{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T13:39:40Z","timestamp":1790429980858,"version":"4.1.0"},"reference-count":36,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T00:00:00Z","timestamp":1751328000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Sporadic renal cell carcinomas with fibromyomatous stroma (RCC FMS) with coexistent haemangioblastoma (HB)\u2010like morphology have been previously reported. Such morphology has not, however, been documented in patients with tuberous sclerosis complex (TSC). We evaluated clinicopathologic, immunohistochemical and molecular findings of RCC FMS with HB\u2010like features in three TSC patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>\n                      All three patients were females with confirmed germline mutations in\n                      <jats:italic>TSC1<\/jats:italic>\n                      in the absence of\n                      <jats:italic>VHL<\/jats:italic>\n                      or other alterations. One patient had bilateral nephrectomy with six separate tumours (three in each kidney), and two patients had one tumour each, resected by partial nephrectomy. Prominent fibromuscular stroma was present at the tumoral periphery and surrounding clear cell nests forming tubules and papillary formations (RCC FMS areas), admixed with solid, clear to eosinophilic and spindle cell areas (HB\u2010like areas). HB\u2010like areas were variably represented in individual tumours, but in some, HB\u2010like features were almost exclusive. HB\u2010like areas were negative for CK7 and showed inhibin\u2010\u03b1 and S100 reactivity, in contrast to typical RCC FMS areas, which were CK7 positive, while negative for inhibin\u2010\u03b1 and S100. GPNMB, which is consistently expressed in\n                      <jats:italic>TSC\/mTOR<\/jats:italic>\n                      altered tumours, was positive in both components. On follow\u2010up (10 months\u201321\u2009years), all patients had an indolent course.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      HB\u2010like morphology found in RCC FMS in TSC patients represents part of the morphologic spectrum of RCC FMS, which is a previously underreported and underrecognized feature. RCC FMS with HB\u2010like areas show uniform GPNMB reactivity and are associated with\n                      <jats:italic>TSC\/MTOR<\/jats:italic>\n                      alterations, but not with\n                      <jats:italic>VHL<\/jats:italic>\n                      alterations.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/his.15505","type":"journal-article","created":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T06:03:25Z","timestamp":1751349805000},"page":"687-699","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Renal cell carcinoma with fibromyomatous stroma (\n                    <scp>RCC FMS<\/scp>\n                    ) and with hemangioblastoma\u2010like areas is part of the\n                    <scp>RCC FMS<\/scp>\n                    spectrum in patients with tuberous sclerosis complex"],"prefix":"10.1111","volume":"87","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4740-9789","authenticated-orcid":false,"given":"Katherina","family":"Baranova","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine London Health Sciences Centre  London ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7505-0681","authenticated-orcid":false,"given":"Jacob A","family":"Houpt","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine London Health Sciences Centre  London ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deaglan","family":"Arnold","sequence":"additional","affiliation":[{"name":"Schulich School of Medicine and Dentistry, London Health Sciences Centre  London ON 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Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1937-3101","authenticated-orcid":false,"given":"Rola","family":"Saleeb","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose A","family":"Gomez","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine London Health Sciences Centre  London ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Asli","family":"Yilmaz","sequence":"additional","affiliation":[{"name":"Diagnostic and Molecular Pathology Cumming School of Medicine, University of Calgary  Calgary AB 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