PRAP study - Partial versus radical adrenalectomy for hereditary pheochromocytomas

Kai Xu; Johannes Langenhuijsen; Charlotte Vietor; Richard Feelders; Ginhoven Tessa van; Yasir Elhassan; Fabio Bioletto; Mirko Parasiliticaprino; Wouter Zandee; Schelto Kruijff; Tobias AEkerstrom; Christina Pamporaki; Nicole Bechmann; Charlotte Lussey-Lepoutre; Letizia Canu; Rebecca Steenaard; Natacha Driessens; Marieke Velema; Koen Dreijerink; Anton Engelsman; Henri Timmers; Laat Marieke de

doi:10.1530/endoabs.99.RC7.1

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Endocrine Abstracts (2024) 99 RC7.1 | DOI: 10.1530/endoabs.99.RC7.1

ECE2024 Rapid Communications Rapid Communications 7: Endocrine-related Cancer (6 abstracts)

PRAP study - Partial versus radical adrenalectomy for hereditary pheochromocytomas

Kai Xu ¹ , Johannes Langenhuijsen ² , Charlotte Vietor ³ , Richard Feelders ⁴ , Tessa van Ginhoven ³ , Yasir Elhassan ⁵ , Fabio Bioletto ⁶ , Mirko Parasiliticaprino ⁶ , Wouter Zandee ⁷ , Schelto Kruijff ⁸ , Tobias Ækerström ⁹ , Christina Pamporaki ¹⁰ , Nicole Bechmann ¹⁰ , Charlotte Lussey-Lepoutre ¹¹ , Letizia Canu ¹² , Rebecca Steenaard ¹³ , Natacha Driessens ¹⁴ , Marieke Velema ¹⁵ , Koen Dreijerink ¹⁶ , Anton Engelsman ¹⁷ , Henri Timmers ¹ & Marieke de Laat ¹

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¹Radboud University Medical Center, Department of Internal Medicine, Division of Endocrinology, Nijmegen, Netherlands; ²Radboud University Medical Center, Department of Urology, Nijmegen, Netherlands; ³Erasmus MC, Department of Surgical Oncology and Gastrointestinal Surgery, Rotterdam, Netherlands; ⁴Erasmus MC, Department of Internal Medicine, Division of Endocrinology, Rotterdam, Netherlands; ⁵University of Birmingham, Institute of Metabolism and Systems Research, Birmingham, United Kingdom; ⁶University of Turin - Campus Luigi Einaudi, Department of Medical Sciences, Division of Endocrinology, Diabetes and Metabolism, Torino, Italy; ⁷University Medical Center Groningen, Department of Internal Medicine, Division of Endocrinology, Groningen, Netherlands; ⁸University Medical Center Groningen, Department of Surgery, Groningen, Netherlands; ⁹Uppsala universitet Inst f kirurgiska vetenskaper, Department of Surgical Sciences, Uppsala, Sweden, ¹⁰University Hospital Carl Gustav Carus Dresden, Department of Internal Medicine III, Dresden, Germany, ¹¹Faculty of Medicine Pierre et Marie Curie, nuclear medicine department, Paris, France, ¹²University of Florence, Department of Experimental and Clinical Biomedical Sciences, Endocrinology Unit, Firenze, Italy, ¹³Máxima Medisch Centrum, Department of Internal Medicine, Division of Endocrinology, Veldhoven, Netherlands, ¹⁴Hospital Erasme, Department of Endocrinology, Bruxelles, Belgium, ¹⁵Slingeland Hospital, Department of Internal Medicine, Division of Endocrinology, Doetinchem, Netherlands, ¹⁶VU University Medical Center, Department of Internal Medicine, Section of Endocrinology, Amsterdam, Netherlands, ¹⁷VU University Medical Center, Department of Surgery, Amsterdam, Netherlands

Introduction: Pheochromocytoma, a rare catecholamine-secreting adrenal tumor, can cause hypertension and life-threatening complications. Hereditary cases have an increased risk of developing bilateral disease. Standard treatment involves radical adrenalectomy, leading to adrenal insufficiency in bilateral cases. Partial adrenalectomy aims to preserve adrenal function but has higher recurrence rates. This study compares outcomes of partial vs radical adrenalectomy in hereditary pheochromocytoma, focusing on cases with initial unilateral disease.

Methods: Standard treatment involves radical adrenalectomy, leading to adrenal insufficiency in bilateral cases. Patients were categorized by initial disease presentation and type of surgery. Patients were categorized by initial presentation and surgery type. Outcomes included contra- and ipsilateral recurrence, adrenocortical insufficiency, metastasis, and disease-specific survival.

Results: 256 patients with hereditary pheochromocytoma were included of which 108 (42%) with RET, 67 (26%) NF1, 62 (24%) VHL, 11 (5%) TMEM127 and 8 (3%) MAX mutations. There were 191 (75%) patients that initially presented with unilateral tumors. Radical adrenalectomy was performed in 223 patients (87%), in 75 of whom bilaterally. A partial adrenalectomy was performed in 33 (13%). Median follow-up was 100 months. In 191 patients who presented with unilateral pheochromocytomas, 50 (26%) developed contralateral tumors, at a median follow-up time of 56 months. Incidence of metachronous contralateral disease was highest in VHL, RET and MAX mutations. Ipsilateral recurrence was low, 6% in the partial group and 3% in the radical group (P=0.47). In patients who developped contralateral pheochromocytoma, partial adrenalectomy as first intervention resulted in 3/4 (75%) adrenal insufficiency compared to 1/7 (14%) when the second adrenalectomy was partial (P = 0.09). Overall, 153 (60%) patients underwent unilateral surgery and 103 (40%) bilateral (synchronously or metachronous). Among 103 patients with bilateral adrenalectomies, adrenal insufficiency risk was 13/28 (46%) in partial cases and 75/75 (100%) in radical cases (P < 0.001). Recurrence rate was 5/28 (18%) for cases with partial adrenalectomy vs 7/75 (9%) those with radical operation (P = 0.30). In survival analysis time till ipislateral recurrence was significantly shorter in the partial group (P=0.035). Metastasis and disease-specific survival did not differ between groups.

Conclusion: In hereditary pheochromocytoma, partial adrenalectomy offers lower rates of adrenal insufficiency compared to radical surgery, with slightly higher recurrence rate for the partial adrenalectomy. Risk of metastasis and disease-specific survival was similar between groups. In patients with metachronous bilateral disease, preserving adrenal function possibly more succesful when partial adrenalectomy was performed as the second intervention.