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Difference between revisions of "Dermatofibrosarcoma protuberans"
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Dermatofibrosarcoma protuberans (view source)
Revision as of 21:35, 11 November 2013
, 21:35, 11 November 2013more
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| Gross = firm plaque +/-ulceration | | Gross = firm plaque +/-ulceration | ||
| Grossing = | | Grossing = | ||
| Site = [[skin]] - usually trunk | | Site = [[skin]] - usually trunk or proximal extremities | ||
| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Clinicalhx = | | Clinicalhx = second to fifth decade | ||
| Signs = | | Signs = | ||
| Symptoms = | | Symptoms = | ||
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| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = | | Prognosis = moderate, locally aggressive, rarely metastases | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = | ||
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*Destroys adnexal structures. | *Destroys adnexal structures. | ||
*Occasionally transforms to a (more aggressive) [[adult fibrosarcoma|fibrosarcoma]].<ref name=pmid21128251>{{Cite journal | last1 = Stacchiotti | first1 = S. | last2 = Pedeutour | first2 = F. | last3 = Negri | first3 = T. | last4 = Conca | first4 = E. | last5 = Marrari | first5 = A. | last6 = Palassini | first6 = E. | last7 = Collini | first7 = P. | last8 = Keslair | first8 = F. | last9 = Morosi | first9 = C. | title = Dermatofibrosarcoma protuberans-derived fibrosarcoma: clinical history, biological profile and sensitivity to imatinib. | journal = Int J Cancer | volume = 129 | issue = 7 | pages = 1761-72 | month = Oct | year = 2011 | doi = 10.1002/ijc.25826 | PMID = 21128251 }}</ref> | *Occasionally transforms to a (more aggressive) [[adult fibrosarcoma|fibrosarcoma]].<ref name=pmid21128251>{{Cite journal | last1 = Stacchiotti | first1 = S. | last2 = Pedeutour | first2 = F. | last3 = Negri | first3 = T. | last4 = Conca | first4 = E. | last5 = Marrari | first5 = A. | last6 = Palassini | first6 = E. | last7 = Collini | first7 = P. | last8 = Keslair | first8 = F. | last9 = Morosi | first9 = C. | title = Dermatofibrosarcoma protuberans-derived fibrosarcoma: clinical history, biological profile and sensitivity to imatinib. | journal = Int J Cancer | volume = 129 | issue = 7 | pages = 1761-72 | month = Oct | year = 2011 | doi = 10.1002/ijc.25826 | PMID = 21128251 }}</ref> | ||
*Typically slow growing.<ref name=pmid23327727>{{Cite journal | last1 = Llombart | first1 = B. | last2 = Serra-Guillén | first2 = C. | last3 = Monteagudo | first3 = C. | last4 = López Guerrero | first4 = JA. | last5 = Sanmartín | first5 = O. | title = Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. | journal = Semin Diagn Pathol | volume = 30 | issue = 1 | pages = 13-28 | month = Feb | year = 2013 | doi = 10.1053/j.semdp.2012.01.002 | PMID = 23327727 }}</ref> | |||
*Usually second to fifth decade..<ref name=pmid23327727/> | |||
Treatment:<ref name=Ref_PBoD8_1183>{{Ref PBoD8|1183}}</ref> | Treatment:<ref name=Ref_PBoD8_1183>{{Ref PBoD8|1183}}</ref> | ||
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*[[Solitary fibrous tumour]]. | *[[Solitary fibrous tumour]]. | ||
*[[Undifferentiated pleomorphic sarcoma]]. | *[[Undifferentiated pleomorphic sarcoma]]. | ||
===Subtypes=== | |||
Numerous variants/subtypes are described:<ref name=pmid23327727>{{Cite journal | last1 = Llombart | first1 = B. | last2 = Serra-Guillén | first2 = C. | last3 = Monteagudo | first3 = C. | last4 = López Guerrero | first4 = JA. | last5 = Sanmartín | first5 = O. | title = Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. | journal = Semin Diagn Pathol | volume = 30 | issue = 1 | pages = 13-28 | month = Feb | year = 2013 | doi = 10.1053/j.semdp.2012.01.002 | PMID = 23327727 }}</ref> | |||
*Pigmented DFSP (Bednar tumour). | |||
*Myxoid DFSP. | |||
*Myoid DFSP. | |||
*Granular cell DFSP. | |||
*Sclerotic DFSP. | |||
*Atrophic DFSP, | |||
*Giant cell fibroblastoma. | |||
*DFSP with fibrosarcomatous areas. | |||
===Images=== | ===Images=== | ||