title: Early onset of a nasal perivascular epithelioid cell neoplasm not related to tuberous sclerosis complex.
abstract: Perivascular epithelioid cell neoplasms are a group of rare tumours reported in various organs under a variety of designations. Such tumours are of interest primarily because of the distinctive morphology of their cell population and their immunoreactivity with melanocytic and myoid markers. There is a strong association between perivascular epithelioid cell neoplasms and tuberous sclerosis complex. Perivascular epithelioid cell neoplasms very rarely occur in the upper aero-digestive tract. To date only three cases of nasal perivascular epithelioid cell neoplasms have been reported in the literature. The present report refers to a 22-year old woman, without any stigmata of tuberous sclerosis complex, with early onset of a polypoid nasal mass with pathological and immunohistochemical features entirely compatible with those of a perivascular epithelioid cell neoplasm.
authors: Medical Genetics, University of Pavia, Pavia, Italy. simonegana@yahoo.it
authors: S
authors: S
authors: Gana
authors: P
authors: P
authors: Morbini
authors: G
authors: G
authors: Giourgos
authors: E
authors: E
authors: Matti
authors: F
authors: F
authors: Chu
authors: C
authors: C
authors: Danesino
authors: F
authors: F
authors: Pagella
mesh_terms: D005260:Female; D006801:Humans; D009669:Nose Neoplasms / Q000473:pathology*; D054973:Perivascular Epithelioid Cell Neoplasms / Q000473:pathology*; D014402:Tuberous Sclerosis; D055815:Young Adult