PRAP study-partial versus radical adrenalectomy in hereditary pheochromocytomas

Kai Xu; Johan F Langenhuijsen; Charlotte L Viëtor; Richard A Feelders; Tessa M van Ginhoven; Yasir S Elhassan; Fabio Bioletto; Mirko Parasiliti-Caprino; Wouter T Zandee; Schelto Kruijff; Samuel Backman; Tobias Åkerström; Christina Pamporaki; Nicole Bechmann; Charlotte Lussey-Lepoutre; Letizia Canu; Rebecca V Steenaard; Natacha Driessens; Marieke Velema; Koen M A Dreijerink; Anton F Engelsman; Henri J L M Timmers; Joanne M de Laat

doi:10.1093/ejendo/lvae108

PRAP study-partial versus radical adrenalectomy in hereditary pheochromocytomas

Kai Xu, Johan F Langenhuijsen, Charlotte L Viëtor, Richard A Feelders, Tessa M van Ginhoven, Yasir S Elhassan, Fabio Bioletto, Mirko Parasiliti-Caprino, Wouter T Zandee, Schelto Kruijff, Samuel Backman, Tobias Åkerström, Christina Pamporaki, Nicole Bechmann, Charlotte Lussey-Lepoutre, Letizia Canu, Rebecca V Steenaard, Natacha Driessens, Marieke Velema, Koen M A DreijerinkAnton F Engelsman, Henri J L M Timmers, Joanne M de Laat^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

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Abstract

OBJECTIVE: Hereditary pheochromocytoma (hPCC) commonly develops bilaterally, causing adrenal insufficiency when standard treatment, radical adrenalectomy (RA), is performed. Partial adrenalectomy (PA) aims to preserve adrenal function, but with higher recurrence rates. This study compares outcomes of PA versus RA in hPCC.

METHODS: Patients with hPCC due to pathogenic variants in RET, VHL, NF1, MAX, and TMEM127 from 12 European centers (1974-2023) were studied retrospectively. Stratified analysis based on surgery type and initial presentation was conducted. The main outcomes included recurrence, adrenal insufficiency, metastasis, and mortality.

RESULTS: The study included 256 patients (223 RA, 33 PA). Ipsilateral recurrence rates were 9/223 (4%) after RA versus 5/33 (15%) after PA (P = 0.02). Metastasis and mortality did not differ between groups. Overall, 103 patients (40%) underwent bilateral adrenalectomy either synchronously or metachronously (75 RA, 28 PA). Of these, 46% developed adrenal insufficiency after PA.In total, 191 patients presented with initial unilateral disease, of whom 50 (26%) developed metachronous contralateral disease, most commonly in RET, VHL, and MAX. In patients with metachronous bilateral disease, adrenal insufficiency developed in 3/4 (75%) when PA was performed as the first operation followed by RA, compared to 1/7 (14%) when PA was performed as the second operation after prior RA (P = 0.09).

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.

Original language	English
Pages (from-to)	345-353
Number of pages	9
Journal	European Journal of Endocrinology
Volume	191
Issue number	3
DOIs	https://doi.org/10.1093/ejendo/lvae108
Publication status	Published - 30-Aug-2024

Keywords

Humans
Pheochromocytoma/surgery
Adrenalectomy/methods
Adrenal Gland Neoplasms/surgery
Female
Male
Adult
Retrospective Studies
Middle Aged
Young Adult
Adolescent
Neoplasm Recurrence, Local/epidemiology
Adrenal Insufficiency/etiology
Treatment Outcome
Aged
Child

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Xu, K., Langenhuijsen, J. F., Viëtor, C. L., Feelders, R. A., van Ginhoven, T. M., Elhassan, Y. S., Bioletto, F., Parasiliti-Caprino, M., Zandee, W. T., Kruijff, S., Backman, S., Åkerström, T., Pamporaki, C., Bechmann, N., Lussey-Lepoutre, C., Canu, L., Steenaard, R. V., Driessens, N., Velema, M., ... de Laat, J. M. (2024). PRAP study-partial versus radical adrenalectomy in hereditary pheochromocytomas. European Journal of Endocrinology, 191(3), 345-353. https://doi.org/10.1093/ejendo/lvae108

