{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T13:39:25Z","timestamp":1790429965564,"version":"4.1.0"},"reference-count":15,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,4]]},"abstract":"<jats:p>Perivascular epithelioid cell tumor (PEComa), mesenchymal tumors morphologically characterized by epithelioid cells, coexpress melanocytic and muscle markers. Herein, we describe a heretofore-undescribed tuberous sclerosis complex (TSC)-related neoplasm, morphologically resembling a soft tissue fibroma-like lesion, but showing an immunophenotype resembling PEComa. We identified 3 soft tissue fibroma-like lesions in individuals with TSC. We also evaluated 6 TSC-related periungual fibroma as well as a range of non-TSC fibroma-like lesions (n=19). Immunohistochemistry for HMB-45, desmin, smooth muscle actin, TFE3, and S100 was performed on the TSC-related fibromas. Periungual fibromas and non-TSC fibroma-like lesions were also stained for HMB-45. All 3 TSC patients were female, ranging in age from 4 to 51 years (mean, 26.7\u2009y). Two tumors were located in extremities and 1 on the chest wall. The tumors showed elongated to stellate spindle-shape cells, prominent collagenous background, and lacked mitotic activity and cytologic atypia. Immunohistochemically, all 3 tumors were positive for HMB-45; smooth muscle actin or desmin was positive in both tumors tested. TFE3 was negative. All patients were alive with no evidence of disease with median follow-up of 55 months (range, 6 to 131\u2009mo). Non-TSC fibroma-like lesions and oral and periungual fibromas were negative for HMB-45. Fibroma-like PEComa, a newly recognized soft tissue tumor with a strong association with TSC, mimics soft tissue fibroma but shows reactivity with melanocytic markers.<\/jats:p>","DOI":"10.1097\/pas.0000000000001024","type":"journal-article","created":{"date-parts":[[2018,1,11]],"date-time":"2018-01-11T23:37:32Z","timestamp":1515713852000},"page":"500-505","source":"Crossref","is-referenced-by-count":19,"title":["Fibroma-like PEComa"],"prefix":"10.1097","volume":"42","author":[{"given":"Ana B.","family":"Larque","sequence":"first","affiliation":[{"name":"Pathology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard L.","family":"Kradin","sequence":"additional","affiliation":[{"name":"Pathology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivan","family":"Chebib","sequence":"additional","affiliation":[{"name":"Pathology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. Petur","family":"Nielsen","sequence":"additional","affiliation":[{"name":"Pathology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin K.","family":"Selig","sequence":"additional","affiliation":[{"name":"Pathology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth A.","family":"Thiele","sequence":"additional","affiliation":[{"name":"Pediatric Epilepsy Program, Massachusetts General Hospital and Harvard Medical School, Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anat","family":"Stemmer-Rachamimov","sequence":"additional","affiliation":[{"name":"Pathology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam A.","family":"Bredella","sequence":"additional","affiliation":[{"name":"Radiology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pawel","family":"Kurzawa","sequence":"additional","affiliation":[{"name":"Department of Clinical Pathology, Poznan University of Medical Sciences, Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vikram","family":"Deshpande","sequence":"additional","affiliation":[{"name":"Pathology"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-20230922","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1080\/00313029400169561","article-title":"Clear cell (\u201csugar\u201d) tumor of the lung is a lesion strictly related to angiomyolipoma\u2014the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC)","volume":"26","author":"Bonetti","year":"1994","journal-title":"Pathology"},{"key":"R2-20230922","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1016\/0046-8177(91)90298-4","article-title":"Cellular heterogeneity in lymphangiomyomatosis of the lung","volume":"22","author":"Bonetti","year":"1991","journal-title":"Hum Pathol"},{"key":"R3-20230922","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1097\/00000478-200009000-00007","article-title":"Clear cell myomelanocytic tumor of the falciform ligament\/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults","volume":"24","author":"Folpe","year":"2000","journal-title":"Am