1.Mimicking Pheochromocytoma: Hypertensive crisis from adrenal hematoma in JAK2-positive polycythemia rubra vera
Dhanya Ganesan ; Hwee Ching Tee ; Jin Hui Ho ; Shireen Siow Leng Lui
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):20-21
Introduction:
Hemorrhagic suprarenal masses presenting with hypertensive emergency pose a significant diagnostic challenge,
particularly when biochemical and radiological findings
are inconclusive. The clinical presentation may mimic
catecholamine-secreting tumors, necessitating consideration of a broad differential diagnosis, including pheochromocytoma, adrenocortical carcinoma, retroperitoneal
hemorrhage, and hematological-related extramedullary
lesions. Accurate diagnosis is essential, as management
strategies differ significantly.
Case:
We report a 48-year-old male smoker with no known prior
medical illness who presented with sudden left-sided chest
pain radiating to the epigastrium, associated with vomiting.
On arrival, he was markedly hypertensive (207/131 mmHg).
He reported a 1-year history of paroxysmal palpitations,
headaches, migraines, and intermittent diaphoresis.
Computed tomography angiography excluded aortic
dissection but demonstrated a left retroperitoneal
hemorrhage with non-visualization of the adrenal gland,
suggestive of adrenal or tumor-related hemorrhage.
He was initially managed empirically as a
pheochromocytoma while undergoing biochemical
evaluation; however, urinary metanephrines were only
mildly elevated. Repeat imaging demonstrated interval
enlargement of a non-enhancing suprarenal mass, raising
concern for tumor-related hemorrhage. Subsequent
ultrasonography, however, favored a liquefied hematoma,
and percutaneous drainage yielded 750 mL of sanguineous
fluid, resulting in marked clinical improvement.
Notably, an elevated hematocrit prompted further
evaluation for erythrocytosis. Subsequent testing
confirmed JAK2 mutation-positive polycythemia rubra
vera, providing a unifying explanation for both the
erythrocytosis and spontaneous adrenal hemorrhage. The
patient was commenced on hydroxyurea and referred for
hematology follow-up.
Conclusion
Adrenal hemorrhage may closely mimic pheochromocytoma in hypertensive emergencies. A systematic, multidisciplinary approach integrating clinical, biochemical, and
imaging findings is essential to avoid misdiagnosis and
guide appropriate management, particularly in patients
with underlying hematological disorders.
Hypertensive Crisis
;
Pheochromocytoma
;
Polycythemia Vera
;
Hematoma


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