Cases reported "Dermatofibrosarcoma"

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1/46. An ultrastructural and immunohistochemical study of pigmented dermatofibrosarcoma protuberans (Bednar tumor).

    A case of Bednar tumor on the right shoulder of a 47-year-old Japanese woman is reported. Histological examination showed plump, spindle cells arranged in a storiform pattern in central areas of the tumor and a diffuse infiltration of the dermal stroma, which was frequently extended into the subcutis at the periphery of the tumor. The tumor contained a fairly identified population of dendritic pigmented cells. Ultrastructurally, most cells had folded nuclei, were spindle-shaped and had long, slender cytoplasmic projections. Dendritic pigmented cells, which were dispersed among neoplastic cells, contained premelanosomes and mature melanosomes. Immunohistochemically, tumor cells exhibited positive reactions for vimentin and CD 34 and failed to show a positive reaction for neuron specific enolase, HMB-45 or S-100 protein. Factor X IIIa was only expressed on tumor cells around melanin-containing cells, which reacted positively with antibodies to S-100 protein and vimentin. These results indicate that the phenotype of tumor cells around melanin-containing cells differs from other tumor cells and that this difference may be caused by the relationship of tumor cells and melanin-containing cells.
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keywords = spindle cell, spindle
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2/46. dermatofibrosarcoma protuberans of the oral cavity.

    dermatofibrosarcoma protuberans (DFSP) is a low grade, malignant spindle cell tumor with an infiltrative growth pattern and a high rate of local recurrence. This tumor's cell of origin is controversial. DFSP usually presents in adult life and is most frequently located on the trunk and proximal extremities. Although 10% to 15% of cases involve the head and neck, this tumor has not been previously described in the oral cavity.
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ranking = 0.80731831563501
keywords = spindle cell, spindle
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3/46. dermatofibrosarcoma protuberans of the vulva: clinicopathologic and immunohistochemical analysis of four cases, one with fibrosarcomatous change, and review of the literature.

    Only 18 cases of dermatofibrosarcoma protuberans (DFSP) of the vulva have been reported. This article describes the clinicopathologic and immunohistochemical findings of four additional vulvar DFSPs. The median patient age was 54 years (range, 36 to 69 years) and the most common clinical presentation was a slowly growing vulvar mass. The tumors occurred in the left paraclitoral area (1 case), right labium majus (1 case), left labium majus (1 case), and mons pubis (1 case). The tumor size, known in three cases, ranged from 1.2 to 5.0 cm in greatest dimension. Microscopically, the tumors were composed of bland spindle cells with oval or wavy nuclei arranged in a cartwheel pattern; in one case, fibrosarcomatous areas were apparent. CD34 immunostaining was positive in the three cases in which it was performed; positive staining was also seen in the area of fibrosarcoma. Estrogen and progesterone receptor immunostains were negative in three cases. Of the three patients who underwent surgery, one had tumor recurrences every 2 to 4 years for 18 to 20 years but has remained free of tumor in the 7 years since the last excision, one was free of tumor for 12 years before dying of an unrelated cause, and the other has been free of disease for 6 months. The fourth patient refused treatment and has persistent disease in the vulva. This study shows that the behavior of DFSP in the vulva is similar to its behavior in its more common locations.
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ranking = 0.80731831563501
keywords = spindle cell, spindle
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4/46. FNAC in the diagnosis of recurrent dermatofibrosarcoma protuberans of the forehead. A case report.

    BACKGROUND: dermatofibrosarcoma protuberans is a rare cutaneous soft tissue tumor of intermediate malignant potential with a characteristic tendency for recurrence. Metastases are unusual. This tumor usually occurs in the trunk and extremities and, infrequently, on the face and scalp. Its cytologic appearance on fine needle aspiration has only been rarely reported. It is characterized by numerous fibroblastlike cells that arrange as single cells or in clusters of spindle cells arrayed in a storiform pattern. CASE: A 42-year-old male presented with a one-year history of an enlarging left forehead mass (lateral brow) that was adjacent to an old surgical scar. Fine needle aspiration revealed a low grade spindle cell neoplasm morphologically identical to a dermatofibrosarcoma protuberans excised 15 years earlier, indicating tumor recurrence. CONCLUSION: Distinguishing dermatofibrosarcoma protuberans from other spindle cell tumors and fibrohistiocytic lesions may pose significant challenges to the pathologist. However, in the appropriate clinical setting and applying strict diagnostic criteria, fine needle aspiration cytology is a reliable tool in establishing the diagnosis of this neoplasm.
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ranking = 2.421954946905
keywords = spindle cell, spindle
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5/46. dermatofibrosarcoma protuberans growing around plantar aponeurosis: excision by Mohs micrographic surgery.

