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Case Reports in Pathology
Volume 2017, Article ID 9236780, 5 pages
https://doi.org/10.1155/2017/9236780
Case Report

Primary Clear Cell Microcystic Adenoma of the Sinonasal Cavity: Pathological or Fortuitous Association?

1Department of Cellular Pathology, University Hospital Southampton NHS Foundation Trust, Southampton, UK
2Wessex Regional Genetics Laboratory, Salisbury NHS Foundation Trust, Salisbury, UK
3University of Southampton, Southampton, UK
4Wessex Clinical Genetics Service, University Hospitals Southampton NHS Foundation Trust, Southampton, UK

Correspondence should be addressed to Rosalin Cooper; ku.shn.shu@repooc.ahsinanilasor

Received 13 August 2016; Revised 14 December 2016; Accepted 15 January 2017; Published 5 February 2017

Academic Editor: Adriana Handra-Luca

Copyright © 2017 Rosalin Cooper et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Primary clear cell microcystic adenoma of the sinonasal cavity is rare. It has previously been described only as a VHL-associated tumour. Von Hippel-Lindau (VHL) syndrome is an inherited cancer syndrome characterised by an elevated risk of neoplasia including clear cell renal cell carcinoma (ccRCC), haemangioblastoma, and phaeochromocytoma. We describe the second reported case of a primary clear cell microcystic adenoma of the sinonasal cavity. The 39-year-old patient with VHL syndrome had previously undergone resection and ablation of ccRCC. He presented with epistaxis. Imaging demonstrated a mass in the ethmoid sinus. Initial clinical suspicion was of metastatic ccRCC. However, tumour morphology and immunoprofile were distinct from the previous ccRCC and supported a diagnosis of primary microcystic adenoma. Analysis of DNA extracted from sinonasal tumour tissue did not show loss of the wild-type allele at the VHL locus. Although this did not support tumour association with VHL disease, it was not possible to look for a loss-of-function mutation. The association of primary microcystic adenoma of the sinonasal cavity with VHL disease remains speculative. These lesions are benign but are likely to require regular surveillance. Such tumours may require repeated surgical excision.