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 123 I-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. Radioguided 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 lifelong 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. (AACE Clinical Case Rep. 2017;3:e170-e175

The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma

“…The other case described pheochromocytosis seen at exploration, and the probe allowed them to detect small lesions that may have been missed without it. 115 The largest and most recent study using RGS in patients with NETs examined results of 44 patients explored after injection of 68 Ga-DOTATATE. 116 In this series, there were only 3 patients with PPGL, where 6 tumors were found at exploration.…”

“…Two additional case reports described 1 patient each with PCC who developed recurrences 15 or 25 years after initial primary diagnosis. 114,115 Both reports describe the use of γprobes after 123 I-MIBG scans, which were helpful to detect the metastatic lesions intraoperatively. One case noted recurrence 6 months later, suggesting that RGS was not entirely successful.…”