title: TFE3-Expressing Perivascular Epithelioid Cell Neoplasm (PEComa) of the Sella Turcica.
abstract: We report a primary central nervous system (CNS) perivascular epithelioid cell tumor (PEComa) in a middle-aged female patient. The tumor occurred in suprasellar location with secondary extension into the sella turcica. The patient presented with intracranial hemorrhage and an altered level of consciousness. The tumor had morphologic features matching those of other previously described TFE3-translocated PEComas, including epithelioid morphology, diffuse and strong nuclear immunoreactivity for TFE3, and minimal staining with myoid markers. The TFE3 break-apart FISH testing showed a slight splitting of one of the TFE3 signals in 49.5 % of nuclei. This case illustrates that PEComas should be added to the growing list of mesenchymal tumors that can be encountered in the CNS and specifically in the vicinity of the pituitary gland. The recognition of this entity is of significance given their underlying pathogenesis and possible management implications.
authors.affiliation: Department of Pathology, University Health Network, Toronto, ON, Canada.|Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Martin D
authors.initials: MD
authors.lastname: Hyrcza
authors.affiliation: Department of Pathology, University Health Network, Toronto, ON, Canada.|Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.|Endocrine Oncology Site Group, The Princess Margaret Cancer Centre, Toronto, Ontario, Canada.
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Winer
authors.affiliation: Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.|Department of Pediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Mary
authors.initials: M
authors.lastname: Shago
authors.affiliation: Department of Neurosurgery, University Health Network and University of Toronto, Toronto, Ontario, Canada.
authors.forename: Karolyn
authors.initials: K
authors.lastname: Au
authors.affiliation: Endocrine Oncology Site Group, The Princess Margaret Cancer Centre, Toronto, Ontario, Canada.|Department of Neurosurgery, University Health Network and University of Toronto, Toronto, Ontario, Canada.
authors.forename: Gelareh
authors.initials: G
authors.lastname: Zadeh
authors.affiliation: Department of Pathology, University Health Network, Toronto, ON, Canada.|Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.|Endocrine Oncology Site Group, The Princess Margaret Cancer Centre, Toronto, Ontario, Canada.
authors.forename: Sylvia L
authors.initials: SL
authors.lastname: Asa
authors.affiliation: Department of Pathology, University Health Network, Toronto, ON, Canada. ozgur.mete2@uhn.ca.|Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada. ozgur.mete2@uhn.ca.|Endocrine Oncology Site Group, The Princess Margaret Cancer Centre, Toronto, Ontario, Canada. ozgur.mete2@uhn.ca.
authors.forename: Ozgur
authors.initials: O
authors.lastname: Mete
mesh_terms: D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000235:genetics*; D014408:Biomarkers, Tumor / Q000032:analysis; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D008875:Middle Aged; D054973:Perivascular Epithelioid Cell Neoplasms / Q000235:genetics / Q000473:pathology*; D012658:Sella Turcica / Q000473:pathology*; D014178:Translocation, Genetic
chemical_list: D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; D014408:Biomarkers, Tumor; C069203:TFE3 protein, human
keywords: FISH; Nonfunctioning sellar mass; PEComa; Pituitary adenoma; TFE3