Mimicking Pheochromocytoma: Hypertensive crisis from adrenal hematoma in JAK2-positive polycythemia rubra vera
https://doi.org/10.15605/jafes.041.S1
- Author:
Dhanya Ganesan
1
;
Hwee Ching Tee
1
;
Jin Hui Ho
1
;
Shireen Siow Leng Lui
1
Author Information
1. Diabetes and Endocrine Unit, Department of Medicine, Hospital Queen Elizabeth II
- Publication Type:Journal Article
- MeSH:
Hypertensive Crisis;
Pheochromocytoma;
Polycythemia Vera;
Hematoma
- From:
Journal of the ASEAN Federation of Endocrine Societies
2026;41(S1):20-21
- CountryPhilippines
- Language:English
-
Abstract:
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.
- Full text:2026072813081992348EP_A005.pdf
