{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T17:50:11Z","timestamp":1791481811273,"version":"4.3.4"},"reference-count":19,"publisher":"Walter de Gruyter GmbH","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,9,25]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec id=\"j_jpem-2026-0223_abs_001\">\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Pheochromocytoma is a rare but potentially life-threatening tumor in children, and its diagnosis may be challenging due to variable clinical presentations. We aimed to describe the diagnostic process and clinical outcomes of a child with a giant pheochromocytoma presenting with severe hypertension and metabolic abnormalities.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"j_jpem-2026-0223_abs_002\">\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>\n                      A 16-year-old female with a three-year history of hypertension and hyperglycemia, previously diagnosed with essential hypertension and type 2 diabetes mellitus, was found to have a 12.4\u202fcm adrenal mass weighing 280\u202fg. Congenital total hemihypertrophy provided an additional clinical clue. Biochemical evaluation demonstrated marked catecholamine excess, impaired glucose regulation (HbA\n                      <jats:sub>1c<\/jats:sub>\n                      : 6.4\u202f%), low HOMA-IR (2.39), and markedly elevated plasma renin activity (60.8\u202fng\/mL\/h), consistent with secondary hyperaldosteronism. Following open total adrenalectomy, biochemical normalization and favorable radiological follow-up were achieved. Despite the large tumor size and multifocal lymphovascular invasion, preserved succinate dehydrogenase subunit B (SDNB) expression and a low Ki-67 proliferation index (2\u20133\u202f%) were observed.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"j_jpem-2026-0223_abs_003\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This case highlights the importance of considering pheochromocytoma in children with persistent hypertension and atypical metabolic findings. Tumor size and individual histological features may not fully reflect biological behavior; integrated assessment incorporating molecular markers may improve risk stratification and clinical management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1515\/jpem-2026-0223","type":"journal-article","created":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T04:56:15Z","timestamp":1786596975000},"page":"951-959","source":"Crossref","is-referenced-by-count":0,"title":["Molecular stability overrides size in giant pediatric pheochromocytoma"],"prefix":"10.1515","volume":"39","author":[{"given":"Enver","family":"Simsek","sequence":"first","affiliation":[{"name":"Eskisehir Osmangazi Universitesi , Pediatric Endocrinology , Eskisehir , T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00fcseyin","family":"\u0130lhan","sequence":"additional","affiliation":[{"name":"Eski\u015fehir Osmangazi University School of Medicine , Pediatric Surgery , Eskisehir , T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Murat","family":"Yilmaz","sequence":"additional","affiliation":[{"name":"Eskisehir Osmangazi University School of Medicine , Pediatrics and Division of Pediatric Endocrinology , Eski\u015fehir , T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cigdem","family":"Oztunali","sequence":"additional","affiliation":[{"name":"Eskisehir Osmangazi University School of Medicine , Radiology , Eskisehir , T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emel","family":"Yaldir","sequence":"additional","affiliation":[{"name":"Eskisehir Osmangazi University School of Medicine , Pathology , Eskisehir , T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tulay","family":"Simsek","sequence":"additional","affiliation":[{"name":"Eskisehir Osmangazi University School of Medicine , Ophthalmology , Eskisehir , T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","published-online":{"date-parts":[[2026,8,14]]},"reference":[{"key":"2026100816572543091_j_jpem-2026-0223_ref_001","doi-asserted-by":"crossref","unstructured":"Lenders, JW, Eisenhofer, G, Mannelli, M, Pacak, K. 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