title: An analysis of five clear cell papillary cystadenomas of mesosalpinx and broad ligament: four associated with von Hippel-Lindau disease and one aggressive sporadic type.
abstract: OBJECTIVE
  Clear cell papillary cystadenoma (CCPC) is associated with von Hippel-Lindau disease (VHLD), but rarely involves mesosalpinx and broad ligament (M/BL). This study provides new data about its behaviour and immunophenotype.
  
  
  RESULTS
  We performed an analysis of four benign cases of CCPC of M/BL with either characteristic clinical features or genetic markers [loss of heterozygosity (LOH)] of VHLD in patients ranging from 24 to 36 years and a sporadic case in a 52-year-old presenting with peritoneal metastases. All CCPCs were papillary but had solid and tubular areas. Haemorrhage, thrombosis and scarring were constant features and related to an unusual pattern of sub-epithelial vascularity. All clear or oxyphilic cells co-expressed cytokeratin 7 (CK7), CAM5.2 and vimentin, with strong apical CD10 and nuclear paired box gene 2 (PAX2) immunoreactivity. Three cases also showed positivity for VHL40, epithelial membrane antigen (EMA), Wilms' tumour suppressor gene (WT-1) and cancer antigen 125 (CA125) but only one expressed renal cell carcinoma (RCC) antigen. Vascular plexus overexpressed nuclear and cytoplasmic WT-1.
  
  
  CONCLUSIONS
  The VHLD-associated cases appeared to be benign, but the sporadic case exhibited a low malignant potential. CCPCs show histological and immunophenotypical similarities with the recently reported clear cell papillary RCC, although the previously unreported apical CD10 and nuclear PAX2 expression may be related to their mesonephric origin. CCPC has a distinctive sub-epithelial vascular pattern that is consistent with its pathogenesis.
authors.affiliation: Departments of Pathology, Hospital Universitario San Cecilio, Granada, Spain. fnogales@ugr.es
authors.forename: Francisco F
authors.initials: FF
authors.lastname: Nogales
authors.forename: Pablo
authors.initials: P
authors.lastname: Goyenaga
authors.forename: Ovidiu
authors.initials: O
authors.lastname: Preda
authors.forename: Alina
authors.initials: A
authors.lastname: Nicolae
authors.forename: Begoña
authors.initials: B
authors.lastname: Vieites
authors.forename: Maria Carmen
authors.initials: MC
authors.lastname: Ruiz-Marcellan
authors.forename: Alberto
authors.initials: A
authors.lastname: Pedrosa
authors.forename: Maria J
authors.initials: MJ
authors.lastname: Merino
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D001956:Broad Ligament / Q000378:metabolism / Q000473:pathology*; D018292:Cystadenoma, Papillary / Q000150:complications / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D005185:Fallopian Tube Neoplasms / Q000150:complications / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D005187:Fallopian Tubes / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D019656:Loss of Heterozygosity; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D014594:Uterine Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000235:genetics; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000235:genetics / Q000378:metabolism / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; D051794:Von Hippel-Lindau Tumor Suppressor Protein; C497446:VHL protein, human