TY - JOUR
TI - Outcomes of adrenal-sparing surgery or total adrenalectomy in
phaeochromocytoma associated with multiple endocrine neoplasia type 2:
an international retrospective population-based study
AU - Castinetti, Frederic
AU - Qi, Xiao-Ping
AU - Walz, Martin K.
AU - Maia, Ana
AU - Luiza
AU - Sanso, Gabriela
AU - Peczkowska, Mariola
AU - Hasse-Lazar,
AU - Kornelia
AU - Links, Thera P.
AU - Dvorakova, Sarka
AU - Toledo, Rodrigo A.
AU - and Mian, Caterina
AU - Bugalho, Maria Joao
AU - Wohllk, Nelson and
AU - Kollyukh, Oleg
AU - Canu, Letizia
AU - Loli, Paola
AU - Bergmann, Simona R.
AU - and Costa, Josefina Biarnes
AU - Makay, Ozer
AU - Patocs, Attila and
AU - Pfeifer, Marija
AU - Shah, Nalini S.
AU - Cuny, Thomas
AU - Brauckhoff,
AU - Michael
AU - Bausch, Birke
AU - von Dobschuetz, Ernst
AU - Letizia, Claudio
AU - and Barczynski, Marcin
AU - Alevizaki, Maria K.
AU - Czetwertynska,
AU - Malgorzata
AU - Ugurlu, M. Umit
AU - Valk, Gerlof
AU - Plukker, John T. M.
AU - and Sartorato, Paola
AU - Siqueira, Debora R.
AU - Barontini, Marta and
AU - Szperl, Malgorzata
AU - Jarzab, Barbara
AU - Verbeek, Hans H. G. and
AU - Zelinka, Tomas
AU - Vlcek, Petr
AU - Toledo, Sergio P. A.
AU - Coutinho,
AU - Flavia L.
AU - Mannelli, Massimo
AU - Recasens, Monica
AU - Demarquet, Lea
AU - and Petramala, Luigi
AU - Yaremchuk, Svetlana
AU - Zabolotnyi, Dmitry and
AU - Schiavi, Francesca
AU - Opocher, Giuseppe
AU - Racz, Karoly and
AU - Januszewicz, Andrzej
AU - Weryha, Georges
AU - Henry, Jean-Francois and
AU - Brue, Thierry
AU - Conte-Devolx, Bernard
AU - Eng, Charis
AU - Neumann,
AU - Hartmut P. H.
JO - The lancet oncology
PY - 2014
VL - 15
TODO - 6
SP - 648-655
PB - EXCERPTA MEDICA INC-ELSEVIER SCIENCE INC
SN - null
TODO - 10.1016/S1470-2045(14)70154-8
TODO - null
TODO - 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.
ER -