Spontaneous Rupture of Adrenal Pheochromocytoma: Review and Analysis of Prognostic Factors

Background And Objectives: Because of its rarity, the clinical characteristics of the manifestation of a ruptured pheochromocytoma and factors influencing on treatment outcomes in patients are still unclear.

Methods: A comprehensive MEDLINE review of the literature on pheochromocytomas, rupture and hemorrhage was performed. Based on the review and one additional case of our hospital, statistical analysis was undertaken to analyze the association of clinical outcome with clinical presentation, preoperative diagnosis, and treatment modality.

Results: We identified 49 other cases of hemorrhagic pheochromocytoma in the literature. After our case was added, a review of all 50 cases revealed that 17 of 38 that could not undergo adequately prepared (elective) surgery died, while all 12 patients who underwent elective surgery survived (P = 0.004). An accurate preoperative diagnosis of pheochromocytoma was associated with elective surgery (P < 0.0001), and hemodynamic instability (shock status) was a significant factor for a failed diagnosis and inadequate management, resulting in a poor prognosis (P = 0.023).

Conclusions: Although it is a very rare condition, physicians should be aware that a pheochromocytoma can bleed and present acutely in the abdomen with shock; an accurate diagnosis and adequately prepared surgical removal are important for a good postoperative prognosis.