Pheochromocytoma crisis complicated by disseminated intravascular coagulation and tumor rupture: A case report

Pheochromocytoma may rarely present with pheochromocytoma multisystem crisis (PMC), and disseminated intravascular coagulation (DIC) is an uncommon manifestation. Their coexistence with spontaneous tumor rupture is particularly unusual and creates major diagnostic and therapeutic difficulty. We report a 27-year-old woman with spontaneously ruptured pheochromocytoma complicated by PMC and DIC. She initially presented with hyperglycemia, high-anion-gap metabolic acidosis, and hyperlactatemia, raising concern for diabetic ketoacidosis. Her condition then evolved rapidly, with marked blood pressure lability, pulmonary edema, shock, acute kidney and liver injury, respiratory failure, and pheochromocytoma-associated stress cardiomyopathy. Imaging showed a hemorrhagic adrenal mass with retroperitoneal hematoma. Laboratory testing demonstrated thrombocytopenia, hypofibrinogenemia, prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT), markedly elevated D-dimer, persistent abnormalities in thrombin-antithrombin III complex (TAT), plasmin–α2-plasmin inhibitor complex (PIC), tissue plasminogen activator–plasminogen activator inhibitor-1 complex (t-PAIC), and thrombomodulin (TM), and severe mixed hypocoagulability on thromboelastography. Plasma metanephrine, normetanephrine, and 3-methoxytyramine were markedly elevated, supporting the diagnosis of pheochromocytoma crisis. Because the immediate priority at presentation was control of active hemorrhage, transarterial embolization (TAE) was performed before pheochromocytoma had been fully recognized. Conventional preoperative optimization was not feasible because even minimal α-blockade worsened hemodynamics, and aggressive fluid loading risked aggravating pulmonary edema. The patient was managed with staged treatment including TAE, organ support, transfusion and coagulation-directed therapy, and delayed adrenalectomy after stabilization. She survived and subsequently underwent definitive surgery. This case shows that DIC, although rare in pheochromocytoma, may occur in the setting of tumor rupture and PMC. In unstable patients in whom immediate adrenalectomy and standard preoperative preparation are not possible, staged management with early TAE and delayed surgery may be a useful approach.