@article{dba0db1763ba47dcbd7539a184dc47f2,

title = "PRAP study-partial versus radical adrenalectomy in hereditary pheochromocytomas",

abstract = "OBJECTIVE: Hereditary pheochromocytoma (hPCC) commonly develops bilaterally, causing adrenal insufficiency when standard treatment, radical adrenalectomy (RA), is performed. Partial adrenalectomy (PA) aims to preserve adrenal function, but with higher recurrence rates. This study compares outcomes of PA versus RA in hPCC.METHODS: Patients with hPCC due to pathogenic variants in RET, VHL, NF1, MAX, and TMEM127 from 12 European centers (1974-2023) were studied retrospectively. Stratified analysis based on surgery type and initial presentation was conducted. The main outcomes included recurrence, adrenal insufficiency, metastasis, and mortality.RESULTS: The study included 256 patients (223 RA, 33 PA). Ipsilateral recurrence rates were 9/223 (4%) after RA versus 5/33 (15%) after PA (P = 0.02). Metastasis and mortality did not differ between groups. Overall, 103 patients (40%) underwent bilateral adrenalectomy either synchronously or metachronously (75 RA, 28 PA). Of these, 46% developed adrenal insufficiency after PA.In total, 191 patients presented with initial unilateral disease, of whom 50 (26%) developed metachronous contralateral disease, most commonly in RET, VHL, and MAX. In patients with metachronous bilateral disease, adrenal insufficiency developed in 3/4 (75%) when PA was performed as the first operation followed by RA, compared to 1/7 (14%) when PA was performed as the second operation after prior RA (P = 0.09).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.",

keywords = "Humans, Pheochromocytoma/surgery, Adrenalectomy/methods, Adrenal Gland Neoplasms/surgery, Female, Male, Adult, Retrospective Studies, Middle Aged, Young Adult, Adolescent, Neoplasm Recurrence, Local/epidemiology, Adrenal Insufficiency/etiology, Treatment Outcome, Aged, Child",

author = "Kai Xu and Langenhuijsen, {Johan F} and Vi{\"e}tor, {Charlotte L} and Feelders, {Richard A} and {van Ginhoven}, {Tessa M} and Elhassan, {Yasir S} and Fabio Bioletto and Mirko Parasiliti-Caprino and Zandee, {Wouter T} and Schelto Kruijff and Samuel Backman and Tobias {\AA}kerstr{\"o}m and Christina Pamporaki and Nicole Bechmann and Charlotte Lussey-Lepoutre and Letizia Canu and Steenaard, {Rebecca V} and Natacha Driessens and Marieke Velema and Dreijerink, {Koen M A} and Engelsman, {Anton F} and Timmers, {Henri J L M} and {de Laat}, {Joanne M}",

note = "{\textcopyright} The Author(s) 2024. Published by Oxford University Press on behalf of European Society of Endocrinology.",

year = "2024",

month = aug,

day = "30",

doi = "10.1093/ejendo/lvae108",

language = "English",

volume = "191",

pages = "345--353",

journal = "European Journal of Endocrinology",

issn = "0804-4643",

publisher = "BIOSCIENTIFICA LTD",

number = "3",

}

Xu, K, Langenhuijsen, JF, Viëtor, CL, Feelders, RA, van Ginhoven, TM, Elhassan, YS, Bioletto, F, Parasiliti-Caprino, M, Zandee, WT , Kruijff, S, Backman, S, Åkerström, T, Pamporaki, C, Bechmann, N, Lussey-Lepoutre, C, Canu, L, Steenaard, RV, Driessens, N, Velema, M, Dreijerink, KMA, Engelsman, AF, Timmers, HJLM & de Laat, JM 2024, 'PRAP study-partial versus radical adrenalectomy in hereditary pheochromocytomas', European Journal of Endocrinology, vol. 191, no. 3, pp. 345-353. https://doi.org/10.1093/ejendo/lvae108

TY - JOUR

T1 - PRAP study-partial versus radical adrenalectomy in hereditary pheochromocytomas

AU - Xu, Kai

AU - Langenhuijsen, Johan F

AU - Viëtor, Charlotte L

AU - Feelders, Richard A

AU - van Ginhoven, Tessa M

AU - Elhassan, Yasir S

AU - Bioletto, Fabio

AU - Parasiliti-Caprino, Mirko

AU - Zandee, Wouter T

AU - Kruijff, Schelto

AU - Backman, Samuel

AU - Åkerström, Tobias

AU - Pamporaki, Christina

AU - Bechmann, Nicole

AU - Lussey-Lepoutre, Charlotte

AU - Canu, Letizia

AU - Steenaard, Rebecca V

AU - Driessens, Natacha

AU - Velema, Marieke

AU - Dreijerink, Koen M A

AU - Engelsman, Anton F

AU - Timmers, Henri J L M

AU - de Laat, Joanne M

N1 - © The Author(s) 2024. Published by Oxford University Press on behalf of European Society of Endocrinology.

