## Abstract The presence of an extra ring chromosome containing material from 17q and 22q, or, less frequently, a t(17;22)(q22;q13), is a cytogenetic hallmark of dermatofibrosarcoma protuberans (DFSP). However, occasionally tumors with other, atypical karyotypes are encountered. We describe a case
Involvement of chromosomes 17 and 22 in dermatofibrosarcoma protuberans
β Scribed by Fabiola Minoletti; Monica Miozzo; Florence Pedeutour; Laura Sard; Silvana Pilotti; Alberto Azzarelli; Claude Turc-Carel; Marco A. Pierotti; Gabriella Sozzi
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 306 KB
- Volume
- 13
- Category
- Article
- ISSN
- 1045-2257
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β¦ Synopsis
Literature on the cytogenetics of dermatofibrosarcoma protuberans (DFSP) is limited; only I0 cases with chromosome aberrations have been reported. They are karyotypically characterized by the presence of supernumerary ring(s), either as the sole cytogenetic abnormality or together with a few additional structural or numerical changes. W e report the cytogenetic and fluorescence in situ hybridization (FISH) analysis of three new DFSP, one primary and two recurrent tumors. In two cases we found a supernumerary ring as the sole change, whereas the third had two copies of a marker chromosome and monosomy of chromosome 22. Sequences of chromosomes I7 and 22 were identified by FISH in the supernumerary rings and in the markers.
The fluorescence pattern suggested that additional sequences were present in the two rings, but showed that the marker chromosomes were entirely painted by chromosome 17 and 22 probes. The findings indicate that juxtaposition and/or amplification of chromosome 17 and 22 sequences could be crucial in the pathogenesis of DFSP. Genes Chromosom Cancer 13:62-65 (1995).
π SIMILAR VOLUMES
Dermatofibrosarcoma protuberans (DP) is a rare, slow-growing, infiltrating dermal neoplasm of intermediate malignancy, made up of spindle-shaped tumor cells often positive for CD34. The preferred treatment is wide surgical excision with pathologically negative margins. At the cytogenetic level, DP c
## Abstract Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous malignant tumor that usually occurs in the trunk, extremities, head, and neck but very rarely in the breast. Fibrosarcomatous transformation of DFSP is even rarer, with a higher risk of distant metastasis and poorer prognosis th