{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T03:58:26Z","timestamp":1785383906474,"version":"3.55.0"},"reference-count":8,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T00:00:00Z","timestamp":1784073600000},"content-version":"vor","delay-in-days":14,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology Case Reports"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Benign metastasizing leiomyoma (BML) is a rare condition characterized by histologically benign smooth muscle tumours arising at extrauterine sites, most commonly the lungs, in women with a history of uterine leiomyomata. Erythropoietin (EPO)\u2010secreting uterine leiomyomata causing secondary erythrocytosis\u2014termed myomatous erythrocytosis syndrome (MES)\u2014is an exceedingly rare and underrecognized phenomenon first described by Thomson and Marson in 1953, with fewer than 70 cases reported to date. We report a 39\u2010year\u2010old woman who presented with incidentally discovered bilateral pulmonary nodules on imaging performed during workup for MES with markedly elevated serum EPO (26.7\u2009mIU\/mL). She had a longstanding history of uterine leiomyomata and endometriosis. CT\u2010guided biopsy of the dominant left lower lobe nodule (14\u2009\u00d7\u200911\u2009mm) confirmed BML: lesional cells were positive for oestrogen receptor (ER), progesterone receptor (PR), smooth muscle actin (SMA) and desmin, with retained fumarate hydratase (FH), excluding hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome. Total hysterectomy with bilateral salpingo\u2010oophorectomy (BSO) was performed; pathology confirmed multiple cellular leiomyomata without features of STUMP or leiomyosarcoma on University of Michigan expert consultation. Within 3\u2009months of surgery, haemoglobin normalized to 14.2\u2009g\/dL and EPO to 6.7\u2009mIU\/mL, confirming the uterus as the source of ectopic EPO production. This case illustrates a novel unified hormonal syndrome in which uterine leiomyomata simultaneously drove pulmonary BML through ER\/PR signalling and caused secondary polycythemia through ectopic EPO secretion. Hysterectomy\/BSO resolved both processes concurrently, and this case highlights BML and MES as diagnoses to consider in premenopausal women with bilateral pulmonary nodules and unexplained erythrocytosis.<\/jats:p>","DOI":"10.1002\/rcr2.70687","type":"journal-article","created":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T01:56:23Z","timestamp":1784166983000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Benign Metastasizing Leiomyoma With Myomatous Erythrocytosis Syndrome: First Reported Coexistence as a Unified Hormonal Syndrome"],"prefix":"10.1002","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8322-4824","authenticated-orcid":false,"given":"Venkatkiran","family":"Kanchustambham","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Sanford Health  Fargo North Dakota USA"},{"name":"Department of Internal Medicine University of North Dakota School of Medicine and Health Sciences  Grand Forks North Dakota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley","family":"McWalter","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Sanford Health  Fargo North Dakota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oluwatobi E.","family":"Odetola","sequence":"additional","affiliation":[{"name":"Division of Hematology Sanford Health  Fargo North Dakota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Recep","family":"Nigdelioglu","sequence":"additional","affiliation":[{"name":"Department of Pathology Sanford Health  Fargo North Dakota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,7,15]]},"reference":[{"issue":"1","key":"e_1_2_10_2_1","first-page":"89","article-title":"Metastasizing Fibroleiomyoma of the Uterus: Report of a Case and Review of the Literature","volume":"15","author":"Steiner P. E.","year":"1939","journal-title":"American Journal of Pathology"},{"issue":"7","key":"e_1_2_10_3_1","first-page":"827","article-title":"Benign Metastasizing Leiomyoma: A Cytogenetically Balanced but Clonal Disease","volume":"13","author":"Tietze L.","year":"2000","journal-title":"Modern Pathology"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.3800504"},{"issue":"3","key":"e_1_2_10_5_1","first-page":"143","article-title":"Benign Metastasizing Leiomyoma\u2014A Review","volume":"19","author":"Barnas E.","year":"2020","journal-title":"Przeglad Menopauzalny"},{"issue":"3","key":"e_1_2_10_6_1","first-page":"72","article-title":"Erythropoietin\u2010Producing Uterine Leiomyoma: A Case Report and Literature Review","volume":"2","author":"Matsuo K.","year":"2012","journal-title":"Gynecologic Oncology Reports"},{"issue":"2","key":"e_1_2_10_7_1","first-page":"387","article-title":"Erythropoietin (EPO)\u2010Producing Uterine Myoma: Immunohistochemical Evidence","volume":"82","author":"Nakajima T.","year":"2001","journal-title":"Gynecologic Oncology"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.fertnstert.2013.01.147"},{"issue":"6","key":"e_1_2_10_9_1","first-page":"744","article-title":"Erythropoietin\u2010Producing Uterine Leiomyoma Harboring a Heterozygous Von Hippel\u2013Lindau Gene Mutation","volume":"31","author":"Mikami Y.","year":"2000","journal-title":"Human Pathology"}],"container-title":["Respirology Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/rcr2.70687","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/rcr2.70687","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/rcr2.70687","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T03:04:57Z","timestamp":1785380697000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/rcr2.70687"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":8,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7]]}},"alternative-id":["10.1002\/rcr2.70687"],"URL":"https:\/\/doi.org\/10.1002\/rcr2.70687","archive":["Portico"],"relation":{},"ISSN":["2051-3380","2051-3380"],"issn-type":[{"value":"2051-3380","type":"print"},{"value":"2051-3380","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]},"assertion":[{"value":"2026-02-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-06","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-15","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70687"}}