PY - 2024/8/30

Y1 - 2024/8/30

N2 - OBJECTIVE: Hereditary pheochromocytoma (hPCC) commonly develops bilaterally, causing adrenal insufficiency when standard treatment, radical adrenalectomy (RA), is performed. Partial adrenalectomy (PA) aims to preserve adrenal function, but with higher recurrence rates. This study compares outcomes of PA versus RA in hPCC.METHODS: Patients with hPCC due to pathogenic variants in RET, VHL, NF1, MAX, and TMEM127 from 12 European centers (1974-2023) were studied retrospectively. Stratified analysis based on surgery type and initial presentation was conducted. The main outcomes included recurrence, adrenal insufficiency, metastasis, and mortality.RESULTS: The study included 256 patients (223 RA, 33 PA). Ipsilateral recurrence rates were 9/223 (4%) after RA versus 5/33 (15%) after PA (P = 0.02). Metastasis and mortality did not differ between groups. Overall, 103 patients (40%) underwent bilateral adrenalectomy either synchronously or metachronously (75 RA, 28 PA). Of these, 46% developed adrenal insufficiency after PA.In total, 191 patients presented with initial unilateral disease, of whom 50 (26%) developed metachronous contralateral disease, most commonly in RET, VHL, and MAX. In patients with metachronous bilateral disease, adrenal insufficiency developed in 3/4 (75%) when PA was performed as the first operation followed by RA, compared to 1/7 (14%) when PA was performed as the second operation after prior RA (P = 0.09).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.

AB - OBJECTIVE: Hereditary pheochromocytoma (hPCC) commonly develops bilaterally, causing adrenal insufficiency when standard treatment, radical adrenalectomy (RA), is performed. Partial adrenalectomy (PA) aims to preserve adrenal function, but with higher recurrence rates. This study compares outcomes of PA versus RA in hPCC.METHODS: Patients with hPCC due to pathogenic variants in RET, VHL, NF1, MAX, and TMEM127 from 12 European centers (1974-2023) were studied retrospectively. Stratified analysis based on surgery type and initial presentation was conducted. The main outcomes included recurrence, adrenal insufficiency, metastasis, and mortality.RESULTS: The study included 256 patients (223 RA, 33 PA). Ipsilateral recurrence rates were 9/223 (4%) after RA versus 5/33 (15%) after PA (P = 0.02). Metastasis and mortality did not differ between groups. Overall, 103 patients (40%) underwent bilateral adrenalectomy either synchronously or metachronously (75 RA, 28 PA). Of these, 46% developed adrenal insufficiency after PA.In total, 191 patients presented with initial unilateral disease, of whom 50 (26%) developed metachronous contralateral disease, most commonly in RET, VHL, and MAX. In patients with metachronous bilateral disease, adrenal insufficiency developed in 3/4 (75%) when PA was performed as the first operation followed by RA, compared to 1/7 (14%) when PA was performed as the second operation after prior RA (P = 0.09).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.

KW - Humans

KW - Pheochromocytoma/surgery

KW - Adrenalectomy/methods

KW - Adrenal Gland Neoplasms/surgery

KW - Female

KW - Male

KW - Adult

KW - Retrospective Studies

KW - Middle Aged

KW - Young Adult

KW - Adolescent

KW - Neoplasm Recurrence, Local/epidemiology

KW - Adrenal Insufficiency/etiology

KW - Treatment Outcome

KW - Aged

KW - Child

U2 - 10.1093/ejendo/lvae108

DO - 10.1093/ejendo/lvae108

M3 - Article

C2 - 39171965

SN - 0804-4643

VL - 191

SP - 345

EP - 353

JO - European Journal of Endocrinology

JF - European Journal of Endocrinology

IS - 3

ER -

PRAP study-partial versus radical adrenalectomy in hereditary pheochromocytomas

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