Pheochromocytoma presenting with arterial and intracardiac thrombus in a 47-year-old woman: a case report

Abstract

Introduction: Pheochromocytoma is a rare cause of hypertension but it could have severe consequences if not recognized and treated appropriately. The association of pheochromocytoma and thrombosis is even rarer but significantly increases management complexity, morbidity and mortality. To the best of our knowledge, this is the first report of a patient with pheochromocytoma presenting with left axillary arterial and intracardiac thrombus.

Case presentation: A 47-year-old Caucasian woman with a past medical history of hypertension presented for medical attention with left arm numbness. Doppler ultrasound showed an obstructing thrombus in her left axillary artery. She had symptom resolution after stent placement in her left axillary artery. A subsequent echocardiogram demonstrated a large intracardiac mass and abdominal computed tomography revealed a 7 cm mass between her spleen and left kidney. Labile blood pressure was noted during admission and she had very high levels of plasma and 24-hour urine catecholamines and metanephrines tests. A (123)I- metaiodobenzylguanidine scan showed intense uptake in the left abdominal mass. After adequate alpha blockage with phenoxybenzamine, laparoscopic tumor resection was performed without complications. She had normal metanephrines and complete symptom resolution afterwards. The intracardiac mass also disappeared with anticoagulation. All other endocrine laboratory abnormalities returned to normal after surgery.

Conclusion: Arterial and ventricular thrombosis occurring in patients with pheochromocytoma is rare. A multi-disciplinary approach is necessary in caring for this type of patient. Catecholamines likely contributed to the development of thrombosis in our patient. Early recognition of pheochromocytoma is the key to improving outcome.

Figure 1

¹²³I-MIBG scan showing intensive uptake in the adrenal mass but no uptake in the lung. A ¹²³I-MIBG scan was obtained to determine whether the left adrenal mass and right lung mass are pheochromocytoma. Shown are the frontal and back views of the total body scan at 72 hours. Significantly increased uptake is seen in the left adrenal lesion. No uptake was found in the lung. Physiological uptake is seen in the salivary glands, heart and liver. ¹²³I-MIBG is renally excreted and is visible in the bladder.

Figure 2

Histological views of the resected adrenal tumor and its intravascular invasion. A. High power (400 ×) view of the resected adrenal tumor. The resected adrenal pheochromocytoma shows chromaffin cells with a classic nested and trabecular architecture. Other characteristic morphologic features include nuclear enlargement and hyperchromasia with cytoplasm that is both oncocytic (pink and granular) in some cells and basophilic (blue) in others. B. Intra-vascular invasion of tumor (400 ×). Pheochromocytoma cells seen within a blood vessel. Vascular invasion is not a reliable indication of a malignant pheochromocytoma. Only metastatic disease to regional lymph nodes or distant sites (most commonly ribs, spine, liver and lung) will define this tumor as a malignant lesion.