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F van Nederveen, Pathology, Erasmus MC, Josephine Nefkens Institute, Rotterdam, Netherlands
E Korpershoek, Pathology, ErasmusMC, Josephine Nefkens Institute, Rotterdam, Netherlands
R deLeeuw, Department of Cancer Genetics, British Columbia Cancer Research Centre, Vancouver, British Columbia, Canada
A Verhofstad, Pathology, Radboud University Nijmegen Medical Center, Nijmegen, Netherlands
J Lenders, Internal medicine, UMC Radboud Nijmegen, Nijmegen, Netherlands
W Dinjens, Pathology, ErasmusMC, Josephine Nefkens Institute, Rotterdam, Netherlands
W Lam, Department of Cancer Genetics, British Columbia Cancer Research Centre, Vancouver, British Columbia, Canada
R de Krijger, Pathology, Erasmus MC, Josephine Nefkens Institute, Rotterdam, Netherlands

Correspondence: Francien van Nederveen, Email: f.vannederveen{at}erasmusmc.nl

Abstract

Pheochromocytomas (PCC) are catecholamine-producing tumors arising from the adrenal medulla that occur either sporadically or in the context of hereditary cancer syndromes, such as multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau disease (VHL), neurofibromatosis type 1, and the PCC-paraganglioma (PGL) syndrome. Conventional comparative genomic hybridization (CGH) studies have shown loss of 1p and 3q in the majority of sporadic and MEN2-related PCC, and 3p and 11p loss in VHL-related PCC. The development of a submegabase tiling resolution array enabled us to perform a genome-wide high-resolution analysis of 36 sporadic benign PCC. The results show that there are two distinct patterns of abnormalities in these sporadic PCC, one consisting of loss of 1p with or without concomitant 3q loss in 20/36 cases (56%), the other characterized by loss of 3p with or without concomitant 11p loss in 11/36 (31%). In addition, we found loss of chromosome 22q at high frequency (35%), as well as the novel finding of high frequency chromosome 21q loss (21%). We conclude that there appear to be two subgroups of benign sporadic PCC, one of which has a pattern of chromosomal abnormalities that is comparable to PCC from patients with MEN2 and the other that is comparable to the PCC that arise in patients with VHL disease. In addition, genes on 21q and 22q might play a more important role in PCC pathogenesis than had been assumed thus far.

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				J. Gaal, F. H. van Nederveen, Z. Erlic, E. Korpershoek, R. Oldenburg, C. C. Boedeker, U. Kontny, H. P. Neumann, W. N. M. Dinjens, and R. R. de Krijger Parasympathetic Paragangliomas Are Part of the Von Hippel-Lindau Syndrome J. Clin. Endocrinol. Metab., November 1, 2009; 94(11): 4367 - 4371. [Abstract] [Full Text] [PDF]