Pheochromocytoma: Stages, Grading, and What You Need to Know (Sep 2024)
This month we covered how doctors classify pheochromocytoma — a rare tumor of the adrenal glands — and what those stages and grades mean for treatment and follow-up. If you or someone you care about is facing this diagnosis, here’s a clear, practical summary of the key points from our article.
How doctors stage and grade pheochromocytoma
Staging looks at where the tumor is and whether it has spread. Clinicians use imaging (CT or MRI) to check size, local invasion, and distant spread to lymph nodes or other organs. Functional imaging like MIBG or PET scans may find metastatic disease that routine scans miss.
Grading estimates how aggressive the tumor might behave. Pathologists look at tumor cell patterns, mitotic activity, and markers such as Ki-67. Systems like GAPP (grading for adrenal pheochromocytoma and paraganglioma) combine these features with biochemical profile. A higher grade or score suggests a greater chance of recurrence or spread, so care teams plan closer follow-up.
Practical steps: diagnosis, treatment, and follow-up
Biochemical testing comes first: blood or 24-hour urine metanephrines and catecholamines. If those are high, imaging follows. Before any biopsy or surgery, doctors control blood pressure — usually with alpha-blockers — because these tumors can release large amounts of adrenaline during handling.
Surgery is the main treatment for localized pheochromocytoma. Many patients have a laparoscopic adrenalectomy if the tumor is small and contained. Larger or invasive tumors may need open surgery. For metastatic cases, options include targeted radiotherapy (MIBG), systemic therapy, clinical trials, or symptom-focused care. Genetic testing is often recommended because inherited gene changes (like SDHB, VHL, RET) affect risk for other tumors and family screening.
Follow-up is lifelong for most people. That usually means periodic biochemical tests and imaging when indicated. The exact schedule depends on the initial stage, grade, and genetic results. If a tumor had features linked to aggressive behavior, follow-up will be more frequent.
Want to be ready for a clinic visit? Bring a copy of your test results and imaging, note when symptoms started and what triggers them, and list all medications. Ask whether genetic testing is advised, what follow-up schedule they recommend, and which symptoms should prompt an urgent call (severe headache, chest pain, dangerously high blood pressure).
We aim to make complex ideas simple and useful. If you missed the full article from September 2024, it walks through the staging details, explains grading systems with examples, and gives tips on preparing for surgery and long-term care. Reach out to your care team with any specific questions — every case is different, and treatment should be personalized.
