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Massive Biochemically Silent Pheochromocytoma Masquerading as Nonfunctioning Adrenocortical Cancer
Shejil Kumar1, Katherine Wu1, Natassia Rodrigo1,2
1Department of Diabetes, Endocrinology & Metabolism, Royal North Shore Hospital, Sydney, NSW 2065, Australia.
JCEM Case Reports
|November 1, 2023
Summary
Rare, massive pheochromocytomas can be clinically silent, presenting without typical symptoms or elevated catecholamines. This case highlights a large adrenal mass initially presumed non-cancerous due to lack of biochemical evidence.
Area of Science:
- Endocrinology
- Oncology
- Pathology
Background:
- Pheochromocytomas, neuroendocrine tumors of adrenal chromaffin cells, typically cause catecholamine excess symptoms.
- Clinically and biochemically silent pheochromocytomas are exceptionally rare, posing diagnostic challenges.
Observation:
- A 71-year-old female presented with flank pain and a hemorrhagic adrenal mass, initially managed conservatively.
- Despite normal catecholamine levels and no adrenergic symptoms, advanced imaging revealed intense fluorodeoxyglucose F 18-positron emission tomography (FDG-PET)/CT avidity.
- Histopathology of the resected adrenal mass confirmed a large pheochromocytoma, initially suspected to be adrenocortical cancer.
Findings:
- The pheochromocytoma was biochemically silent, lacking elevated plasma normetanephrines and metanephrines.
- Immunohistochemistry suggested a possible FH-deficient pheochromocytoma, though germline sequencing was negative for common mutations.
- Post-operative imaging showed no recurrence or metastasis.
Implications:
- This case underscores the rarity of massive, biochemically silent pheochromocytomas.
- It emphasizes the importance of considering pheochromocytoma in adrenal masses with high FDG-PET/CT avidity, even without biochemical confirmation.
- Further research into the mechanisms of biochemically silent pheochromocytomas is warranted.
Keywords:
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