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JAMA Netw Open. 2019 Aug 2;2(8):e198898. doi: 10.1001/jamanetworkopen.2019.8898.

Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy.

Author information

1
Section of Preventive Medicine, Medical Center-University of Freiburg, Faculty of Medicine, Albert-Ludwig-University Freiburg, Freiburg, Germany.
2
Neuroendocrinology Laboratory, Endocrinology Institute, Almazov National Medical Research Centre, St Petersburg, Russia.
3
Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, Minnesota.
4
Aix Marseille University, INSERM, Marseille Medical Genetics, Department of Endocrinology, Assistance Publique Hopitaux de Marseille, Marseille, France.
5
Department of Surgery, Huyssens Foundation Clinics, Essen, Germany.
6
Neuroendocrine Tumors Service, Sheba Medical Center and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
7
Division of General Surgery, Mayo Clinic, Rochester, Minnesota.
8
Department of Oncologic and Urologic Surgery, the 903rd PLA Hospital, Wenzhou Medical University, Hangzhou, Zhejiang, People's Republic of China.
9
Department of Endocrinology, Seth GS Medical College and KEM Hospital, Mumbai, India.
10
Department of Surgical Oncology, Bakhrushin Brothers Moscow City Hospital, Moscow, Russia.
11
Department of Surgery, Endocrinology Research Center, Moscow, Russia.
12
Department of Endocrinology, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.
13
Instituto do Cancer do Estado de São Paulo (ICESP), Serviço de Endocrinologia, Hospital das Clínicas (HCFMUSP), Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
14
Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy.
15
Operative Unit of the Endocrinology Department of Medicine (DIMED), University of Padua, Padua, Italy.
16
Department of Molecular Endocrinology, Institute of Endocrinology, Prague, Czech Republic.
17
Department of Endocrine Oncology and Nuclear Medicine, Maria Sklodowska-Curie Institute-Oncology Center, Gliwice Branch, Gliwice, Poland.
18
Institute of Endocrinology and Metabolism NAMS of Ukraine, Kiev, Ukraine.
19
Institute of Cardiology, Department of Hypertension, Warsaw, Poland.
20
Department of Endocrinology, Ospedale Niguarda Cà Granda, Milan, Italy.
21
Department of Medicine, Division of Endocrinology, Tufts Medical Center, Boston, Massachusetts.
22
Department of Radiology, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
23
Department of Urology, Xiangya Hospital, Central South University, Changsha, China.
24
Department of Biochemistry and Molecular Biology, School of Life Sciences, Central South University, Changsha, China.
25
Hospital Universitari de Girona, Gerencia Territorial Girona, Institut Català de la Salut, Girona, Spain.
26
Endocrine Section, Hospital del Salvador, Santiago de Chile, Department of Medicine University of Chile, Santiago, Chile.
27
Department of Endocrinology and Metabolic Diseases, Leiden University Medical Center, Leiden, the Netherlands.
28
Department of Endocrinology, E.E. Eichwald Clinic, I.I. Mechnikov Northwestern State Medical University, St Petersburg, Russia.
29
Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.
30
Neuroendocrine Tumor Unit, Endocrinology and Metabolism Service, Department of Medicine, ENETS Centre of Excellence, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
31
Division of Endocrinology and Metabolism, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
32
Endocrinology, Abdominal Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
33
Department of Nuclear Medicine and Endocrinology, Second Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic.
34
Department of Medicine II, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
35
Genomic Medicine Institute, Lerner Research Institute and Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio.

Abstract

Importance:

Large studies investigating long-term outcomes of patients with bilateral pheochromocytomas treated with either total or cortical-sparing adrenalectomies are needed to inform clinical management.

Objective:

To determine the association of total vs cortical-sparing adrenalectomy with pheochromocytoma-specific mortality, the burden of primary adrenal insufficiency after bilateral adrenalectomy, and the risk of pheochromocytoma recurrence.

Design, Setting, and Participants:

This cohort study used data from a multicenter consortium-based registry for 625 patients treated for bilateral pheochromocytomas between 1950 and 2018. Data were analyzed from September 1, 2018, to June 1, 2019.

Exposures:

Total or cortical-sparing adrenalectomy.

Main Outcomes and Measures:

Primary adrenal insufficiency, recurrent pheochromocytoma, and mortality.

Results:

Of 625 patients (300 [48%] female) with a median (interquartile range [IQR]) age of 30 (22-40) years at diagnosis, 401 (64%) were diagnosed with synchronous bilateral pheochromocytomas and 224 (36%) were diagnosed with metachronous pheochromocytomas (median [IQR] interval to second adrenalectomy, 6 [1-13] years). In 505 of 526 tested patients (96%), germline mutations were detected in the genes RET (282 patients [54%]), VHL (184 patients [35%]), and other genes (39 patients [7%]). Of 849 adrenalectomies performed in 625 patients, 324 (52%) were planned as cortical sparing and were successful in 248 of 324 patients (76.5%). Primary adrenal insufficiency occurred in all patients treated with total adrenalectomy but only in 23.5% of patients treated with attempted cortical-sparing adrenalectomy. A third of patients with adrenal insufficiency developed complications, such as adrenal crisis or iatrogenic Cushing syndrome. Of 377 patients who became steroid dependent, 67 (18%) developed at least 1 adrenal crisis and 50 (13%) developed iatrogenic Cushing syndrome during median (IQR) follow-up of 8 (3-25) years. Two patients developed recurrent pheochromocytoma in the adrenal bed despite total adrenalectomy. In contrast, 33 patients (13%) treated with successful cortical-sparing adrenalectomy developed another pheochromocytoma within the remnant adrenal after a median (IQR) of 8 (4-13) years, all of which were successfully treated with another surgery. Cortical-sparing surgery was not associated with survival. Overall survival was associated with comorbidities unrelated to pheochromocytoma: of 63 patients who died, only 3 (5%) died of metastatic pheochromocytoma.

Conclusions and Relevance:

Patients undergoing cortical-sparing adrenalectomy did not demonstrate decreased survival, despite development of recurrent pheochromocytoma in 13%. Cortical-sparing adrenalectomy should be considered in all patients with hereditary pheochromocytoma.

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