Management Approach for Pheochromocytoma

Diagnostic Evaluation

The American College of Medical Genetics recommends measurement of plasma free metanephrines as the most sensitive and specific single test for diagnosing pheochromocytoma in clinically suspected cases 3, 4
If plasma testing is equivocal, a follow-up 24-hour urine collection for catecholamines and metanephrines should be performed, as suggested by the European Society of Endocrinology 3, 4
Additional measurement of plasma methoxytyramine provides useful information to assess the likelihood of malignancy, according to the European Society of Medical Oncology 5

The European Society of Medical Oncology recommends localization by CT or MRI of the abdomen after positive biochemical testing 5, 4
Chest CT should be included to evaluate for metastatic disease, as suggested by the American College of Radiology 1, 6
Functional imaging, such as Meta-iodobenzylguanidine (MIBG) scintigraphy, is helpful to detect multifocal disease, according to the Society of Nuclear Medicine and Molecular Imaging 3, 4

The European Society of Medical Oncology states that complete surgical extirpation (R0 resection) is the mainstay of potentially curative treatment for pheochromocytoma 1, 2
For malignant pheochromocytoma, cytoreductive surgery might be considered, as suggested by the American College of Surgeons 1, 2

The American College of Medical Oncology recommends disease and symptom control as the main treatment goal for patients with inoperable pheochromocytoma 1, 2
Treatment options include radiopharmaceuticals, such as 131I-MIBG, for patients with sufficient MIBG uptake, as suggested by the Society of Nuclear Medicine and Molecular Imaging 1, 2

The European Society of Endocrinology recommends biochemical testing approximately 14 days following surgery to check for remaining disease 1
Long-term follow-up includes clinical evaluation, biochemical testing, and imaging with CT of thorax and abdomen as clinically indicated, according to the American College of Endocrinology 1, 2

The European Society of Medical Oncology states that risk factors for malignancy include tumor size ≥5 cm, extra-adrenal paraganglioma, SDHB germline mutation, and elevated plasma methoxytyramine (>3x upper limit) 5, 7
Malignancy is defined only by the presence of metastatic lesions at sites where chromaffin cells are normally absent, as suggested by the European Association of Nuclear Medicine 8

The American College of Medical Genetics recommends considering genetic testing, particularly in patients with family history of pheochromocytoma/paraganglioma, young age at diagnosis, bilateral or multifocal disease, or extra-adrenal location 7, 8
Associated genetic syndromes include MEN2, VHL, NF1, and hereditary paraganglioma syndromes, according to the European Society of Human Genetics 3, 7