Treatment of Multiple Endocrine Neoplasia Type 2A with Solitary Unilateral Adrenal Pheochromocytoma
This protocol applies to patients with Multiple Endocrine Neoplasia Type 2A (MEN2A) who present with a pheochromocytoma of the adrenal gland that is solitary and unilateral, with no bilateral adrenal involvement at the time of diagnosis.
The unilateral nature of the pheochromocytoma is central to the treatment decision. Bilateral adrenalectomy carries a significant risk of adrenal insufficiency; therefore, a unilateral approach is indicated in patients with a solitary pheochromocytoma in this setting — even recognising that contralateral disease may develop over subsequent years.
Management involves surgical resection of the pheochromocytoma following appropriate preoperative preparation. A minimally invasive surgical route is the established approach in this setting; a procedure designed to preserve adrenal cortical function may be considered as an alternative. The full preoperative requirements, surgical selection criteria, and procedural sequence are specified in the complete protocol.
References
- Following bilateral adrenalectomy there is a significant risk of adrenal insufficiency, thus unilateral adrenalectomy is indicated in patients with a solitary PHEO, even though the majority of them will develop a contralateral PHEO within 10 years.
- 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.
DOI: 10.1089/thy.2014.0335
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