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

Neumann, Hartmut P. H.; Tsoy, Uliana; Bancos, Irina; Amodru, Vincent; Walz, Martin K.; Tirosh, Amit; Kaur, Ravinder; McKenzie, Travis J.; Qi, Xiao-Ping; Bandgar, Tushar; Petrov, Roman; Yukina, Marina; Roslyakova, Anna; Horst-Schrivers, Anouk N A van der; Berends, Annika M A; Hoff, Ana O.; Castroneves, Luciana A.; Ferrara, Alfonso Massimiliano; Rizzati, Silvia; Mian, Caterina; Dvořáková, Šárka; Hasse-Lazar, Kornelia; Kvachenyuk, А.M.; Pęczkowska, Mariola; Loli, Paola; Erenler, Feyza; Krauß, Tobias; Almeida, Madson Q.; Liu, Longfei; Zhu, Fenlu; Sala, Monica; Wohllk, Nelson; Corssmit, Eleonora P M; Shafigullina, Zulfiya; Calissendorff, Jan; Grozinsky‐Glasberg, Simona; Kunavisarut, Tada; Schalin‐Jäntti, Camilla; Castinetti, Frédéric; Vlček, Petr; Beltsevich, D. G.; Egorov, V.; Schiavi, Francesca; Links, Thera P.; Lechan, Ronald M.; Bausch, Birke; Young, William F.; Eng, Charis

doi:10.1001/jamanetworkopen.2019.8898

Cited by 88 publications

(67 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A recent international retrospective population-based study conducted by 30 academic medical centers (including our center) showed that, compared to total adrenalectomy, the rate of PHEO recurrence after ASS was comparable, whereas the risk of postoperative adrenal insufficiency and steroid dependency was significantly lower (87 vs. 43%; P = 0.03) ( 90 ). These results indicated that ASS is a highly successful treatment approach of choice that can reduce the frequency of complications and should be considered for all MEN2-related PHEO, although ASS was considered as an alternative procedure to adrenalectomy in the ATA-2015 guidelines ( 11 , 90 , 91 ). There are several issues that should be considered when selecting ASS as a treatment option ( 2 , 11 , 57 , 90 ).…”

Section: P Strategies For Management Of Men2mentioning

confidence: 99%

5P Strategies for Management of Multiple Endocrine Neoplasia Type 2: A Paradigm of Precision Medicine

Ding

et al. 2020

Front. Endocrinol.

View full text Add to dashboard Cite

Multiple endocrine neoplasia type 2 (MEN2) is a neuroendocrine cancer syndrome characterized by medullary thyroid carcinoma, in combination or not with pheochromocytoma, hyperparathyroidism, and extra-endocrine features. MEN2 syndrome includes two clinically distinct forms subtyped as MEN2A and MEN2B. Nearly all MEN2 cases are caused by germline mutations of the RET proto-oncogene. In this review, we propose "5P" strategies for management of MEN2: prevention, prediction, personalization, psychological support, and participation, which could effectively improve clinical outcomes of patients. Based on RET mutations, MEN2 could be prevented through prenatal diagnosis or preimplantation genetic testing. Identification of pathogenic mutations in RET can enable early diagnosis of MEN2. Combining RET mutation testing with measurement of serum calcitonin, plasma or urinary metanephrine/normetanephrine, and serum parathyroid hormone levels could allow risk stratification and accurately prediction of MEN2 progression, thus facilitating implementation of personalized precision treatments to increase disease-free survival and overall survival. Furthermore, increased awareness of MEN2 is needed, which requires participation of physicians, patients, family members, and related organizations. Psychological support is also important for patients with MEN2 to promote comprehensive management of MEN2 symptoms. The "5P" strategies for management of MEN2 represent a typical clinical example of precision medicine. These strategies could effectively improve the health of MEN2 patient, and avoid adverse outcomes, including death and major morbidity, from MEN2.

show abstract

Section: P Strategies For Management Of Men2mentioning

confidence: 99%

5P Strategies for Management of Multiple Endocrine Neoplasia Type 2: A Paradigm of Precision Medicine

Ding

et al. 2020

Front. Endocrinol.

