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Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy
- Source :
- JAMA Network Open, Jama network open, 2(8):198898. AMER MEDICAL ASSOC, Jama Network Open, 2(8). AMER MEDICAL ASSOC
- Publication Year :
- 2019
- Publisher :
- Amer Medical Assoc, 2019.
-
Abstract
- Key Points Question Is cortical-sparing adrenalectomy associated with increased pheochromocytoma-specific morbidity and mortality for patients with bilateral pheochromocytomas compared with total adrenalectomy? Findings In this cohort study of 625 patients with bilateral pheochromocytomas, most had hereditary syndromes, but 36% initially presented with unilateral pheochromocytoma. Bilateral total adrenalectomy resulted in a high rate of adverse effects from glucocorticoid replacement therapy, whereas cortical-sparing surgery was not associated with a worse outcome. Meaning These findings suggest that cortical-sparing surgery may be the preferred approach for patients at risk for, or diagnosed with, bilateral pheochromocytomas, especially those harboring a germline mutation in one of the known predisposition genes.<br />This cohort study compares outcomes associated with cortical-sparing adrenalectomy vs total adrenalectomy for patients with bilateral pheochromocytomas.<br />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.
- Subjects :
- Male
endocrine system diseases
SURGERY
medicine.medical_treatment
Adrenal Gland Neoplasms
030230 surgery
Primary Adrenal Insufficiency
0302 clinical medicine
Interquartile range
Paraganglioma
Registries
Original Investigation
OUTCOMES
integumentary system
Adrenal crisis
Adrenalectomy
General Medicine
GERMLINE MUTATIONS
3. Good health
Online Only
Diabetes and Endocrinology
PARAGANGLIOMA
CONFER
030220 oncology & carcinogenesis
Female
medicine.symptom
hormones, hormone substitutes, and hormone antagonists
Adult
medicine.medical_specialty
endocrine system
GENETICS
Urology
Pheochromocytoma
Bilateral Pheochromocytomas
03 medical and health sciences
Young Adult
medicine
Adrenal insufficiency
MANAGEMENT
Humans
Medicine [Science]
RECURRENCE
Retrospective Studies
business.industry
Research
Retrospective cohort study
medicine.disease
PREDISPOSITION
3121 General medicine, internal medicine and other clinical medicine
Morbidity
Neoplasm Recurrence, Local
business
Organ Sparing Treatments
Subjects
Details
- Language :
- English
- ISSN :
- 25743805
- Database :
- OpenAIRE
- Journal :
- JAMA Network Open, Jama network open, 2(8):198898. AMER MEDICAL ASSOC, Jama Network Open, 2(8). AMER MEDICAL ASSOC
- Accession number :
- edsair.doi.dedup.....738c71edd24d1d48104851a041f25f2b