ArticleHistopathology2025
Genetic correlates of malignancy in TFE3-rearranged PEComa: a series of 14 cases.
Article in Histopathology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
1 citing paper in PubMed.
- YAP1::TFE3-rearranged inflammatory spindle cell PEComa of the anterior mediastinum in a 5-year-old child: first pediatric case.Virchows Archiv : an international journal of pathology · 2026Article
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5 authors.
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Abstract
aimsPerivascular epithelioid cell tumours (PEComas) show variable smooth muscle and melanocytic differentiation and mostly harbour mTOR pathway activation via TSC2 inactivation. Five-10% of sporadic PEComas instead harbour fusions involving TFE3, an vmTOR pathway target. Malignancy in TSC2/1-inactivated PEComa correlates with TP53, RB1 or ATRX inactivation. Here, we investigated genetic correlates of malignancy in TFE3-rearranged PEComa. METHODS AND
resultsFourteen TFE3-rearranged PEComas, confirmed by FISH and/or sequencing, occurred in 11 females (79%) and 3 males aged 9-64 years (median: 31.5 yr). Body sites were uterus (3 tumours), extremities (3), colon (2), nasal cavity (2), neck (1), retroperitoneum (1), bladder (1) and ovary (1). Nine tumours (64%) lacking cytologic atypia were diagnosed prospectively as benign, and five cytologically atypical tumours were diagnosed prospectively as malignant. By immunohistochemistry, tumours expressed SMA (6/13; 46%), HMB-45 (5/13; 38%), desmin (3/13; 23%) and melan-A (2/13; 15%), and not MITF (10 tumours), S-100 (7), SOX10 (4), pan-K (5) or EMA (3). By DNA sequencing, all nine benign tumours lacked complex copy number alterations (CNAs) or inactivation of TP53, ATRX or RB1. In contrast, three of four (75%) assessable malignant tumours showed complex CNAs, and only one of five malignant PEComas (20%) harboured TP53 inactivation. Among eight patients with follow-up (57%), all four benign PEComas neither recurred nor metastasized (median: 5.0 yr; range: 3.3-8.1 yr), while all four malignant tumours metastasized.
conclusionsWe conclude that malignant TFE3-rearranged PEComas frequently harbour complex CNAs, which could be of diagnostic utility. Malignant TFE3-rearranged PEComas lacked highly recurrent alterations in TP53, RB1 or ATRX.
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