View full text Add to dashboard Cite

show abstract

“…In patients with hereditary PCCs, cortical sparing surgery should always be considered since there is frequently a high risk of bilateral PCCs in hereditary disease, and cortical-sparing surgery was not associated with decreased survival despite PCC recurrence in 13% of cases in a very recently published study [103].…”

Section: Therapymentioning

confidence: 99%

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

Nölting

Ullrich

Pietzsch

et al. 2019

Cancers

124

201

View full text Add to dashboard Cite

Pheochromocytomas and paragangliomas (PCC/PGLs) are rare, mostly catecholamine-producing neuroendocrine tumors of the adrenal gland (PCCs) or the extra-adrenal paraganglia (PGL). They can be separated into three different molecular clusters depending on their underlying gene mutations in any of the at least 20 known susceptibility genes: The pseudohypoxia-associated cluster 1, the kinase signaling-associated cluster 2, and the Wnt signaling-associated cluster 3. In addition to tumor size, location (adrenal vs. extra-adrenal), multiplicity, age of first diagnosis, and presence of metastatic disease (including tumor burden), other decisive factors for best clinical management of PCC/PGL include the underlying germline mutation. The above factors can impact the choice of different biomarkers and imaging modalities for PCC/PGL diagnosis, as well as screening for other neoplasms, staging, follow-up, and therapy options. This review provides a guide for practicing clinicians summarizing current management of PCC/PGL according to tumor size, location, age of first diagnosis, presence of metastases, and especially underlying mutations in the era of precision medicine.

show abstract

“…Bei Phäochromozytomen spielen, insbesondere bei Patienten mit einer genetischen Prädisposition, nebennierenparenchymerhaltende Eingriffe eine größere Rolle. Aktuell kann jedoch die „Cortical-sparing“-Variante [ 20 ] einer Adrenalektomie nicht als solche im EUROCRINE®-Register angegeben werden. Diese Resektionen finden sich unter „andere Nebenniereneingriffe“ wieder (Tab.…”

Section: Diskussionunclassified

EUROCRINE®: Nebennierenoperationen 2015 bis 2019 – überraschende erste Ergebnisse

Staubitz

Clérici

Riss

et al. 2020

Chirurg

View full text Add to dashboard Cite

Zusammenfassung Hintergrund Seit 2015 erfolgt in Europa mithilfe des EUROCRINE®-Registers eine systematische Dokumentation endokrin-chirurgischer Operationen. Ziel dieser ersten Auswertung war eine Darstellung der Versorgungsrealität für Nebenniereneingriffe in einem homogenen Versorgungsumfeld, entsprechend des deutschsprachigen Raums – bzw. des Präsenzgebiets der Chirurgischen Arbeitsgemeinschaft Endokrinologie (CAEK) der Deutschen Gesellschaft für Allgemein- und Viszeralchirurgie (DGAV) – einschließlich einer Analyse der Adhärenz zu geltenden Therapieempfehlungen. Methodik Es erfolgte eine deskriptive Analyse der präoperativen Diagnostik, der angewandten Operationstechniken sowie der zugrunde liegenden histologischen Entitäten der zwischen den Jahren 2015 und 2019 über EUROCRINE® in Deutschland, Österreich und der Schweiz dokumentierten Nebennierenoperationen. Ergebnisse In den insgesamt 21 teilnehmenden Kliniken des deutschsprachigen EUROCRINE®-Gebiets wurden 658 Operationen an Nebennieren durchgeführt. In 90 % erfolgten unilaterale, in 3 % bilaterale Adrenalektomien und in 7 % andere Resektionsverfahren. Die in 41 % der Operationen dokumentierte histologische Hauptdiagnose war das adrenokortikale Adenom. In 15 % lagen maligne Befunde zugrunde (einschließlich 6 % Nebennierenrindenkarzinome (ACC) und 8 % Nebennierenmetastasen). 23 % der Operationen erfolgten bei Phäochromozytomen. Diese wurden zu 82 % minimal-invasiv operiert, Nebennierenrindenkarzinome lediglich zu 28 % und Nebennierenmetastasen zu 66 %. Schlussfolgerung Überraschenderweise wurden nach Nebennierenadenomen und Phäochromozytomen an dritthäufigster Stelle Nebennierenmetastasen unterschiedlicher Primärtumoren reseziert. 28 % der ACC waren für minimal-invasive Techniken vorgesehen, wobei 20 % dieser Fälle eine Konversion zur offenen Operation erforderten. Die aktuelle Analyse deckte Diskrepanzen zwischen Versorgungsrealität und Leitlinienempfehlungen auf, aus denen sich zahlreiche Fragestellungen ergeben, welche nun in ein überarbeitetes EUROCRINE®-Modul zur Dokumentation von Nebennierenoperationen einfließen werden.

show abstract

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

Cited by 88 publications

References 31 publications

5P Strategies for Management of Multiple Endocrine Neoplasia Type 2: A Paradigm of Precision Medicine

5P Strategies for Management of Multiple Endocrine Neoplasia Type 2: A Paradigm of Precision Medicine

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

EUROCRINE®: Nebennierenoperationen 2015 bis 2019 – überraschende erste Ergebnisse

Contact Info

Product

Resources

About