Cases reported "Dermatofibrosarcoma"

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1/7. Composite cervicofacial flap for reconstruction of complex cheek defects.

    The authors present the reconstructive technique for complex cheek defects using the composite cervicofacial flap and study the possibilities, advantages, disadvantages, and results that can be expected. The design follows the classic outline of Mustarde's flap. The skin is undermined for 2 cm anterior to the ear, then after incision of the superficial musculoaponeurotic system (SMAS), undermining is continued below the plane of the SMAS, level with the facial nerve branches. It is continued forward to the facial vessels, which give rise to branches that ensure the blood supply of this composite flap and contribute to its high reliability. In the cervical region, undermining is done beneath the platysma, which is transected transversely in the lower cervical region to allow good upward mobility and satisfactory transposition of the flap. The flap is adapted to the defect and the medial suture line is placed as near as possible to the medial limit of the cheek aesthetic unit. The authors carried out a retrospective study of 7 patients with complex facial reconstruction after excision of malignant lesions. The defects measured from 4x4 cm to 9x7 cm. In 4 patients excision included the periosteum, and in 1 patient excision involved the entire thickness and removed the entire anterior half of the cheek. In 4 patients reconstruction involved the cheek and eyelid. In spite of the advanced age of the patients (88, 69, 91, 67, 70, 82, and 59 years), there was no distal edge necrosis. The only complication was a single case of facial paresis, which resolved spontaneously. The results were considered very good in all 7 patients. The authors conclude that the composite flap increases the possibilities of the cervicofacial flap. It is more mobile, more reliable, thicker, and more adaptable. It can be used in complex cheek defects that involve the periosteum, or even in full-thickness defects. The quality of the results obtained using this flap represents a considerable advance in facial reconstruction.
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2/7. Detection of COL1A1-PDGFB fusion transcripts and platelet-derived growth factor alpha and beta receptors in giant cell fibroblastoma of the postsacrococcygeal region.

    We describe a 2-year-old girl with recurrent giant cell fibroblastoma (GCF) of the postsacrococcygeal region. Both the initial and recurrent tumours contained solid and angiectoid areas. The former was composed of loosely arranged wavy spindle cells and giant cells with a well-vascularized myxoid to collagenous stroma. The angiectoid spaces were often lined by multinucleated giant cells. Immunohistochemically, the tumour cells and small vessels in the tumour tissue were positive for platelet-derived growth factor (PDGF) alpha and beta receptors. Molecular analysis revealed fusion of collagen type Ialpha1 exon 26 with PDGF-B chain exon 2 that induced unscheduled production of PDGF-BB. These findings suggest that PDGF and its receptors significantly contribute to the development of GCF in both an autocrine and a paracrine manner.
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3/7. Myoid differentiation and EMA expression in fibrosarcomatous dermatofibrosarcoma protuberans.

    dermatofibrosarcoma protuberans (DFSP) is a relatively unusual, locally aggressive cutaneous tumor of intermediate malignancy. Fibrosarcomatous DFSP (FS-DFSP), a rare variant of DFSP, has a higher tendency for recurrence and metastasis. Recently, a small number of cases of another variant of FSDFSB characterized by areas of myoid differentiation have been reported. We present here a 35 yearold female patient with myoid differentiation in FS-DFSP. The tumor on the left scapular region had slowly grown over six years. Examination revealed a domeshaped, firm, nontender, violaceous dermal nodule. Histologically, it was composed of a monotonous spindle cell population arranged predominantly in a storiform pattern and to a lesser extent in a fascicular fibrosarcomatous pattern with a parallel arrangement of the cells. Immunohistochemically, the tumor cells showed diffuse expression for vimentin and CD34. In the center of the tumor areas with frequent mitosis, hypercellular and negative reactive for CD34 were seen. In addition, approximately 10% of the cells were positive for epithelial membrane antigen. Myoid differentiation was found around the blood vessels. The myoid areas were positive for smooth muscle actin and negative for desmin. It is possible that the presence of hyperplastic myofibroblasts is a reactive phenomenon to the proliferation of tumor cells. We believe that this finding around blood vessels may be present in DFSP or FS-DFSP. However, when myoid areas, myoid fascicles and myoid nodules are seen in the stroma, it may be a new morphological variant of DFSP and/or FS-DFSP.
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4/7. CD34-reactive fibrous papule of the nose.

