Division of Pediatric Endocrinology and Diabetes, Children's Mercy Hospital, Kansas City, MO, USA.
Division of Radiology, Children's Mercy Hospital, Kansas City, MO, USA.
J Pediatr Endocrinol Metab. 2024 May 30;37(7):657-662. doi: 10.1515/jpem-2024-0033. Print 2024 Jul 26.
Pheochromocytomas (PHEO) are neuroendocrine tumors rarely diagnosed in children. We are reporting on the management challenges of three adolescent patients who present with hereditary PHEO.
The index patient and his male sibling presented with bilateral PHEO, while a third patient presented with a unilateral PHEO, all associated with von Hippel-Lindau (VHL) syndrome. The patients were treated with computed tomography (CT)-guided percutaneous cryoablation (CRA) of the adrenal lesions, with varying degrees of success.
CT-guided percutaneous CRA of hereditary PHEO has not been reported in the pediatric population and may represent a novel treatment strategy that reduces the risk of intraprocedural complications and adrenal insufficiency (AI).
嗜铬细胞瘤（pheochromocytoma，pheo）是一种罕见的儿童神经内分泌肿瘤。我们报告了三例遗传性 pheo 青少年患者的治疗管理挑战。
索引患者及其男性同胞均表现为双侧 pheo，而第三位患者则表现为单侧 pheo，均与 von Hippel-Lindau（VHL）综合征相关。这些患者均接受了计算机断层扫描（CT）引导下经皮冷冻消融术（CRA）治疗肾上腺病变，疗效不一。
CT 引导下经皮 CRA 治疗遗传性 pheo 尚未在儿科人群中报道，可能代表一种新的治疗策略，可以降低术中并发症和肾上腺功能不全（adrenocortical insufficiency，AI）的风险。
