Giant cell fibroblastoma associated with dermatofibrosarcoma protuberans: a case report. 1998

T Maeda, and T Hirose, and K Furuya, and K Shirakawa, and K Kobayashi
Department of Pathology, Ehime Prefectural Central Hospital, Matsuyama, Japan.

A case of congenital giant cell fibroblastoma (GCF) associated with dermatofibrosarcoma protuberans (DFSP) in a 1.5-year-old girl is reported. The GCF component was characterized by a mixed proliferation of fibroblastic cells and multinucleated giant cells and sinusoid-like spaces. The DFSP component, which was spatially distinct from the GCF region, was composed of short spindle-shaped cells arranged in a storiform pattern. Mitotic figures were frequently identified in the DFSP area but not in the GCF region. Immunohistochemically, tumor cells in both components were positive for CD34. The Ki-67 labeling index of the DFSP area (4.9%) was markedly higher than that of the GCF (0.5%). Ultrastructurally, spindle-shaped tumor cells in both components, as well as giant cells, were fibroblastic cells that frequently exhibited multilobulated nuclei. This case illustrates the close histogenetic relationship between GCF and DFSP, although the degree of proliferative activity is much higher in the DFSP area.

UI MeSH Term Description Entries
D007150 Immunohistochemistry Histochemical localization of immunoreactive substances using labeled antibodies as reagents. Immunocytochemistry,Immunogold Techniques,Immunogold-Silver Techniques,Immunohistocytochemistry,Immunolabeling Techniques,Immunogold Technics,Immunogold-Silver Technics,Immunolabeling Technics,Immunogold Silver Technics,Immunogold Silver Techniques,Immunogold Technic,Immunogold Technique,Immunogold-Silver Technic,Immunogold-Silver Technique,Immunolabeling Technic,Immunolabeling Technique,Technic, Immunogold,Technic, Immunogold-Silver,Technic, Immunolabeling,Technics, Immunogold,Technics, Immunogold-Silver,Technics, Immunolabeling,Technique, Immunogold,Technique, Immunogold-Silver,Technique, Immunolabeling,Techniques, Immunogold,Techniques, Immunogold-Silver,Techniques, Immunolabeling
D007223 Infant A child between 1 and 23 months of age. Infants
D008854 Microscopy, Electron Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen. Electron Microscopy
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D016609 Neoplasms, Second Primary Abnormal growths of tissue that follow a previous neoplasm but are not metastases of the latter. The second neoplasm may have the same or different histological type and can occur in the same or different organs as the previous neoplasm but in all cases arises from an independent oncogenic event. The development of the second neoplasm may or may not be related to the treatment for the previous neoplasm since genetic risk or predisposing factors may actually be the cause. Neoplasms, Metachronous,Neoplasms, Metachronous Second Primary,Neoplasms, Therapy-Related,Neoplasms, Treatment-Related,Second Malignancy,Second Neoplasm,Second Primary Neoplasms,Therapy-Associated Neoplasms,Therapy-Related Cancer,Treatment-Associated Neoplasms,Treatment-Related Cancer,Cancer, Second Primary,Metachronous Neoplasms,Metachronous Second Primary Neoplasms,Neoplasms, Therapy-Associated,Neoplasms, Treatment-Associated,Second Cancer,Second Primary Neoplasms, Metachronous,Therapy-Associated Cancer,Therapy-Related Neoplasms,Treatment-Associated Cancer,Treatment-Related Neoplasms,Cancer, Second,Cancer, Therapy-Associated,Cancer, Therapy-Related,Cancer, Treatment-Associated,Cancer, Treatment-Related,Cancers, Second,Cancers, Second Primary,Cancers, Therapy-Associated,Cancers, Therapy-Related,Cancers, Treatment-Associated,Cancers, Treatment-Related,Malignancies, Second,Malignancy, Second,Metachronous