Pheochromocytomatosis Treated By Radio-Guided Surgery

ABSTRACT Objective: Pheochromocytomatosis is a rare cause of recurrent pheochromocytoma due to seeding of tumor cells at the time of adrenalectomy. The diagnosis can be difficult and a high degree of suspicion is required pre-operatively. We present the case of a 57-year-old woman with a long history of symptoms suggestive of recurrent pheochromocytoma that evaded diagnosis for many years. Methods: We describe the clinical course, diagnosis, and management. We also present a comprehensive review of the current literature. Results: Pheochromocytomatosis in our patient presented as multiple recurrent episodes of tachycardia and hypertension 15 years after left adrenalectomy for pheochromocytoma. Diagnosis was evaded until complete hormonal evaluation confirmed biochemical evidence of catecholamine overproduction. Imaging revealed two sites of recurrence. Management included alpha-blockade followed by radio-guided surgery using 123I-labeled metaiodobenzylguanidine scintigraphy and a gamma probe. At surgery, pheochromocytomatosis involved the spleen, omentum, and adrenal bed. A complete en bloc resection was performed, allowing normalization of the patient's blood pressure and heart rate postoperatively. Review of the literature identified 4 similar cases. Radio-guided surgery has been used in a few cases to localize pheochromocytoma, but has not been reported previously for intra-operative localization of pheochromocytomatosis. Conclusion: Our case highlights the importance of life-long follow-up and thorough endocrine evaluation for patients with recurrent symptoms after initial surgical treatment for pheochromocytoma. It also describes the utility of radio-guided surgery in the treatment of pheochromocytomatosis. Abbreviations: CPS = counts per second; CT = computed tomography; MIBG = 123I-labeled metaiodobenzylguanidine; SDH = succinate dehydrogenase