Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based study
Language English Country England, Great Britain Media print-electronic
Document type Journal Article, Observational Study, Research Support, Non-U.S. Gov't
PubMed
24745698
DOI
10.1016/s1470-2045(14)70154-8
PII: S1470-2045(14)70154-8
Knihovny.cz E-resources
- MeSH
- Adrenalectomy mortality MeSH
- Child MeSH
- Adult MeSH
- Pheochromocytoma etiology mortality surgery MeSH
- Kaplan-Meier Estimate MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Multiple Endocrine Neoplasia Type 2a complications mortality surgery MeSH
- Adrenal Gland Neoplasms etiology mortality surgery MeSH
- Disease-Free Survival MeSH
- Retrospective Studies MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Treatment Outcome MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Observational Study MeSH
- Research Support, Non-U.S. Gov't MeSH
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.
2nd Department of Internal Medicine Semmelweis University Budapest Hungary
Aix Marseille University Department of Endocrine Surgery La Timone Hospital Marseille France
Center for Endocrinological Investigations Hospital de Ninos R Gutierrez Buenos Aires Argentina
Department of Endocrine Surgery University of Bergen Bergen Norway
Department of Endocrinology Ospedale Niguarda Cà Granda Milan Italy
Department of Endocrinology University Hospital Nancy France
Department of Endocrinology University Medical Center Groningen University of Groningen Netherlands
Department of Endocrinology University Medical Center Ljubljana Ljubljana Slovenia
Department of Endocrinology University of Sao Paulo School of Medicine Sao Paulo Brazil
Department of General Surgery Breast and Endocrine Surgery Unit Marmara University Istanbul Turkey
Department of General Surgery Division of Endocrine Surgery Ege University Hospital Izmir Turkey
Department of Hypertension Institute of Cardiology Warsaw Poland
Department of Internal Medicine and Medical Specialties University La Sapienza Rome Italy
Department of Internal Medicine General Hospital Montebelluna Treviso Italy
Department of Internal Medicine University Medical Centre Utrecht Utrecht Netherlands
Department of Medicine University of Padova Padova Italy
Department of Molecular Endocrinology Institute of Endocrinology Prague Czech Republic
Department of Surgery and Center of Minimally Invasive Surgery Kliniken Essen Mitte Essen Germany
Department of Surgery University Medical Centre Groningen Netherlands
Endocrine Section Universidad de Chile Hospital del Salvador Santiago de Chile Chile
Familial Cancer Clinic and Oncoendocrinology Veneto Institute of Oncology IRCCS Padova Padova Italy
Hospital Universitari de Girona Gerencia Territorial Girona Institut Català de la Salut Girona Spain
Institute of Endocrinology Kiev Ukraine
Institute of Otolaryngology NAMS of Ukraine Kiev Ukraine
Operative Unit of the Endocrinology Department of Medicine University of Padova Padova Italy
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