1/25. A case of dermatofibrosarcoma protuberans with a ring chromosome 5 and a rearranged chromosome 22 containing amplified COL1A1 and PDGFB sequences.dermatofibrosarcoma protuberans (DFSP) is a cutaneous tumour of borderline malignancy, the cytogenetic features of which include the translocation t(17;22)(q22;q13) or, more commonly, supernumerary ring chromosomes containing material from 17q22 and 22q13. These rearrangements result in the COL1A1/PDGFB fusion gene. Here, we describe a case of DFSP displaying a ring chromosome 5 together with a large marker chromosome composed of chromosome 22 alphoid dna, material from distal 12q and amplified COL1A1 and PDGFB sequences. This is the first case of DFSP with multiple copies of COL1A1 and PDGFB not confined to ring chromosomes, showing that DFSP is similar to other borderline malignant mesenchymal tumours, where rings and giant markers are alternative vehicles for amplified material.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/25. A case of dermatofibrosarcoma protuberans of the vulva with a COL1A1/PDGFB fusion identical to a case of giant cell fibroblastoma.dermatofibrosarcoma protuberans (DFSP) is a highly recurrent low-grade soft tissue sarcoma, which is usually located on the trunk. Presentation in the vulva is rare, with only 13 cases being reported to date, none of which have been investigated at the cytogenetic or molecular level. Specific cytogenetic abnormalities, involving chromosomes 17 and 22, are characteristic features of DFSP and giant cell fibroblastoma (GCF), a tumor closely related to DFSP. These chromosomal rearrangements result in the fusion of the COL1A1 and PDGFB genes in both lesions and show wide variation in the position of the fusion point in COL1A1. Here, we describe a case of DFSP of the vulva with a typical monotonous storiform pattern, with no foci of multinucleated giant cells. cytogenetic analysis showed a 47,XX, r karyotype in 50% of the cells, and molecular investigation disclosed the presence of a transcript fusing COL1A1 exon 37 to PDGFB exon 2. This is the first case of DFSP showing such a fusion point, which is intriguingly identical to that found in a GCF case, indicating that the COL1A1/PDGFB fusion point position does not seem to affect tumor morphology. This finding further underlines the very close relationship between these two morphologically distinct entities.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |
3/25. 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 = 1keywords = giant (Clic here for more details about this article) |
4/25. 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.- - - - - - - - - - ranking = 9keywords = giant (Clic here for more details about this article) |
5/25. 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 = giant (Clic here for more details about this article) |
6/25. Composite tumor consisting of dermatofibrosarcoma protuberans and giant cell fibroblastoma associated with intratumoral endometriosis. Report of a case.We present a unique case of composite skin tumor of the vulva consisting of dermatofibrosarcoma protuberans (DFSP) and giant cell fibroblastoma (GCF) with an intratumoral focus of endometriosis. A 31-year-old female with a 10-year-history of a recurring subcutaneous tumor in the vulvar area underwent excision of the seventh recurrence of the tumor. Microscopic examination revealed a composite fibrohistiocytic tumor consisting of DFSP and GCF. Additionally, a focus of endometriosis within the tumor tissue was found. Malignant transformation of extragonadal endometriosis has already been described; we present, however, the occurrence of a focus of endometriosis within the tissue of a hormonally independent soft tissue tumor. There is a possible link to the occurrence of cutaneous endometriosis at previous surgery sites and in the scars. The presence of endometriosis within the soft tissue tumor represents, to the best of our knowledge, a previously undescribed collision phenomenon.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
7/25. 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.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
8/25. Giant cell fibroblastoma in a child misdiagnosed as a dermatofibroma.We report a 9-year-old African-American boy with a giant cell fibroblastoma of the shoulder that was incorrectly diagnosed as a keloid and dermatofibroma. Initial misdiagnosis led to a delay of 4 years in the correct diagnosis, with the tumor producing significant local destruction. We review herein the clinical manifestations, histologic findings, histogenesis, relationship to dermatofibrosarcoma protuberans (DFSP), treatment, and differential diagnosis of giant cell fibroblastoma (GCF). This information is important in correctly diagnosing this uncommon, benign, but locally aggressive and recurrent tumor of childhood. The clinician should consider GCF and DFSP when the pathologic diagnosis of dermatofibroma is made in lesions more than 2 cm in diameter, or when this diagnosis is made in a prepubertal child.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
9/25. Giant fibrosarcoma arising in dermatofibrosarcoma protuberans on the scalp during pregnancy.BACKGROUND: dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence; however, it seldom metastasizes. Most lesions occur over the trunk or proximal extremities, and there is slight male preponderance. DFSP is rare on the head and neck. Less than 5% tumors are located on the scalp. OBJECTIVE: To describe a case of giant fibrosarcoma arising in DFSP on the scalp during pregnancy. METHOD: In our patient, the tumor was on scalp and measured 15 x 11 x 7 cm. The recurrent tumor that developed in the same region was measured 33 x 30 x 15 cm. Histopathologic examination of this mass revealed fibrosarcoma. The mass was excised from the subperiosteal plane with a 3-cm free margin. Because of the high possibility of recurrence, the defect site was reconstructed with a split-thickness skin graft. RESULTS: After 1 year of follow-up, no recurrence or metastasis was detected. CONCLUSION: We present a unique case in which the person has a giant fibrosarcoma on her scalp arising from DFSP with fibrosarcomatous change during pregnancy.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
10/25. Composite dermatofibrosarcoma protuberans-giant cell fibroblastoma recurring as Bednar tumor-giant cell fibroblastoma with mucoid lakes and with amputation neuroma.We report an unusual case of composite giant cell fibroblastoma-dermatofibrosarcoma protuberans (DFSP) that, in its second recurrence, contained a pattern of Bednar tumor (BT) and giant cell fibroblastoma (GCF). The recurrent tumor showed extreme myxoid change with creation of mucoid lakes, which mimicked a pattern of myxoid liposarcoma. One area in the recurrent lesion contained amputation neuroma overgrown with neoplastic spindle cells, which simulated a nerve sheath neoplasm. This case demonstrates common histogenesis of GCF, DFSP and BT, and it shows how broad morphological spectrum can be produced by a composite tumor, especially when the tumor includes unconventional growth pattern or additional non-neoplastic lesion.- - - - - - - - - - ranking = 10keywords = giant (Clic here for more details about this article) |
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