Neoplasm,Neoplasm, Metachronous,Neoplasm, Second,Neoplasm, Second Primary,Neoplasm, Therapy-Associated,Neoplasm, Therapy-Related,Neoplasm, Treatment-Associated,Neoplasm, Treatment-Related,Neoplasms, Second,Neoplasms, Therapy Associated,Neoplasms, Therapy Related,Neoplasms, Treatment Associated,Neoplasms, Treatment Related,Second Cancers,Second Malignancies,Second Neoplasms,Second Primary Cancer,Second Primary Cancers,Second Primary Neoplasm,Therapy Associated Cancer,Therapy Associated Neoplasms,Therapy Related Cancer,Therapy Related Neoplasms,Therapy-Associated Cancers,Therapy-Associated Neoplasm,Therapy-Related Cancers,Therapy-Related Neoplasm,Treatment Associated Cancer,Treatment Associated Neoplasms,Treatment Related Cancer,Treatment Related Neoplasms,Treatment-Associated Cancers,Treatment-Associated Neoplasm,Treatment-Related Cancers,Treatment-Related Neoplasm
D018223 Dermatofibrosarcoma A sarcoma of the deep layers of the skin. The tumors are locally aggressive tends to recur but rarely metastatic. It can be classified into variants depending on the cell type tumors are derived from or by its characteristics: Pigmented variant from MELANIN-containing DERMAL DENDRITIC CELLS; Myxoid variant, myxoid STROMAL CELLS; Giant cell variant characterized by GIANT CELLS in the tumors; and Fibrosarcomatous variant chracterized by tumor areas histologically indistinguishable from FIBROSARCOMA. Bednar Tumor,Bednar's Tumor,DFSP, Fibrosarcomatous,DFSP, Juvenile,DFSP, Myxoid,DFSP, Pigmented,Darier-Ferrand Tumor,Darier-Hoffmann Tumor,Dermatofibrosarcoma Protuberan,Dermatofibrosarcoma Protuberans, Familial,Dermatofibrosarcoma Protuberans, Fibrosarcomatous,Dermatofibrosarcoma Protuberans, Giant Cell,Dermatofibrosarcoma Protuberans, Myxoid,Dermatofibrosarcoma Protuberans, Pigmented,FS-DFSP,Familial Dermatofibrosarcoma Protuberans,Fibrosarcomatous Dermatofibrosarcoma Protuberans,Giant Cell Fibroblastoma,Giant Dermatofibrosarcoma Protuberans,Metastatic Dermatofibrosarcoma Protuberans,Bednars Tumor,Cell Fibroblastoma, Giant,Cell Fibroblastomas, Giant,DFSPs, Fibrosarcomatous,DFSPs, Juvenile,DFSPs, Myxoid,DFSPs, Pigmented,Darier Ferrand Tumor,Darier Hoffmann Tumor,Dermatofibrosarcoma Protuberan, Familial,Dermatofibrosarcoma Protuberan, Fibrosarcomatous,Dermatofibrosarcoma Protuberan, Giant,Dermatofibrosarcoma Protuberan, Metastatic,Dermatofibrosarcoma Protuberan, Myxoid,Dermatofibrosarcoma Protuberan, Pigmented,Dermatofibrosarcoma Protuberans,Dermatofibrosarcoma Protuberans, Giant,Dermatofibrosarcoma Protuberans, Metastatic,Dermatofibrosarcomas,Familial Dermatofibrosarcoma Protuberan,Fibroblastoma, Giant Cell,Fibroblastomas, Giant Cell,Fibrosarcomatous DFSP,Fibrosarcomatous DFSPs,Fibrosarcomatous Dermatofibrosarcoma Protuberan,Giant Cell Fibroblastomas,Giant Dermatofibrosarcoma Protuberan,Juvenile DFSP,Juvenile DFSPs,Metastatic Dermatofibrosarcoma Protuberan,Myxoid DFSP,Myxoid DFSPs,Myxoid Dermatofibrosarcoma Protuberan,Myxoid Dermatofibrosarcoma Protuberans,Pigmented DFSP,Pigmented DFSPs,Pigmented Dermatofibrosarcoma Protuberan,Pigmented Dermatofibrosarcoma Protuberans,Protuberan, Dermatofibrosarcoma,Protuberan, Familial Dermatofibrosarcoma,Protuberan, Fibrosarcomatous Dermatofibrosarcoma,Protuberan, Giant Dermatofibrosarcoma,Protuberan, Metastatic Dermatofibrosarcoma,Protuberan, Myxoid Dermatofibrosarcoma,Protuberan, Pigmented Dermatofibrosarcoma,Protuberans, Dermatofibrosarcoma,Protuberans, Familial Dermatofibrosarcoma,Protuberans, Fibrosarcomatous Dermatofibrosarcoma,Protuberans, Giant Dermatofibrosarcoma,Protuberans, Metastatic Dermatofibrosarcoma,Protuberans, Myxoid Dermatofibrosarcoma,Protuberans, Pigmented Dermatofibrosarcoma,Tumor, Bednar,Tumor, Bednar's,Tumor, Darier-Ferrand,Tumor, Darier-Hoffmann

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