Update on Pheochromocytoma and Paraganglioma from the SSO Endocrine and Head and Neck Disease Site Working Group, Part 2 of 2: Perioperative Management and Outcomes of Pheochromocytoma and Paraganglioma

Abstract

This is the second part of a two-part review on pheochromocytoma and paragangliomas (PPGLs). In this part, perioperative management, including preoperative preparation, intraoperative, and postoperative interventions are reviewed. Current data on outcomes following resection are presented, including outcomes after cortical-sparing adrenalectomy for bilateral adrenal disease. In addition, pathological features of malignancy, surveillance considerations, and the management of advanced disease are also discussed.

FIG. 1

a Diagram outlining port placement for robotic retroperitoneoscopic approach to an adrenalectomy. b Patient prepped, draped, and ports placed for robotic retroperitoneal total adrenalectomy

FIG. 2

Eleven-year-old male with VHL syndrome who underwent laparoscopic bilateral partial adrenalectomies guided with intraoperative ultrasound for bilateral pheochromocytomas. a Right adrenal tumor measuring 1.8 cm. b Post right partial adrenalectomy with preservation of the right adrenal vein. c Left adrenal tumor measuring 3.0 cm. d Post left partial adrenalectomy with preservation of the left adrenal vein. *Left and right adrenal tumors, respectively, ^Left and right adrenal veins, respectively, IVC inferior vena cava

FIG. 3

A 59-year-old male with SDHB who underwent PRRT for progressive metastatic PPGL showing mild response after four cycles of treatment. a Baseline 68-gallium DOTATATE PET/CT scan showing widely metastatic disease. b 68-Gallium DOTATATE PET/CT scan showing mild tumor response after four cycles of Lutathera therapy