J Surg Pathol"},{"key":"R4-20230922","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1097\/PAS.0b013e318161dc34","article-title":"Sclerosing PEComa: clinicopathologic analysis of a distinctive variant with a predilection for the retroperitoneum","volume":"32","author":"Hornick","year":"2008","journal-title":"Am J Surg Pathol"},{"key":"R5-20230922","doi-asserted-by":"crossref","first-page":"1345","DOI":"10.1056\/NEJMra055323","article-title":"The tuberous sclerosis complex","volume":"355","author":"Crino","year":"2006","journal-title":"N Engl J Med"},{"key":"R6-20230922","doi-asserted-by":"crossref","first-page":"2155","DOI":"10.1093\/hmg\/6.12.2155","article-title":"Molecular genetic and phenotypic analysis reveals differences between TSC1 and TSC2 associated familial and sporadic tuberous sclerosis","volume":"6","author":"Jones","year":"1997","journal-title":"Hum Mol Genet"},{"key":"R7-20230922","doi-asserted-by":"crossref","first-page":"16035","DOI":"10.1038\/nrdp.2016.35","article-title":"Tuberous sclerosis complex","volume":"2","author":"Henske","year":"2016","journal-title":"Nat Rev Dis Primer"},{"key":"R8-20230922","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1002\/path.2289","article-title":"Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid cell tumour (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma","volume":"214","author":"Pan","year":"2008","journal-title":"J Pathol"},{"key":"R9-20230922","doi-asserted-by":"crossref","first-page":"14676","DOI":"10.1073\/pnas.1512570112","article-title":"Anti-VEGF treatment improves neurological function and augments radiation response in NF2 schwannoma model","volume":"112","author":"Gao","year":"2015","journal-title":"Proc Natl Acad Sci U S A"},{"key":"R10-20230922","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1097\/PAS.0b013e31824a8a37","article-title":"Perivascular epithelioid cell tumors (PEComas) harboring TFE3 gene rearrangements lack the TSC2 alterations characteristic of conventional PEComas: further evidence for a biological distinction","volume":"36","author":"Malinowska","year":"2012","journal-title":"Am J Surg Pathol"},{"key":"R11-20230922","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1097\/00000478-199606000-00010","article-title":"Clear cell \u201csugar\u201d tumor of the pancreas. A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells","volume":"20","author":"Zamboni","year":"1996","journal-title":"Am J Surg Pathol"},{"key":"R12-20230922","doi-asserted-by":"crossref","first-page":"1295","DOI":"10.1097\/00000478-200009000-00015","article-title":"HMB-45\/melan-A and smooth muscle actin-positive clear-cell epithelioid tumor arising in the ligamentum teres hepatis: additional example of clear cell \u201csugar\u201d tumors","volume":"24","author":"Tanaka","year":"2000","journal-title":"Am J Surg Pathol"},{"key":"R13-20230922","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1172\/JCI84262","article-title":"Tuberous sclerosis complex inactivation disrupts melanogenesis via mTORC1 activation","volume":"127","author":"Cao","year":"2017","journal-title":"J Clin Invest"},{"key":"R14-20230922","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1097\/01.pas.0000213348.65014.0a","article-title":"Gardner fibroma: a clinicopathologic and immunohistochemical analysis of 45 patients with 57 fibromas","volume":"31","author":"Coffin","year":"2007","journal-title":"Am J Surg Pathol"},{"key":"R15-20230922","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/j.pediatrneurol.2013.08.001","article-title":"Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference","volume":"49","author":"Northrup","year":"2013","journal-title":"Pediatr Neurol"}],"container-title":["American Journal of Surgical Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.lww.com\/00000478-201804000-00009","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,22]],"date-time":"2023-09-22T22:38:47Z","timestamp":1695422327000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/00000478-201804000-00009"}},"subtitle":["A Tuberous Sclerosis Complex-related Lesion"],"short-title":[],"issued":{"date-parts":[[2018,4]]},"references-count":15,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2018]]}},"URL":"https:\/\/doi.org\/10.1097\/pas.0000000000001024","relation":{},"ISSN":["0147-5185"],"issn-type":[{"value":"0147-5185","type":"print"}],"subject":[],"published":{"date-parts":[[2018,4]]}}}