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Childhood pheochromocytoma crisis complicated with brain stem infarction: A case report
Fujing Xie1, Qingbing Zhao2, Wenwen Pan2
1Department of Pediatrics, Liaocheng People's Hospital, Liaocheng, Shandong Province, People's Republic of China.
Insights
Pediatric pheochromocytoma crises are rare but can be fatal. This case highlights a child’s death from an occult pheochromocytoma crisis complicated by cerebral infarction, emphasizing the need for early diagnosis.
Area of Science:
- Pediatric Endocrinology
- Neurology
- Oncology
Background:
- Pheochromocytoma crises are exceptionally rare in pediatric populations.
- This report details a fatal case involving an occult pheochromocytoma crisis and subsequent cerebral infarction in a child.
Observation:
- A 5-year-old boy presented with polydipsia, polyuria, sweating, weight loss, anorexia, vomiting, convulsions, and confusion.
- Brain MRI revealed abnormal signals in the left parie-occipital lobe and brainstem, leading to a provisional diagnosis of pheochromocytoma crisis with brainstem infarction.
Findings:
- Despite interventions including intubation and dobutamine infusion, the patient died.
- Autopsy confirmed pheochromocytoma, acute myocarditis, medulla oblongata necrosis, and cerebral edema.
- Genetic analysis revealed a synonymous mutation in the von Hippel-Lindau syndrome gene.
Implications:
- Early clinical presentation of pheochromocytoma in children is often atypical.
- Untreated pheochromocytoma can lead to severe complications, including crisis and death.
- Prompt recognition and management are crucial for improving outcomes in pediatric pheochromocytoma cases.
Rationale:
Pheochromocytoma crises are very rarely seen in children. In this report, we present a case of the death related to occult pheochromocytoma crisis combined cerebral infarction.
Patient Concerns:
A 5-year-old boy has a 1-month history of polydipsia, polyuria, sweating, and weight loss of 2.5 kg. He was admitted to our hospital because of 1 week of anorexia, 2 days of vomiting, and 12 hours of convulsions and confusion. Magnetic resonance imaging of the brain and cervical spinal cord showed abnormal signals in the left parie-occipital lobe, medulla oblongata till C7 cervical vertebrae.
Diagnoses:
Based on patient's complaints and clinical appearance, provisional diagnosis of pheochromocytoma crisis complicated brainstem infarction was considered.
Interventions:
Tracheal intubation, volume expansion, continuous infusion of dobutamine, and sedation reduce intracranial pressure. Chest compression was performed when the child suddenly developed sobbing respiration.
Outcomes:
The patient was dead. Congenital metabolic defects screening suggested mild ketonuria. Trio whole exon sequencing revealed a synonymous mutation of von Hippel-Lindau syndrome c.414 A > G in the decedent. Autopsy revealed pheochromocytoma, acute myocarditis, liquefaction necrosis of the medulla oblongata cerebral edema, and congestion.
Lessons:
Early clinical symptoms of pheochromocytoma in children are not typical. It may induce serious complications and develop into a pheochromocytoma crisis and cause death without proper treatment.
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