PRAP study—partial versus radical adrenalectomy in hereditary pheochromocytomas
Authors' conclusion
In patients with hPCC undergoing PA, local recurrence rates are higher than after RA, but metastasis and disease-specific mortality are similar. Therefore, PA seems a safe method to preserve adrenal function in patients with hPCC, in cases of both synchronous and metachronous bilateral disease, when performed as a second operation.
Study context
- Population
- Patients with hereditary pheochromocytoma due to pathogenic variants in RET, VHL, NF1, MAX and TMEM127, 12 European centers (1974–2023)
- Participants
- 256 patients
- Exposure
- Partial adrenalectomy vs radical adrenalectomy
- Partial adrenalectomy (PA)n = 33
- Radical adrenalectomy (RA)n = 223reference
- Outcome
- Main outcomes: recurrence, adrenal insufficiency, metastasis and mortality
Methodological detailsmulticentre, 12 European centers, stratified analysis by surgery type and initial presentation
- Multicentre
- 12 European centers
- Stratified analysis by surgery type and initial presentation
Main finding
As reported in the abstract
Ipsilateral recurrence
P = 0.02
Observational data: associations, not effects.
Further findings
Events
| Outcome | PA | RA | P value |
|---|---|---|---|
| Bilateral adrenalectomy (synchronous or metachronous) | 28 | 75 | |
| Adrenal insufficiency in metachronous bilateral disease: PA first then RA vs PA second after prior RA | P = 0.09 |
- Bilateral adrenalectomy (synchronous or metachronous) — Overall bilateral adrenalectomy
- 103 patients (40%)
- Bilateral adrenalectomy (synchronous or metachronous) — Adrenal insufficiency after PA among bilateral adrenalectomy
- 46%
- Adrenal insufficiency in metachronous bilateral disease: PA first then RA vs PA second after prior RA: PA first, then RA 75%
- Adrenal insufficiency in metachronous bilateral disease: PA first then RA vs PA second after prior RA: PA second, after prior RA 14%
- Adrenal insufficiency in metachronous bilateral disease: PA first then RA vs PA second after prior RA: 3/4 vs 1/7
Metachronous contralateral disease in initial unilateral disease
- Initial unilateral disease
- 191 patients
- Developed metachronous contralateral disease (most commonly RET, VHL, MAX)
- 50 (26%)
Original publication
PRAP study—partial versus radical adrenalectomy in hereditary pheochromocytomas. Eur J Endocrinol. Published 22 August 2024. doi:10.
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