Horm Metab Res 2019; 51(07): 403-413
DOI: 10.1055/a-0672-1266
Review

An Update on the Histology of Pheochromocytomas: How Does it Relate to Genetics?

Lindsey Oudijk
1   Department of Pathology, Erasmus MC University Medical Center, Rotterdam, The Netherlands
,
José Gaal
2   Department of Pathology, Isala Clinics, Zwolle, The Netherlands
,
Karen Koopman
2   Department of Pathology, Isala Clinics, Zwolle, The Netherlands
,
Ronald R. de Krijger
3   Department of Pathology, University Medical Center/Princess Maxima Center for Pediatric Oncology, Utrecht and Reinier de Graaf Hospital, Delft, The Netherlands
› Author Affiliations

Abstract

Pheochromocytomas are rare neuroendocrine tumors of the adrenal gland, whereas any extra-adrenal tumor with similar histology is designated as paraganglioma. These tumors have a very high rate of germline mutations in a large number of genes, up to 35% to 40%, frequently predisposing for other tumors as well. Therefore, they represent a phenomenal challenge for treating physicians. This review focuses on pheochromocytomas only, with special attention to gross and microscopic clues to the diagnosis of genetic syndromes, including the role of succinate dehydrogenase subunit A and subunit B immunohistochemistry as surrogate markers for genetic analysis in the field of succinate dehydrogenase subunit gene mutations.



Publication History

Received: 17 May 2018

Accepted: 02 August 2018

Article published online:
24 August 2018

Georg Thieme Verlag
Rüdigerstraße 14,70469 Stuttgart, Germany

 
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