{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T13:39:34Z","timestamp":1790429974842,"version":"4.1.0"},"reference-count":39,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2007,12,11]],"date-time":"2007-12-11T00:00:00Z","timestamp":1197331200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Veterans General Hospitals University System of Taiwan Joint Research Program, Tsou's Foundation","award":["VGHUST95-P7-35"],"award-info":[{"award-number":["VGHUST95-P7-35"]}]}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2008,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Perivascular epithelioid cell tumours (PEComas) are a family of tumours including classic angiomyolipoma, lymphangioleiomyomatosis, and clear epithelioid cell tumours reported under a variety of names such as epithelioid angiomyolipoma, pulmonary and extrapulmonary clear cell sugar tumour, and PEComa. Our previous comparative genomic hybridization study of PEComas demonstrated recurrent chromosomal aberrations including deletions on chromosome 16p, where the\n                    <jats:italic>TSC2<\/jats:italic>\n                    gene is located. In this study, we focused on the alteration of chromosome 16p, including\n                    <jats:italic>TSC2<\/jats:italic>\n                    . We collected ten sporadic and two tuberous sclerosis complex\u2010associated PEComas, as well as 14 sporadic classic hepatic and renal angiomyolipomas (AMLs) as controls. We used 16 microsatellite markers distributed along chromosome 16p to test for allelic imbalances on chromosome 16p and at\n                    <jats:italic>TSC2<\/jats:italic>\n                    , and two markers for\n                    <jats:italic>TSC1<\/jats:italic>\n                    . Furthermore, we carried out immunohistochemical staining for phospho\u2010p706K, phospho\u2010AKT, and phospho\u2010S6 to evaluate the effect of\n                    <jats:italic>TSC2<\/jats:italic>\n                    alterations on the mTOR signalling pathway. Loss of heterozygosity (LOH) was found in 11 PEComas and involved the region of the\n                    <jats:italic>TSC2<\/jats:italic>\n                    locus in seven. Six classic angiomyolipomas had allelic changes at chromosome 16p. Microsatellite instability was detected in two PEComas. The incidence of genetic aberrations was significantly higher in the PEComa group. Only one PEComa showed LOH at the\n                    <jats:italic>TSC1<\/jats:italic>\n                    locus. Eleven PEComas and 13 AMLs revealed elevated phospho\u2010p70S6K accompanied by reduced phospho\u2010AKT. Five PEComas and eight classic angiomyolipomas were positive for phospho\u2010S6. The phosphorylation profile indicates functional activation of the mTOR pathway through a disrupted TSC1\/2 complex. Our observations of frequent deletion of\n                    <jats:italic>TSC2<\/jats:italic>\n                    and the mTOR signalling pathway provide evidence that the oncogenetic lineage of PEComa, as a distinct\n                    <jats:italic>TSC2<\/jats:italic>\n                    \u2010linked neoplasm, is similar to that of angiomyolipoma. Copyright \u00a9 2007 Pathological Society of Great Britain and Ireland. Published by John Wiley &amp; Sons, Ltd.\n                  <\/jats:p>","DOI":"10.1002\/path.2289","type":"journal-article","created":{"date-parts":[[2007,12,17]],"date-time":"2007-12-17T09:47:46Z","timestamp":1197884866000},"page":"387-393","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":202,"title":["Constant allelic alteration on chromosome 16p (\n                    <i>TSC2<\/i>\n                    gene) in perivascular epithelioid cell tumour (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma"],"prefix":"10.1002","volume":"214","author":[{"given":"C\u2010C","family":"Pan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u2010Y","family":"Chung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K\u2010F","family":"Ng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u2010Y","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u2010S","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u2010Y","family":"Chai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u2010H","family":"Huang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"PC\u2010H","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"DMT","family":"Ho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,1,10]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199203000-00013"},{"key":"e_1_2_6_3_2","first-page":"221","volume-title":"World Health Organization Classification of Tumours. 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