title: Sclerosing polycystic adenosis of parotid gland: a unique report of two cases occurring in two sisters.
abstract: Sclerosing polycystic adenosis (SPA) of salivary glands is a tumorous lesion of salivary glands, with clinical presentation of a slow-growing mass characterized by a combination of histological features, some of which are reminiscent of mammary fibrocystic disease. SPA is mostly unifocal, but rarely may be multifocal and/or bilateral. Recurrences have been reported in up to 19% of cases. Although originally considered pseudoneoplastic, the occurrence of "dysplasia" and carcinoma in situ of ductal epithelium, and recent evidence of clonality suggest a possible neoplastic nature. Herein we describe, for the first time, two cases of SPA in two sisters (7 and 33 years old). The younger patient experienced multiple recurrences. This is the first report of familial occurrence of SPA, suggesting a possible genetic background.
authors.affiliation: University of Zagreb School of Medicine, Department of Pathology, University Hospital Dubrava, Zagreb, Croatia. Electronic address: spoma@mef.hr.
authors.forename: Spomenka
authors.initials: S
authors.lastname: Manojlović
authors.affiliation: University of Zagreb School of Medicine, Department of Maxillofacial Surgery, University Hospital Dubrava, Zagreb, Croatia.
authors.forename: Mišo
authors.initials: M
authors.lastname: Virag
authors.affiliation: University of Zagreb School of Medicine, Department of Maxillofacial Surgery, University Hospital Dubrava, Zagreb, Croatia.
authors.forename: Aleksandar
authors.initials: A
authors.lastname: Milenović
authors.affiliation: University of Zagreb School of Medicine, Croatia.
authors.forename: Luka
authors.initials: L
authors.lastname: Manojlović
authors.affiliation: University of Zagreb School of Medicine, Croatia.
authors.forename: Zrinko
authors.initials: Z
authors.lastname: Salek
authors.affiliation: Department of Pathology, Charles University in Prague, Faculty of Medicine in Plzen, Plzen, Czech Republic.
authors.forename: Alena
authors.initials: A
authors.lastname: Skálová
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D001706:Biopsy; D002648:Child; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007150:Immunohistochemistry; D009364:Neoplasm Recurrence, Local; D010306:Parotid Gland / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D010307:Parotid Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D010641:Phenotype; D012598:Sclerosis; D035781:Siblings; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor
keywords: Familial occurrence; Recurrence; Salivary gland; Sclerosing polycystic adenosis