{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T17:19:07Z","timestamp":1787505547153,"version":"build-2736575974"},"reference-count":12,"publisher":"BMJ","issue":"12","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2021,11,2]]},"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:p>Pheochromocytomas are rare in children. The diagnosis is usually established from a raised urinary or plasma catecholamine or their metabolites. We present a girl aged 11 years who manifested with a hypertensive crisis secondary to an adrenal tumour but with unexpectedly normal urinary metanephrine and catecholamine results. She improved spontaneously following the crisis and underwent surgery later. The histopathological study confirmed a pheochromocytoma with large central necrosis. Her genetic screening reported a pathogenic von Hippel-Lindau gene mutation. Surveillance scan postsurgery detected no other tumours. Following the catecholamine crisis, an acute infarct occurred, resulting in extensive tumour necrosis and subsequent rapid remission of symptoms and paradoxically normal biochemical markers. Although not unheard of in adults, we believe this is the first reported case of an extensive spontaneous necrosis resulting in a biochemically normal pheochromocytoma in a child.<\/jats:p>","DOI":"10.1136\/bcr-2021-245154","type":"journal-article","created":{"date-parts":[[2021,12,22]],"date-time":"2021-12-22T12:00:31Z","timestamp":1640174431000},"page":"e245154","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":7,"title":["Biochemically normal adrenal pheochromocytoma following extensive central necrosis in a child with von Hippel-Lindau (VHL) gene mutation"],"prefix":"10.1136","volume":"14","author":[{"given":"Benjamin Wei-Liang","family":"Ng","sequence":"first","affiliation":[{"name":"Faculty of Medicine, SEGi University, Kota Damansara, Malaysia"},{"name":"Department of Paediatrics, Sibu Hospital, Ministry of Health Malaysia, Sibu, Sarawak, Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeanne Sze-Lyn","family":"Wong","sequence":"additional","affiliation":[{"name":"Department of Paediatric Endocrinology, Putrajaya Hospital, Ministry of Health Malaysia, Putrajaya, Wilayah Persekutuan, Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5909-4855","authenticated-orcid":false,"given":"Teck-Hock","family":"Toh","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, SEGi University, Kota Damansara, Malaysia"},{"name":"Department of Paediatrics, Sibu Hospital, Ministry of Health Malaysia, Sibu, Sarawak, Malaysia"},{"name":"Clinical Research Centre, Sibu Hospital, Ministry of Health Malaysia, Sibu, Sarawak, Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2021,12,22]]},"reference":[{"key":"2025082707295850000_14.12.e245154.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.currproblcancer.2014.01.001"},{"key":"2025082707295850000_14.12.e245154.2","doi-asserted-by":"crossref","first-page":"179","DOI":"10.4274\/jcrpe.5078","article-title":"Two childhood pheochromocytoma cases due to von Hippel-Lindau disease, one associated with pancreatic neuroendocrine tumor: a very rare manifestation","volume":"10","author":"Da\u011fdeviren \u00c7ak\u0131r","year":"2018","journal-title":"J Clin Res Pediatr Endocrinol"},{"key":"2025082707295850000_14.12.e245154.3","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1297\/cpe.27.87","article-title":"A pediatric case of pheochromocytoma without apparent hypertension associated with von Hippel-Lindau disease","volume":"27","author":"Igaki","year":"2018","journal-title":"Clin Pediatr Endocrinol"},{"key":"2025082707295850000_14.12.e245154.4","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1016\/S0140-6736(05)67139-5","article-title":"Phaeochromocytoma","volume":"366","author":"Lenders","year":"2005","journal-title":"The Lancet"},{"key":"2025082707295850000_14.12.e245154.5","doi-asserted-by":"crossref","first-page":"470","DOI":"10.4103\/ijem.IJEM_558_16","article-title":"Characteristics of pediatric pheochromocytoma\/paraganglioma","volume":"21","author":"Sarathi","year":"2017","journal-title":"Indian J Endocrinol Metab"},{"key":"2025082707295850000_14.12.e245154.6","first-page":"86","article-title":"Pheochromocytoma: an uncommon presentation of an asymptomatic and biochemically silent adrenal incidentaloma","volume":"19","author":"Kota","year":"2012","journal-title":"Malays J Med Sci"},{"key":"2025082707295850000_14.12.e245154.7","doi-asserted-by":"crossref","DOI":"10.1186\/s13256-016-1068-3","article-title":"Histopathological analysis of spontaneous large necrosis of adrenal pheochromocytoma manifested as acute attacks of alternating hypertension and hypotension: a case report","volume":"10","author":"Ohara","year":"2016","journal-title":"J Med Case Rep"},{"key":"2025082707295850000_14.12.e245154.8","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-14-0916"},{"key":"2025082707295850000_14.12.e245154.9","doi-asserted-by":"publisher","DOI":"10.1586\/eem.12.76"},{"key":"2025082707295850000_14.12.e245154.10","doi-asserted-by":"crossref","first-page":"212","DOI":"10.5507\/bp.2019.047","article-title":"Characteristic CT features of pheochromocytomas - probability model calculation tool based on a multicentric study","volume":"163","author":"Ctvrtlik","year":"2019","journal-title":"Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub"},{"key":"2025082707295850000_14.12.e245154.11","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2014-1498"},{"key":"2025082707295850000_14.12.e245154.12","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2017.00155","article-title":"Review of pediatric pheochromocytoma and paraganglioma","volume":"5","author":"Bholah","year":"2017","journal-title":"Front Pediatr"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2021-245154","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T14:30:22Z","timestamp":1756305022000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2021-245154"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12]]},"references-count":12,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2021,12,22]]},"published-print":{"date-parts":[[2021,12]]}},"alternative-id":["10.1136\/bcr-2021-245154"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2021-245154","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,12]]}}}