{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T02:31:08Z","timestamp":1791858668066,"version":"4.3.4"},"reference-count":17,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2020,4,6]],"date-time":"2020-04-06T00:00:00Z","timestamp":1586131200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cutan Pathol"],"published-print":{"date-parts":[[2020,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pleomorphic fibromas are rare benign cutaneous neoplasms associated with deletion\/loss of chromosomes 13q and 17p, where <jats:italic>RB1<\/jats:italic> and <jats:italic>TP53<\/jats:italic> are located, respectively. Herein, we report five cases of pleomorphic fibroma arising in patients with germline <jats:italic>TP53<\/jats:italic> mutations, suggesting a potential link with Li\u2010Fraumeni syndrome. All three patients were female and young (mean age 27) with a strong personal and\/or family oncologic history and confirmed pathogenic germline <jats:italic>TP53<\/jats:italic> mutations. In two patients, multiple pleomorphic fibromas were diagnosed. Clinically, the lesions arose at various cutaneous sites and were small (\u22642\u2009cm) and raised (4\/5). Histopathologically, the tumors were paucicellular, composed of atypical spindled to stellate cells with hyperchromatic and variably pleomorphic nuclei. Mitotic activity was exceedingly low, although rare atypical mitotic figures were seen in one case. Immunohistochemically, the tumor cells were diffusely positive for p16 (3\/3) and showed loss of Rb expression (5\/5). All cases showed aberrant p53 expression (overexpression in 4, complete loss in 1). The tumors have followed a benign clinical course with no evidence of progression or recurrence. In conclusion, the development of multiple pleomorphic fibromas in a young patient may be a clue to an underlying genetic cancer syndrome involving <jats:italic>TP53<\/jats:italic>.<\/jats:p>","DOI":"10.1111\/cup.13686","type":"journal-article","created":{"date-parts":[[2020,3,18]],"date-time":"2020-03-18T21:42:05Z","timestamp":1584567725000},"page":"734-741","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Cutaneous pleomorphic fibromas arising in patients with germline <scp><i>TP53<\/i><\/scp> mutations"],"prefix":"10.1111","volume":"47","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8630-8609","authenticated-orcid":false,"given":"Jeffrey M.","family":"Cloutier","sequence":"first","affiliation":[{"name":"Department of Pathology Stanford University School of Medicine  Stanford California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara C.","family":"Shalin","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Arkansas for Medical Sciences  Little Rock Arkansas"},{"name":"Department of Dermatology University of Arkansas for Medical Sciences  Little Rock Arkansas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Lindberg","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Arkansas for Medical Sciences  Little Rock Arkansas"},{"name":"Department of Dermatology University of Arkansas for Medical Sciences  Little Rock Arkansas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jerad M.","family":"Gardner","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Arkansas for Medical Sciences  Little Rock Arkansas"},{"name":"Department of Dermatology University of Arkansas for Medical Sciences  Little Rock Arkansas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0428-0199","authenticated-orcid":false,"given":"Sebastian","family":"Fernandez\u2010Pol","sequence":"additional","affiliation":[{"name":"Department of Pathology Stanford University School of Medicine  Stanford California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Zaba","sequence":"additional","affiliation":[{"name":"Department of Dermatology Stanford University School of Medicine  Stanford California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Novoa","sequence":"additional","affiliation":[{"name":"Department of Pathology Stanford University School of Medicine  Stanford California"},{"name":"Department of Dermatology Stanford University School of Medicine  Stanford California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9099-7227","authenticated-orcid":false,"given":"Ryanne A.","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Pathology Stanford University School of Medicine  Stanford California"},{"name":"Department of Dermatology Stanford University School of Medicine  Stanford California"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,4,6]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-198902000-00003"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/cup.12965"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-12-0095"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/scisignal.2004088"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2010.12.016"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2018.205013"},{"key":"e_1_2_5_8_1","unstructured":"VCV0004065901 \u2010 ClinVar \u2010 NCBI.https:\/\/www.ncbi.nlm.nih.gov\/clinvar\/variation\/406590\/. 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