    BACKGROUND: dermatofibrosarcoma protuberans (DFSP) is a spindle cell malignancy that has a high local recurrence rate after excision with minimal or no immediate tissue margin assessment. DFSP is exceedingly rare on the palms and soles. OBJECTIVE: To report a case of a locally aggressive DFSP on the sole excised using Mohs micrographic surgery. methods: Case report and review of the literature. RESULTS: Mohs micrographic surgery unmasked tumor infiltration that extended around plantar aponeurosis and into underlying plantar muscle fascia. CONCLUSION: Mohs micrographic surgery should be considered the treatment of choice for DFSP, especially in acral locations. This technique allows the surgeon to trace out deep tumor extensions that may wrap around underlying tendon, a finding that may not be appreciated clinically.
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ranking = 0.80731831563501
keywords = spindle cell, spindle
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6/46. Palisading and verocay body-prominent dermatofibrosarcoma protuberans: a report of three cases.

    The aim of this report is to draw attention to nuclear palisading and Verocay body formation as peculiar, previously undescribed histological findings in rare instances of dermatofibrosarcoma protuberans (DFSP). methods AND RESULTS: Three indurated, nodular or plaque skin lesions were diagnosed as DFSP on the basis of their storiform proliferation of spindle-shaped cells diffusely infiltrating the dermis and subcutaneous tissue. Sclerosing and giant cell areas were also identified. Unexpectedly, conspicuous nuclear palisading was also noted in all cases and Verocay body formation was present in two. Immunostains were positive for CD34 and negative for S100 protein in every instance. Proliferating cells were seen to display fibroblast-like features by ultrastructural study of one case. CONCLUSIONS: DFSP may rarely show a schwannoid histological appearance as the result of nuclear palisading and even Verocay body formation. In this setting, both the search for DFSP characteristic morphologic features and the performance of CD34 and S100 protein immunohistochemistry will facilitate the correct diagnosis.
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ranking = 0.19268168436499
keywords = spindle
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7/46. Fibrosarcomatous dermatofibrosarcoma protuberans with giant rosettes.

    A rare case of dermatofibrosarcoma protuberans, which contained low-grade fibrosarcoma with giant rosettes in its recurrence areas is described. The giant rosettes lying in the fibrosarcomatous area produced a pattern similar to that of so-called hyalinizing spindle cell tumor with giant rosettes. The transitions between the tumor components were gradual, and both primary and recurrent lesions showed diffuse immunoreactivity for CD34. The presence of giant rosettes in fibrosarcomatous areas of dermatofibrosarcoma protuberans suggests a possible histogenetic relationship between fibrosarcomatous areas of DFSP and hyalinizing spindle cell tumor with giant rosettes.
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ranking = 1.61463663127
keywords = spindle cell, spindle
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8/46. Bednar tumor of the foot: a case report.

    Pigmented dermatofibrosarcoma protuberans (Bednar tumor) is a rare neoplasm accounting for approximately 1-5% of all cases of dermatofibrosarcoma protuberans. The majority occurs on the trunk, and the remainders are more or less equally distributed in the upper and lower extremities and the head and neck. Microscopically it is characterized by spindled cells arranged in a tight storiform pattern and admixed with a small population of melanin-containing dendritic cells. The dendritic cells are the primary feature that distinguish this lesion from conventional dermatofibrosarcoma protuberans. We report here a case of Bednar tumor occurring on the dorsal aspect of the foot in a young female.
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ranking = 0.19268168436499
keywords = spindle
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9/46. Pigmented neurofibroma: report of two cases and literature review.

    Two cases of pigmented neurofibroma of the skin are reported. In case 1, the tumor was removed from the back of a 55-year-old man with no associated neurofibromatosis. In case 2, the tumor was removed from the abdominal wall of a 21-year-old woman with neurofibromatosis. Both tumors consisted of benign, short spindle cells and multiple foci of scattered melanin-laden cells. In case 1, the spindle cells were arranged in a storiform pattern, resembling features of dermatofibrosarcoma protuberans. Immunohistochemically, the spindle cells of both cases were demonstrated to be positive for S-100 protein and CD34. The melanin-laden cells stained positively for HMB-45. This report describes an additional two cases of pigmented neurofibroma that conform to the new diagnostic criteria for this disease.
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ranking = 2.421954946905
keywords = spindle cell, spindle
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10/46. Giant dermatofibroma with monster cells.

    We report a case of a 64-year-old woman with a giant dermatofibroma on her back with the unusual histologic feature of monster cells. The firm, exophytic, 3-cm nodule had purple and yellow components with surface telangiectasia. Histologic examination demonstrated characteristic findings of a dermatofibroma, including rete ridge flattening and bridging; a stroma containing scattered, large, round, eosinophilic collagen bundles; and a polymorphous dermal infiltrate of spindle and xanthomatous cells with scattered siderophages. Some xanthomatous cells demonstrated features consistent with monster cells, including huge bizarre nuclei and one or more nucleoli. Immunohistochemical staining for factor xiiia was positive. A diagnosis of giant dermatofibroma with monster cells (DFMC) was made. Giant dermatofibromas are rare, with monster cells being an uncommon finding in dermatofibroma. To our knowledge, this is the first report of DFMC.
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ranking = 0.19268168436499
keywords = spindle
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