Molecular stability overrides size in giant pediatric pheochromocytoma.
Enver Simsek, Hüseyin İlhan, Murat Yilmaz et al.
✦ AI-curated · Sources linked
Case in brief
A 16-year-old female presented with severe hypertension and hyperglycemia, initially misdiagnosed with essential hypertension and type 2 diabetes. Imaging revealed a 12.4 cm adrenal mass, and biochemical tests indicated significant catecholamine excess. After open total adrenalectomy, the patient achieved biochemical normalization and favorable follow-up, despite the tumor's large size and multifocal lymphovascular invasion.
What made this case unusual
The case is notable for the preserved expression of succinate dehydrogenase subunit B (SDHB) and a low Ki-67 proliferation index, despite the tumor's large size and aggressive features.
Diagnostic / clinical pearl
Clinicians should consider pheochromocytoma in pediatric patients with persistent hypertension and atypical metabolic abnormalities, as traditional size metrics may not predict tumor behavior.
Why it matters
This case underscores the need for integrating molecular markers in the assessment of pheochromocytoma to enhance risk stratification and inform clinical management.