1/30. The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases.dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid-like plaques. We believe that DFSP in childhood is probably under-estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/30. 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.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/30. A pediatric case of dermatofibrosarcoma protuberans: an immunohistochemical study.dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that usually occurs in early or mid-adult life as a nodular cutaneous mass. DFSP has rarely been reported during childhood or at birth. We report a case of childhood DFSP that illustrates the usefulness of immunohistochemical analysis in the differential diagnosis between DFSP and other fibrohistiocytic proliferations occurring in the skin. A prompt and correct diagnosis is very important in order to ensure appropriate treatment and to prevent local recurrences.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
4/30. Congenital and childhood dermatofibrosarcoma protuberans: a case report and review of the literature.dermatofibrosarcoma protuberans (DFSP) occurs most commonly on the trunk, affects all races, and often develops between the second and fifth decades of life. It is uncommon in childhood and is sometimes mistaken for a vascular lesion, as it often presents as a blue macule or small nodule. review of the English literature revealed approximately 152 cases of DFSP developing before 16 years of age and only 19 claimed congenital cases. A case of congenital dermatofibrosarcoma protuberans is presented with a literature review and discussion of congenital and childhood presentations of the tumor.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/30. A case of a hard inguinal nodule.dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor with its onset typically in the second to fifth decades of life. It most commonly presents on the trunk, and recent cytogenetic studies suggest a neural origin. A case presentation and review of the recent literature on the diagnosis, differential diagnosis, and treatment of DFSP is presented.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/30. 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.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
7/30. Giant-cell fibroblastoma and dermato fibro sarcoma protuberans: the same tumoral spectrum? Report of two cases of association in children.We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/30. dermatofibrosarcoma protuberans with onset in early childhood: a case report.dermatofibrosarcoma protuberans is a tumour that grows locally, infiltrating the subcutaneous tissue; it is uncommon in children. We report the case of a 27-year-old man who had had such a lesion from the age of 3 years, and it had enlarged during his lifetime. A wide local excision with margins of 3.0 cm was performed, but the lesion was not entirely removed. This is a new report of infantile dermatofibrosarcoma protuberans, and this case reflects the need for Mohs micrographic surgery.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/30. Multiple spindle cell lipomas and dermatofibrosarcoma protuberans within a single patient: evidence for a common neoplastic process of interstitial dendritic cells?This case report describes a 48-year-old man with multiple spindle cell lipomas of the neck and a dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation of the chest. The presence of familial and nonfamilial multiple spindle cell lipomas within a single patient is a rare event, with only two reports in the current literature. This case represents the first report of multiple spindle cell lipomas occurring in association with a DFSP. It is of particular interest in that both spindle cell lipoma and DFSP represent, at least in part, neoplastic proliferations of CD34( ) spindled cells. The exact nature and differentiation of these spindled cells remains controversial, but prior studies have suggested that they could represent neoplastic interstitial dendritic cells. The association of DFSP and spindle cell lipoma within this single patient suggests that these two tumors (and their histologic variants) may well be linked, conceptually, as neoplastic proliferations of CD34( ) interstitial dendritic cells.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
10/30. Congenital dermatofibrosarcoma protuberans: variability in presentation.BACKGROUND: dermatofibrosarcoma protuberans (DFSP) is an uncommon low-grade fibrohistiocytic tumor that usually occurs on the trunk or proximal extremities and typically appears during the second to fifth decade of life. It most commonly begins as a red-blue plaque that grows slowly and ultimately becomes nodular. The tumor is associated with a high recurrence rate but low metastatic potential. It rarely presents in childhood and is even more rarely present at birth. The clinical diagnosis of DFSP in infancy or childhood may be difficult because, in its early stages, the tumor often resembles a vascular birthmark. OBSERVATIONS: We studied 6 patients with congenital DFSP who were initially thought to have other diagnoses, highlighting the potential clinical variability in presentation. Half of the cases in this series occurred in areas of the body outside of the typically reported distribution pattern of acquired DFSP and in locations that, therefore, may not arouse suspicion of congenital DFSP. CONCLUSIONS: Given the aggressive local potential and high recurrence rate of DFSP, early diagnosis is preferable to facilitate appropriate excision. We recommend that any infant or child presenting with a cutaneous plaque or nodule, even congenital, that does not have characteristic or diagnostic clinical features undergo tissue biopsy for histologic evaluation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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