A Liver Transplant for Local Control in a Pediatric Patient with Metastatic TFE3-Associated Perivascular Epithelioid Cell Tumor (PEComa) to the Liver

Table 1

Graph demonstrating results.

Age (yr.)/sex	Location	Size (cm)	Margin status	Lymph node	Nuclear atypia	Mitosis/10 HPF	Prognosis^a	TFE3 IHC^b	TFE3 FISH^c	Reference
6/F	Appendix	1.3	NA	NA	Yes	0	NA	NA	NA	[10]
9/F	Rectum	3	Neg	Neg	No	Rare	14 MO NED	NA	NA	[11]
12/M	Duodenum	3.5	Neg	Neg	No	Low	24 MO NED	NA	NA	[12]
16/F	Transverse colon	2	NA	Neg	NA	0	24 MO NED	NA	NA	[13]
11/M	Sigmoid colon	1.2	Neg	Pos	No	Occasional	5 MO NED	NA	NA	[14]
11/M	Descending/sigmoid colon	3.5	Neg	NA	NA	Infrequent	NA	Pos	NA	[15]
16/F	Transverse colon	1.8	NA	Neg	NA	NA	41 MO NED	Pos	NA	[16]
14/F	Sigmoid colon	6.4	Neg	NA	NA	Rare	NA	Pos	Pos	[17]
15/F	Rectum	3.7	Neg	Pos	No	2	9 MO NED	NA	NA	[18]
16/F	Colon	NA	NA	NA	NA	NA	NA	Pos	Pos	[5]
17/F	Sigmoid colon	6	Neg	NA	No	NA	180 MO NED	NA	NA	[19]
7/M	Ascending colon	4	Neg	Neg	No	Low	26 MO NED	NA	NA	[20]
17/M	Rectum	3	Neg	Neg	No	<1	10 MO NED	NA	NA	[21]
16/F	Ascending colon	5	Neg	Neg	No	Up to 9	Liver met. after 3 MO	Pos	Pos	Our case

Abbreviation: F: female; FISH: fluorescent in situ hybridization; HPF: high power fields; IHC: immunohistochemistry; M: male; Met: metastasis; MO: month; NA: no data available; Neg: negative; NED: no evidence of disease; Pos: positive; Ref.: reference; yr.: year(s). ^aFollow-up since the first diagnosis. ^bTFE3 expression by immunohistochemistry. ^cGene rearrangement involving TFE3 (Xp11.23) by FISH.