Pheochromocytoma
ICD-10 E27.5 · ICD-11 5A75

Pheochromocytoma with Paraganglioma — After Blood Pressure Control Did Not Meet Target

This protocol applies to pheochromocytoma arising in the context of paraganglioma — a tumor derived from extra-adrenal chromaffin cells of the sympathetic paravertebral ganglia of the thorax, abdomen, or pelvis. The management step described here is triggered when prior blood pressure optimization has not achieved defined target values.

The preceding line added a calcium channel blocker (nifedipine or amlodipine) to established α-adrenergic receptor blockade, with α-methyl-paratyrosine (metyrosine) as an optional adjunct. The targets for that step were: blood pressure below 130/80 mm Hg seated, systolic above 90 mm Hg standing, and heart rate 60–70 bpm seated / 70–80 bpm standing. This protocol is indicated when those targets are not met.

The approach at this stage centres on surgical resection of the paraganglioma. Which operative technique is appropriate depends on specific tumor and patient factors — the full decision algorithm is available in the structured protocol below.

References

DOI: 10.1210/jc.2014-1498

We suggest open resection for paragangliomas, but laparoscopic resection can be performed for small, noninvasive paragangliomas in surgically favorable locations.

A paraganglioma is a tumor derived from extra-adrenal chromaffin cells of the sympathetic paravertebral ganglia of thorax, abdomen, and pelvis.

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