{"doi":"10.1111/cup.12688","title":"An unusual presentation of dermatofibrosarcoma protuberans with pleomorphic sarcomatous transformation: potential pitfall and diagnostic strategy","abstract":"<jats:p>Dermatofibrosarcoma protuberans (<jats:styled-content style=\"fixed-case\">DFSP</jats:styled-content>) is a low grade, superficial sarcoma characterized by a proliferation of monomorphous, spindle cells arranged in a storiform pattern and infiltrating the subcutaneous tissue. The tumor is typically <jats:styled-content style=\"fixed-case\">CD34</jats:styled-content> positive, and shows the characteristic <jats:styled-content style=\"fixed-case\">COL1A1‐PDGFB</jats:styled-content> fusion gene, detectable either by florescent <jats:italic>in situ</jats:italic> hybridization (<jats:styled-content style=\"fixed-case\">FISH</jats:styled-content>) and polymerase chain reaction (<jats:styled-content style=\"fixed-case\">PCR</jats:styled-content>). We describe a case of <jats:styled-content style=\"fixed-case\">DFSP</jats:styled-content> with a focus of peculiar pleomorphic sarcomatous transformation. The focus constituted the entire bioptic tissue that was initially excised, raising considerable diagnostic problems for pathologist. The use of <jats:styled-content style=\"fixed-case\">FISH</jats:styled-content> as an ancillary technique allowed the right diagnosis.</jats:p>","journal":"Journal of Cutaneous Pathology","year":2016,"id":614170,"datarank":0.31191623125197543,"base_score":2.0794415416798357,"endowment":2.0794415416798357,"self_citation_contribution":0.31191623125197543,"citation_network_contribution":0.0,"self_endowment_contribution":0.31191623125197543,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":7,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1582604,"name":"Ema Mataca","orcid":null,"position":1,"is_corresponding":false},{"id":1582606,"name":"Claudio Gambini","orcid":null,"position":2,"is_corresponding":false},{"id":620914,"name":"Heinz Kutzner","orcid":"0000-0001-6248-3050","position":3,"is_corresponding":false},{"id":1582602,"name":"Anna Maria Cesinaro","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"An unusual presentation of dermatofibrosarcoma protuberans with pleomorphic sarcomatous transformation: potential pitfall and diagnostic strategy","abstract":"<jats:p>Dermatofibrosarcoma protuberans (<jats:styled-content style=\"fixed-case\">DFSP</jats:styled-content>) is a low grade, superficial sarcoma characterized by a proliferation of monomorphous, spindle cells arranged in a storiform pattern and infiltrating the subcutaneous tissue. The tumor is typically <jats:styled-content style=\"fixed-case\">CD34</jats:styled-content> positive, and shows the characteristic <jats:styled-content style=\"fixed-case\">COL1A1‐PDGFB</jats:styled-content> fusion gene, detectable either by florescent <jats:italic>in situ</jats:italic> hybridization (<jats:styled-content style=\"fixed-case\">FISH</jats:styled-content>) and polymerase chain reaction (<jats:styled-content style=\"fixed-case\">PCR</jats:styled-content>). We describe a case of <jats:styled-content style=\"fixed-case\">DFSP</jats:styled-content> with a focus of peculiar pleomorphic sarcomatous transformation. The focus constituted the entire bioptic tissue that was initially excised, raising considerable diagnostic problems for pathologist. The use of <jats:styled-content style=\"fixed-case\">FISH</jats:styled-content> as an ancillary technique allowed the right diagnosis.</jats:p>","is_dataset_classified":null,"base_score":2.0794415416798357,"endowment":2.0794415416798357,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"26955897","pmcid":null,"openalex_id":"https://openalex.org/W2302627870","authors":[],"funders":[],"total_grants":0,"fwci":0.4689,"citation_percentile":0.67858644,"influential_citations":0,"citation_trend":[{"year":2016,"count":1},{"year":2018,"count":1},{"year":2020,"count":1},{"year":2025,"count":2},{"year":2026,"count":2}],"oa_status":"closed","license":"http://onlinelibrary.wiley.com/termsAndConditions#vor","oa_locations":[{"url":"https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fcup.12688","host_type":"publisher"},{"url":"https://onlinelibrary.wiley.com/doi/pdf/10.1111/cup.12688","host_type":"publisher"},{"url":"https://doi.org/10.1111/cup.12688","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/26955897","host_type":"repository"}],"fields_of_study":["Sarcoma Diagnosis and Treatment","Tumors and Oncological Cases","Vascular Tumors and Angiosarcomas"],"mesh_terms":[],"keywords":["Dermatofibrosarcoma protuberans","PDGFB","Pathology","Dermatofibrosarcoma","CD34","Undifferentiated Pleomorphic Sarcoma","Sarcoma","Factor XIIIa","Biology","Polymerase chain reaction","Fusion gene","Soft tissue","Soft tissue sarcoma","Medicine","Immunohistochemistry","Gene","Stem cell","Growth factor","Transformation","FISH","Pleomorphic Sarcoma"],"sdg_mappings":[{"sdg_number":0,"sdg_label":"Life below water"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-02T11:15:09.547753Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}