Role of Chromogranin A in Pheochromocytoma Diagnosis and Management

Diagnostic Value

Immunohistochemical staining for chromogranin A is used to identify pheochromocytoma in pathological specimens, helping differentiate it from adrenocortical tumors, as recommended by the European Society for Medical Oncology 1

Chromogranin A is valuable in long-term follow-up of patients with pheochromocytoma/paraganglioma, with testing recommended approximately 14 days following surgery and thereafter every 3-4 months for 2-3 years, according to the European Society for Medical Oncology 2, 3
Elevated circulating chromogranin A during follow-up should prompt imaging studies including thorax and abdomen CT and functional imaging (PET FDG in most cases), as suggested by the European Society for Medical Oncology 2, 3

Lifelong follow-up with chromogranin A monitoring is recommended for patients with malignant pheochromocytoma/paraganglioma, SDHB mutation, extra-adrenal primary disease, or pheochromocytoma without relevant preoperative hormone secretion, as recommended by the European Society for Medical Oncology 2, 3
Certain conditions can cause false elevations in chromogranin A, including renal failure and use of proton pump inhibitors, as noted by the American College of Chest Physicians 4

At diagnosis: Measure chromogranin A along with metanephrines and normetanephrines, as recommended by the European Society for Medical Oncology and the European Association of Nuclear Medicine 2, 5
Post-surgery: Repeat chromogranin A measurement ~14 days after surgery to check for remaining disease, as suggested by the European Society for Medical Oncology 2, 3
Follow-up schedule: Every 3-4 months for first 2-3 years, then every 6 months thereafter, as recommended by the European Society for Medical Oncology 2, 3