TFE3-Expressing Perivascular Epithelioid Cell Neoplasm (PEComa) of the Sella Turcica

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• 作者：Martin D. Hyrcza ; Daniel A. Winer ; Mary Shago ; Karolyn Au…
• 关键词：Pituitary adenoma ; PEComa ; TFE3 ; FISH ; Nonfunctioning sellar mass
• 刊名：Endocrine Pathology
• 出版年：2017
• 出版时间：March 2017
• 年：2017
• 卷：28
• 期：1
• 页码：22-26
• 全文大小：
• 刊物主题：Pathology; Endocrinology; Oncology;
• 出版者：Springer US
• ISSN：1559-0097
• 卷排序：28

文摘

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.