Objective: To describe a patient presenting with hemorrhagic shock attributable to bleeding pheochromocytomas and the sequential management strategy used for treating this patient.
Methods: We summarize the clinical presentation, diagnostic work-up, surgical management, and pathologic features of our patient and review the pertinent literature.
Results: A 38-year-old man with multiple endocrine neoplasia type 2A and bilateral pheochromocytomas presented initially with nearly fatal retroperitoneal and intraperitoneal hemorrhage rather than the characteristic hypertensive paroxysms. After lifesaving operative intervention and a 5-month period of rehabilitation and convalescence, the patient underwent bilateral retroperitoneoscopic adrenalectomy as definitive treatment. Thus, the abdomen that had been operated on multiple times because of hemorrhage was left undisturbed, and the patient had a successful recovery.
Conclusion: Near-fatal intraperitoneal hemorrhage is a very rare initial manifestation of pheochromocytoma. Our current patient with bilateral pheochromocytomas presented in this dramatic manner. This case shows that a sequential management strategy of damage-control surgical treatment followed by future resection of the tumors after appropriate a-adrenergic blockade is a safe and effective therapeutic option.