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

Basit Öğe Kaydı
dc.contributor.authorNeumann, Hartmut P. H.
dc.contributor.authorTsoy, Uliana
dc.contributor.authorBancos, Irina
dc.contributor.authorAmodru, Vincent
dc.contributor.authorWalz, Martin K.
dc.contributor.authorTirosh, Amit
dc.contributor.authorKaur, Ravinder Jeet
dc.contributor.authorMcKenzie, Travis
dc.contributor.authorQi, Xiaoping
dc.contributor.authorBandgar, Tushar
dc.contributor.authorPetrov, Roman
dc.contributor.authorYukina, Marina Y.
dc.contributor.authorRoslyakova, Anna
dc.contributor.authorvan der Horst-Schrivers, Anouk N. A.
dc.contributor.authorBerends, Annika M. A.
dc.contributor.authorHoff, Ana O.
dc.contributor.authorCastroneves, Luciana Audi
dc.contributor.authorFerrara, Alfonso Massimiliano
dc.contributor.authorRizzati, Silvia
dc.contributor.authorMian, Caterina
dc.contributor.authorDvorakova, Sarka
dc.contributor.authorHasse-Lazar, Kornelia
dc.contributor.authorKvachenyuk, Andrey
dc.contributor.authorPeczkowska, Mariola
dc.contributor.authorLoli, Paola
dc.contributor.authorErenler, Feyza
dc.contributor.authorKrauss, Tobias
dc.contributor.authorAlmeida, Madson Q.
dc.contributor.authorLiu, Longfei
dc.contributor.authorZhu, Feizhou
dc.contributor.authorRecasens, Monica
dc.contributor.authorWohllk, Nelson
dc.contributor.authorCorssmit, Eleonora P. M.
dc.contributor.authorShafigullina, Zulfiya
dc.contributor.authorCalissendorff, Jan
dc.contributor.authorGrozinsky-Glasberg, Simona
dc.contributor.authorKunavisarut, Tada
dc.contributor.authorSchalin-Jantti, Camilla
dc.contributor.authorCastinetti, Frederic
dc.contributor.authorVlcek, Petr
dc.contributor.authorBeltsevich, Dmitry
dc.contributor.authorEgorov, Viacheslav, I
dc.contributor.authorSchiavi, Francesca
dc.contributor.authorLinks, Thera P.
dc.contributor.authorLechan, Ronald M.
dc.contributor.authorBausch, Birke
dc.contributor.authorYoung, William F., Jr.
dc.contributor.authorEng, Charis
dc.contributor.authorJaiswal, Sanjeet Kumar
dc.contributor.authorZschiedrich, Stefan
dc.contributor.authorFragoso, Maria C. B., V
dc.contributor.authorPereira, Maria A. A.
dc.contributor.authorLi, Minghao
dc.contributor.authorBiarnes Costa, Josefina
dc.contributor.authorJuhlin, Carl Christofer
dc.contributor.authorGross, David
dc.contributor.authorViolante, Alice H. D.
dc.contributor.authorKocjan, Tomaz
dc.contributor.authorNgeow, Joanne
dc.contributor.authorYoel, Uri
dc.contributor.authorFraenkel, Merav
dc.contributor.authorSimsir, Ilgin Yildirim
dc.contributor.authorUgurlu, M. Umit
dc.contributor.authorZiagaki, Athanasia
dc.contributor.authorRobles Diaz, Luis
dc.contributor.authorKudlai, Inna Stepanovna
dc.contributor.authorGimm, Oliver
dc.contributor.authorScherbaum, Christina Rebecca
dc.contributor.authorAbebe-Campino, Gadi
dc.contributor.authorBarbon, Giovanni
dc.contributor.authorTaschin, Elisa
dc.contributor.authorMalinoc, Angelica
dc.contributor.authorKhudiakova, Natalia Valeryevna
dc.contributor.authorIvanov, Nikita, V
dc.contributor.authorPfeifer, Marija
dc.contributor.authorZovato, Stefania
dc.contributor.authorPloeckinger, Ursula
dc.contributor.authorMakay, Ozer
dc.contributor.authorGrineva, Elena
dc.contributor.authorJarzab, Barbara
dc.contributor.authorJanuszewicz, Andrzej
dc.contributor.authorShah, Nalini
dc.contributor.authorSeufert, Jochen
dc.contributor.authorOpocher, Giuseppe
dc.contributor.authorLarsson, Catharina
dc.date.accessioned2019-10-27T09:42:08Z
dc.date.available2019-10-27T09:42:08Z
dc.date.issued2019
dc.departmentEge Üniversitesien_US
dc.description.abstractIMPORTANCE 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 mutationswere 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 survivalwas 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.en_US
dc.identifier.doi10.1001/jamanetworkopen.2019.8898en_US
dc.identifier.issn2574-3805
dc.identifier.issue8en_US
dc.identifier.pmid31397861en_US
dc.identifier.urihttps://doi.org/10.1001/jamanetworkopen.2019.8898
dc.identifier.urihttps://hdl.handle.net/11454/28720
dc.identifier.volume2en_US
dc.identifier.wosWOS:000482143700031en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherAmer Medical Assocen_US
dc.relation.ispartofJama Network Openen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleComparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomyen_US
dc.typeArticleen_US

Dosyalar

Koleksiyon

WoS İndeksli Yayınlar Koleksiyonu
PubMed İndeksli Yayın Koleksiyonu