@article{3161691,
    title = "Outcomes of adrenal-sparing surgery or total adrenalectomy in
phaeochromocytoma associated with multiple endocrine neoplasia type 2:
an international retrospective population-based study",
    author = "Castinetti, Frederic and Qi, Xiao-Ping and Walz, Martin K. and Maia, Ana and Luiza and Sanso, Gabriela and Peczkowska, Mariola and Hasse-Lazar, and Kornelia and Links, Thera P. and Dvorakova, Sarka and Toledo, Rodrigo A. and and Mian, Caterina and Bugalho, Maria Joao and Wohllk, Nelson and and Kollyukh, Oleg and Canu, Letizia and Loli, Paola and Bergmann, Simona R. and and Costa, Josefina Biarnes and Makay, Ozer and Patocs, Attila and and Pfeifer, Marija and Shah, Nalini S. and Cuny, Thomas and Brauckhoff, and Michael and Bausch, Birke and von Dobschuetz, Ernst and Letizia, Claudio and and Barczynski, Marcin and Alevizaki, Maria K. and Czetwertynska, and Malgorzata and Ugurlu, M. Umit and Valk, Gerlof and Plukker, John T. M. and and Sartorato, Paola and Siqueira, Debora R. and Barontini, Marta and and Szperl, Malgorzata and Jarzab, Barbara and Verbeek, Hans H. G. and and Zelinka, Tomas and Vlcek, Petr and Toledo, Sergio P. A. and Coutinho, and Flavia L. and Mannelli, Massimo and Recasens, Monica and Demarquet, Lea and and Petramala, Luigi and Yaremchuk, Svetlana and Zabolotnyi, Dmitry and and Schiavi, Francesca and Opocher, Giuseppe and Racz, Karoly and and Januszewicz, Andrzej and Weryha, Georges and Henry, Jean-Francois and and Brue, Thierry and Conte-Devolx, Bernard and Eng, Charis and Neumann, and Hartmut P. H.",
    journal = "The lancet oncology",
    year = "2014",
    volume = "15",
    number = "6",
    pages = "648-655",
    publisher = "EXCERPTA MEDICA INC-ELSEVIER SCIENCE INC",
    doi = "10.1016/S1470-2045(14)70154-8",
    abstract = "Background The prevention of medullary thyroid cancer in patients with
multiple endocrine neoplasia type 2 syndrome has demonstrated the
ability of molecular diagnosis and prophylactic surgery to improve
patient outcomes. However, the other major neoplasia associated with
multiple endocrine neoplasia type 2, phaeochromocytoma, is not as well
characterised in terms of occurrence and treatment outcomes. In this
study, we aimed to systematically characterise the outcomes of
management of phaeochromocytoma associated with multiple endocrine
neoplasia type 2.
Methods This multinational observational retrospective population-based
study compiled data on patients with multiple endocrine neoplasia type 2
from 30 academic medical centres across Europe, the Americas, and Asia.
Patients were included if they were carriers of germline pathogenic
mutations of the RET gene, or were first-degree relatives with
histologically proven medullary thyroid cancer and phaeochromocytoma. We
gathered clinical information about patients’ RET genotype, type of
treatment for phaeochromocytoma (ie, unilateral or bilateral operations
as adrenalectomy or adrenal-sparing surgery, and as open or endoscopic
operations), and postoperative outcomes (adrenal function, malignancy,
and death). The type of surgery was decided by each investigator and the
timing of surgery was patient driven. The primary aim of our analysis
was to compare disease-free survival after either adrenal-sparing
surgery or adrenalectomy.
Findings 1210 patients with multiple endocrine neoplasia type 2 were
included in our database, 563 of whom had phaeochromocytoma. Treatment
was adrenalectomy in 438 (79%) of 552 operated patients, and
adrenal-sparing surgery in 114 (21%). Phaeochromocytoma recurrence
occurred in four (3%) of 153 of the operated glands after
adrenal-sparing surgery after 6-13 years, compared with 11 (2%) of 717
glands operated by adrenalectomy (p=0.57). Postoperative adrenal
insufficiency or steroid dependency developed in 292 (86%) of 339
patients with bilateral phaeochromocytoma who underwent surgery.
However, 47 (57%) of 82 patients with bilateral phaeochromocytoma who
underwent adrenal-sparing surgery did not become steroid dependent.
Interpretation The treatment of multiple endocrine neoplasia type
2-related phaeochromocytoma continues to rely on adrenalectomies with
their associated Addisonian-like complications and consequent lifelong
dependency on steroids. Adrenal-sparing surgery, a highly successful
treatment option in experienced centres, should be the surgical approach
of choice to reduce these complications."
}