aged ; Aged, 80 and over ; Female ; Histiocytoma, Malignant Fibrous/*metabolism ; Humans ; Immunohistochemistry ; Male ; Matrix Metalloproteinase 2/*biosynthesis ; Matrix Metalloproteinase 9/*biosynthesis ; Middle Aged ; Neoplasm Metastasis ; Prognosis ; Tissue Inhibitor of Metalloproteinase-1/*biosynthesis ; Tissue Inhibitor of Metalloproteinase-2/*biosynthesis