This protocol addresses patients with multiple endocrine neoplasia type 2A or 2B (MEN2A/2B) who have a concurrent pheochromocytoma (PHEO). Approximately 50% of patients with MEN2B develop pheochromocytomas, making recognition and appropriate sequencing of care critical.
When a pheochromocytoma coexists with other MEN2-related tumours, the order of surgical intervention is a key clinical decision.
Management centres on surgical removal of the pheochromocytoma, which must be addressed before any other MEN2-related surgical procedures. The operative approach involves minimally invasive adrenalectomy techniques, with a cortical-sparing strategy available as an alternative in appropriate cases.
Approximately 50% of patients with MEN2B develop PHEOs.
If they coexist, a PHEO should be removed prior to surgery for either MTC or HPTH.
After appropriate preoperative preparation a PHEO should be resected by laparoscopic or retroperitoneoscopic adrenalectomy.
Subtotal adrenalectomy to preserve adrenal cortical function should be considered as an alternative procedure.
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