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Extent of surgery for phaeochromocytomas in the genomic era
Linköping University, Department of Clinical and Experimental Medicine, Division of Cell Biology. Linköping University, Faculty of Medicine and Health Sciences.
Linköping University, Department of Clinical and Experimental Medicine, Division of Cell Biology. Linköping University, Faculty of Medicine and Health Sciences. Region Östergötland, Center for Diagnostics, Clinical genetics.
Linköping University, Department of Clinical and Experimental Medicine, Division of Surgery, Orthopedics and Oncology. Linköping University, Faculty of Medicine and Health Sciences. Region Östergötland, Center for Surgery, Orthopaedics and Cancer Treatment, Department of Surgery in Linköping.
2018 (English)In: British Journal of Surgery, ISSN 0007-1323, E-ISSN 1365-2168, Vol. 105, no 2, p. E84-E98Article, review/survey (Refereed) Published
Abstract [en]

Background

Germline mutations are present in 20–30 per cent of patients with phaeochromocytoma. For patients who develop bilateral disease, complete removal of both adrenal glands (total adrenalectomy) will result in lifelong adrenal insufficiency with an increased risk of death from adrenal crisis. Unilateral/bilateral adrenal‐sparing surgery (subtotal adrenalectomy) offers preservation of cortical function and independence from steroids, but leaves the adrenal medulla in situ and thus at risk of developing new and possibly malignant disease. Here, present knowledge about how tumour genotype relates to clinical behaviour is reviewed, and application of this knowledge when choosing the extent of adrenalectomy is discussed.

Methods

A literature review was undertaken of the penetrance of the different genotypes in phaeochromocytomas, the frequency of bilateral disease and malignancy, and the underlying pathophysiological mechanisms, with emphasis on explaining the clinical phenotypes of phaeochromocytomas and their associated syndromes.

Results

Patients with bilateral phaeochromocytomas most often have multiple endocrine neoplasia type 2 (MEN2) or von Hippel–Lindau disease (VHL) with high‐penetrance mutations for benign disease, whereas patients with mutations in the genes encoding SDHB (succinate dehydrogenase subunit B) or MAX (myelocytomatosis viral proto‐oncogene homologue‐associated factor X) are at increased risk of malignancy.

Conclusion

Adrenal‐sparing surgery should be the standard approach for patients who have already been diagnosed with MEN2 or VHL when operating on the first side, whereas complete removal of the affected adrenal gland(s) is generally recommended for patients with SDHB or MAX germline mutations. Routine assessment of a patient's genotype, even after the first operation, can be crucial for adopting an appropriate strategy for follow‐up and future surgery.

Place, publisher, year, edition, pages
John Wiley & Sons, 2018. Vol. 105, no 2, p. E84-E98
National Category
Surgery
Identifiers
URN: urn:nbn:se:liu:diva-147443DOI: 10.1002/bjs.10744ISI: 000429002100009PubMedID: 29341163Scopus ID: 2-s2.0-85040740402OAI: oai:DiVA.org:liu-147443DiVA, id: diva2:1206337
Available from: 2018-05-16 Created: 2018-05-16 Last updated: 2019-05-01Bibliographically approved

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Rossitti, HugoSöderkvist, PeterGimm, Oliver
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Division of Cell BiologyFaculty of Medicine and Health SciencesClinical geneticsDivision of Surgery, Orthopedics and OncologyDepartment of Surgery in Linköping
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British Journal of Surgery
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