    In human skin, the CD34 antigen is expressed on endothelium, periadnexal cells, and a population of reticular dermal interstitial cells. CD34 expression is characteristic of dermatofibrosarcoma protuberans and several other neoplasms, but not of typical fibrous papules of the nose. We describe a 16-year-old white girl with a slowly growing papule on the nose. Histopathology showed a dermal tumor with a superficial component of branched, thin-walled blood vessels and a deeper component of benign-appearing, spindle-shaped cells. These cells uniformly and strongly expressed CD34, but not factor xiiia or markers of melanocytic, neural, muscular, vascular, or histiocytic differentiation. We consider this lesion a CD34-reactive fibrous papule. This benign tumor must be clearly distinguished from dermatofibrosarcoma protuberans, which also is composed of bundles of CD34-reactive spindle-shaped cells in most cases but has locally aggressive behavior.
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5/7. Leiomyomatous nodules and bundles of vascular origin in the fibrosarcomatous variant of dermatofibrosarcoma protuberans.

    We report 5 cases of the fibrosarcomatous variant of dermatofibrosarcoma protuberans, 4 of which presented a morphologic change of intraneoplastic blood vessels not previously recognized. This change consisted of focal proliferation of smooth muscle cells, resulting in hypertrophy, generally eccentric, of vascular walls with reduction and collapsing of vascular lumina. In 3 cases the proliferation was so intense it formed leiomyomatous nodules and bundles. This proliferation may originate in the smooth muscle cells of the vessel walls either by means of a hyperplastic mechanism or in the pericytes via a line of differentiation leading to mature smooth muscle cells. In either case, we believe that it concerns a reactive process of the vessel walls very probably induced by adjacent neoplastic cells. The cases recently reported by Calonje and Fletcher as "myoid differentiation" of neoplastic cells in dermatofibrosarcoma protuberans (DFSP) may well be an expression of the same phenomenon, and therefore the presence of leiomyomatous areas in this tumor should not be used to support the theory of a fibroblastic/myofibroblastic line of differentiation for DFSP.
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6/7. dermatofibrosarcoma protuberans and its fibrosarcomatous variant with areas of myoid differentiation: a report of three cases.

    AIMS: We describe three examples of dermatofibrosarcoma protuberans which demonstrated focal myofibroblastic differentiation, and discuss the nature of myofibroblastic differentiation. methods AND RESULTS: We studied three cases of dermatofibrosarcoma protuberans, two of which had fibrosarcomatous portions containing myoid areas, and one which showed myoid areas in ordinary dermatofibrosarcoma protuberans. Myoid areas were recognized histologically as randomly distributed, scattered bundles or small nodules of spindle cells with deeply eosinophilic cytoplasm. Myofibroblastic differentiation was demonstrated immunohistochemically by positive staining for smooth muscle actin and muscle-specific actin, and ultrastructurally by the presence of microfilament bundles with focal dense bodies and pinocytic vesicles. The myoid areas occasionally contained intraneoplastic blood vessels. CONCLUSION: The presence of myoid areas may be related to reactive hyperplasia of stroma, and its relation to the histogenesis of dermatofibrosarcoma protuberans is uncertain.
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7/7. A cutaneous case of giant cell angiofibroma occurring with dermatofibrosarcoma protuberans and showing bimodal CD34 fibroblastic and FXIIIa histiocytic immunophenotype.

    Dei Tos and colleagues in 1995 reported a series of seven distinctive orbital tumors in adults which they named giant cell angiofibroma (GCA). The morphologic features are intermediate between giant cell fibroblastoma and solitary fibrous tumor with a richly vascularized, patternless spindle cell proliferation forming a collagenous or myxoid stroma with pseudovascular angiectoid spaces. The spindled tumor cells have large, rounded nuclei, sometimes with complex folded shape and pseudoinclusions. There also are multi- or mononuclear giant cells, and these tumor cells partly line so-called angiectoid spaces. Cells express human progenitor cell antigen CD34 and vimentin. One case in the buccinator fascia was also noted by the authors, but similar cutaneous lesions are thus far unknown. We report our experience with a polypoid tumor that ocurred on the thigh of a 49-year-old woman that conforms to the description of GCA. The tumor has variegated vessels admixed with patternless spindle and giant cell stroma with angiectoid spaces as well as areas of dermatofibrosarcoma protuberans (DFSP). Most tumor cells express vimentin and CD34, including giant and spindle cells lining angiectoid spaces. Focally up to 40% of the lesional cells express coagulation factor xiiia with histiocytoid to highly dendritic cytosomes. The DFSP component is composed of admixed CD34 and FXIIIa dendritic cells arranged in a storiform pattern. Tumor cells are negative for actin, desmin, S-100, and cytokeratin. The Ki67 proliferation index is 1% in GCA areas and 3% in DFSP areas; Ki 67 stains mainly fibroblasts. We conclude that this cutaneous GCA is a fibrohistiocytic tumor closely related to and representing a more organoid angioformative analog of GCF, with both being related histogenetically also to DFSP. These lesions represent part of a greater spectrum of fibrovascular tissue patterns, all probably derived from proliferations of interactive microvascular CD34 fibroblasts and FXIIIa histiocytes.
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