{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T02:23:55Z","timestamp":1790821435812,"version":"4.1.0"},"reference-count":43,"publisher":"Oxford University Press (OUP)","issue":"4","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2016,10]]},"abstract":"<jats:sec>\n<jats:title>Objective<\/jats:title>\n<jats:p>Pheochromocytoma (PHEO) may occur in 0.1\u20135.7% of patients presenting with a neurofibromatosis type 1 (NF1). Current recommendations are to explore only symptomatic patients. The objective of the study is to evaluate the prevalence and the interest of a systematic PHEO screening in this population.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Design<\/jats:title>\n<jats:p>A prospective study in a French tertiary center including consecutive NF1 patients older than 18\u2009years.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>A systematic screening combining abdominal imaging and urinary fractionated metanephrines was proposed. In case of positivity of one or both exams, <jats:sup>123<\/jats:sup>I-metaiodobenzylguanidine scintigraphy or [<jats:sup>18<\/jats:sup>F]-fluoro-dihydroxyphenylalanine PET imaging was performed. The diagnosis of secreting PHEO was retained in case of elevated urinary metanephrines associated with positive scintigraphy and non-secreting PHEO when urinary metanephrines were normal with a positive scintigraphy.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>Between January 2014 and August 2015, 234 patients were included and 156 patients (66.7%) completed both exams. In these 156 patients, 12 PHEOs were diagnosed, representing a prevalence of 7.7%. Of these, six PHEOs were secreting, with only two symptomatic patients. The tumor size of these PHEOs were bigger than that of non-secreting PHEO (25.2\u2009\u00b1\u20096.6 vs 14\u2009\u00b1\u20096.9\u2009mm, P\u2009=\u20090.0165). One lesion was bilateral. Mean metanephrine and normetanephrine levels were 3.2\u2009\u00b1\u20092.6N and 2.8\u2009\u00b1\u20091N respectively. Three patients underwent surgery. The six patients with non-secreting PHEO were asymptomatic. One of them had bilateral lesion and one underwent surgery.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>PHEO in NF1, whether or not secreting, are mostly asymptomatic. The current strategy to explore only symptomatic patients leads to an underestimation of prevalence with the risks inherent to the existence of an unrecognized PHEO.<\/jats:p>\n<\/jats:sec>","DOI":"10.1530\/eje-16-0233","type":"journal-article","created":{"date-parts":[[2016,7,23]],"date-time":"2016-07-23T01:58:15Z","timestamp":1469239095000},"page":"335-344","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":64,"title":["Interest of systematic screening of pheochromocytoma in patients with neurofibromatosis type 1"],"prefix":"10.1093","volume":"175","author":[{"given":"Lori","family":"K\u00e9p\u00e9n\u00e9kian","sequence":"first","affiliation":[{"name":"1Department of Endocrinology and DiabetologyHospices Civils de Lyon, Groupement Hospitalier Est, H\u00f4pital Louis Pradel, Bron Cedex, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Mognetti","sequence":"additional","affiliation":[{"name":"2Department of Nuclear MedicineL\u00e9on B\u00e9rard Comprehensive Cancer Center, Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Christophe","family":"Lifante","sequence":"additional","affiliation":[{"name":"3Department of GeneralDigestive and Endocrine Surgery, Centre Hospitalier Lyon Sud, Universit\u00e9 Claude Bernard Lyon 1, Pierre B\u00e9nite, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Laure","family":"Giraudet","sequence":"additional","affiliation":[{"name":"2Department of Nuclear MedicineL\u00e9on B\u00e9rard Comprehensive Cancer Center, Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Houzard","sequence":"additional","affiliation":[{"name":"4Nuclear Medicine UnitImaging Department, Centre Hospitalier Lyon Sud, Pierre-B\u00e9nite, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Pinson","sequence":"additional","affiliation":[{"name":"5Molecular Genetics LaboratoryH\u00f4pital Edouard Herriot, B\u00e2timent E, Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7oise","family":"Borson-Chazot","sequence":"additional","affiliation":[{"name":"1Department of Endocrinology and DiabetologyHospices Civils de Lyon, Groupement Hospitalier Est, H\u00f4pital Louis Pradel, Bron Cedex, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Combemale","sequence":"additional","affiliation":[{"name":"6Rh\u00f4ne-Alpes Auvergne Competence Center for the treatment of Neurofibromatosis type 1L\u00e9on B\u00e9rard Comprehensive Cancer Center, Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","reference":[{"key":"2016082219090521000_175.4.335.1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.141.1.71"},{"key":"2016082219090521000_175.4.335.2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/111.6.1355"},{"key":"2016082219090521000_175.4.335.3","first-page":"172","volume":"1","author":"National Institutes of Health Consensus Development Conference Statement","year":"1988","journal-title":"Neurofibromatosis"},{"key":"2016082219090521000_175.4.335.4","doi-asserted-by":"crossref","unstructured":"Pacak K Del Rivero J. 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