PRAP study-partial versus radical adrenalectomy in hereditary pheochromocytomas

Area of Science:

• Endocrinology
• Surgical Oncology
• Genetics

Background:

• Hereditary pheochromocytoma (hPCC) often presents bilaterally, posing a challenge for adrenal-preserving surgery.
• Radical adrenalectomy (RA) ensures tumor removal but leads to adrenal insufficiency.
• Partial adrenalectomy (PA) aims to preserve adrenal function but carries a risk of recurrence.

Purpose of the Study:

• To compare the outcomes of PA versus RA in patients with hPCC.
• To evaluate the safety and efficacy of PA in preserving adrenal function.
• To analyze recurrence, adrenal insufficiency, metastasis, and mortality rates between surgical approaches.

Main Methods:

• Retrospective analysis of 256 hPCC patients from 12 European centers (1974-2023).
• Patients had pathogenic variants in RET, VHL, NF1, MAX, or TMEM127.
• Outcomes stratified by surgery type (PA vs. RA) and initial presentation.

Main Results:

• Ipsilateral recurrence was higher after PA (15%) than RA (4%) (P=0.02).
• Metastasis and mortality rates were similar between PA and RA groups.
• Adrenal insufficiency occurred in 46% of patients undergoing PA for bilateral disease; this rate was lower when PA was the second operation (14% vs 75%).

Conclusions:

• PA is associated with higher local recurrence rates than RA in hPCC.
• PA is a safe option for preserving adrenal function in hPCC, particularly when performed as a second procedure for bilateral disease.
• Metastasis and disease-specific mortality are comparable between PA and RA, supporting PA's role in select hPCC cases.