Authors
»
Quan X, Wang Z, Zhang L, Chen C, Wang F -More
Category
»
Primary study
Journal»BMC pediatrics
Year
»
2026
BACKGROUND: Pheochromocytoma is a rare neuroendocrine tumor in children,
typically presenting with hypertension, headache, and palpitations. Presentation
with abdominal pain as the sole initial symptom, rapidly progressing to
catecholamine-induced cardiomyopathy, cardiogenic shock, and multiple organ
dysfunction syndrome (MODS), is exceedingly uncommon and poses significant
diagnostic and therapeutic challenges. We report a case of adolescent
pheochromocytoma multisystem crisis to highlight the diagnostic pitfalls,
pathophysiological cascade, and the dilemma of extracorporeal life support. CASE
PRESENTATION: A 13-year-8-month-old male presented with a 3-day history of
abdominal pain and poor responsiveness. Initial treatment for acute
gastroenteritis was ineffective, and his condition rapidly deteriorated into
cardiogenic shock and multiple organ failure, requiring mechanical ventilation
and veno-arterial extracorporeal membrane oxygenation (VA-ECMO) prior to transfer
to our hospital. The clinical course was characterized by sustained abdominal
pain as the initial manifestation, absence of typical palpitations or headache,
transient hypertension (175/109 mmHg) followed by circulatory collapse, and
physical findings of a sedated state, mottled skin with extremity ecchymoses,
muffled heart sounds, absent bowel sounds, and prolonged capillary refill time of
5 s. Bedside echocardiography revealed a left ventricular ejection fraction of
only 26%. Abdominal computed tomography and ultrasonography demonstrated a left
adrenal mass. Plasma normetanephrine was markedly elevated at > 20.56 nmol/L
(normal ≤ 0.9 nmol/L) and 3-methoxytyramine at 5.47 nmol/L (normal < 0.18
nmol/L), confirming the diagnosis of pheochromocytoma. VA-ECMO and mechanical
ventilation were maintained. Multiple sessions of plasma exchange and continuous
renal replacement therapy were instituted, alongside vasoactive agents. Surgical
resection was planned but never performed due to progressive multiorgan
deterioration. Despite 6 days of ECMO support and comprehensive therapy, the
patient developed hepatic encephalopathy and digital dry gangrene. The family
elected to withdraw care, and the patient died shortly after discharge.
CONCLUSIONS: This case illustrates that pheochromocytoma can present with
abdominal pain as the sole initial symptom in adolescents, mimicking acute
gastroenteritis. The rapid progression from catecholamine storm to irreversible
multiorgan failure underscores the critical importance of early recognition of
atypical presentations. While VA-ECMO may serve as a bridge to diagnosis and
stabilization, it cannot reverse established multiorgan injury when timely
surgical source control is not achieved.
Epistemonikos ID: da9d63ad18ca55cb36e4a563d01f827005512dc9
First added on: Sep 01, 2026