Biochemically Negative Pheochromocytoma With Spontaneous Hemorrhage

Pheochromocytomas are neuroendocrine tumors that arise from adrenomedullary chromaffin cells that produce excess catecholamines. With advancements in imaging modalities, increasing numbers of incidental adrenal masses are discovered with pheochromocytomas making up approximately 4.2% of incidental adrenal masses. The objective of this report is to describe a patient with an unusual presentation of pheochromocytoma that was asymptomatic and lacked classic laboratory findings.

We present the case of a 31-year-old female who presented to the emergency department with abdominal pain and was found to have an adrenal mass on computed tomography scan. Biopsy of the mass was attempted multiple times prior to biochemical evaluation. The mass was later discovered to be a biochemically negative pheochromocytoma with spontaneous hemorrhage on histological examination following adrenal resection.

Pheochromocytomas are the most commonly reported adrenal mass found to have spontaneous hemorrhage. Between 9% and 32% of pheochromocytomas may be biochemically silent adding to the complexity of making an accurate diagnosis and calls into questions current perioperative management of all adrenal masses. In addition, this case serves as a reminder to complete biochemical evaluation prior to invasive adrenal procedures.

This case offers insight into challenges with diagnosis and management of pheochromocytoma with spontaneous hemorrhage, and encourages caution when evaluating adrenal masses due to risk of pheochromocytoma even when biochemically negative.