Article Text

Download PDFPDF
Biochemically normal adrenal pheochromocytoma following extensive central necrosis in a child with von Hippel-Lindau (VHL) gene mutation
  1. Benjamin Wei-Liang Ng1,2,
  2. Jeanne Sze-Lyn Wong3 and
  3. Teck-Hock Toh1,2,4
  1. 1Faculty of Medicine, SEGi University, Kota Damansara, Malaysia
  2. 2Department of Paediatrics, Sibu Hospital, Ministry of Health Malaysia, Sibu, Sarawak, Malaysia
  3. 3Department of Paediatric Endocrinology, Putrajaya Hospital, Ministry of Health Malaysia, Putrajaya, Wilayah Persekutuan, Malaysia
  4. 4Clinical Research Centre, Sibu Hospital, Ministry of Health Malaysia, Sibu, Sarawak, Malaysia
  1. Correspondence to Dr Benjamin Wei-Liang Ng; benng85{at}


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.

  • adrenal disorders
  • radiology

Statistics from


  • Contributors BW-LN: conceptualised, designed and drafted the initial draft of the manuscript and approved the final manuscript. T-HT and JS-LW conceptualised and designed the manuscript, critically revised the manuscript as submitted and also approved the final manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.