Hepatic perivascular epithelioid cell tumor: Clinicopathological analysis of 26 cases with emphasis on disease management and prognosis

Table 1 Antibodies used in this study

Antigen	Clone	Dilution	Antigen retrieval	Localization
HMB-45	HMB-45	1:400	None	Cytoplasm
Melan-A	A103	1:200	Citrate buffer pressure cook	Cytoplasm
SMA	1A4	1:20000	None	Cytoplasm
Desmin	D33	1:500	None	Cytoplasm
S100 protein	Polyclonal	1:4000	Citrate buffer pressure cook	Cytoplasm/nucleus
Hepatocyte	OCH1E5	1:1000	Citrate buffer pressure cook	Cytoplasm
Vimentin	V9	1:200	Citrate buffer pressure cook	Cytoplasm
CD34	QBEnd/10	1:500	Citrate buffer pressure cook	Cell membrane
TFE-3	MRQ-0663	1:500	ETDA buffer pressure cook	Nucleus
Ki-67	MX006	1:200	Citrate buffer pressure cook	Nucleus

Table 2 Clinicopathological features of the 26 cases of hepatic PEComa

No.	Sex/age (yr)	Tumor location	Tumor size (cm)	First diagnosis	Treatment	Follow-up (mo) and prognosis
1	F/40	Left lobe	2.5	Left lobe occupying lesion	Left hepatic tumor simple resection	91, favorable prognosis
2	M/57	Left lobe	7.5	Left lobe occupying lesion	Left hepatic tumor simple resection	80, favorable prognosis
3	F/58	Left lobe	8.5	Left lobe occupying lesion	Left hepatic tumor simple resection	79, favorable prognosis
4	F/48	Right lobe	8.0	Right lobe occupying lesion	Right hepatic tumor simple resection	69, favorable prognosis
5	F/64	Right lobe	7.0	Right lobe occupying lesion	Right hepatic tumor simple resection	66, favorable prognosis
6	M/72	Right lobe	8.0	Right lobe occupying lesion	Right hepatic tumor simple resection	59, favorable prognosis
7	F/26	Right lobe	3.0	Right hepatic hamartoma	Extended hepatic lobe resection	55, favorable prognosis
8	M/47	Right lobe	6.5	Right lobe occupying lesion	mTOR inhibitor-sirolimus	51, favorable prognosis
9	F/47	Left lobe	5.5	Left lobe occupying lesion	Extended hepatic lobe resection	25, favorable prognosis
10	M/72	Right lobe	8.0	Right lobe occupying lesion	Extended right hepatic lobe resection	57, favorable prognosis
11	F/56	Right lobe	8.0	Right lobe occupying lesion	mTOR inhibitor-sirolimus	32, favorable prognosis
12	F/54	Right lobe	13.0	Left lobe occupying lesion	Extended left hepatic lobe resection	99, favorable prognosis
13	F/41	Caudate lobe	8.0	Caudate lobe occupying lesion	Caudate hepatic tumor simple resection	98, favorable prognosis
14	F/46	Left lobe	2.0	Left lobe occupying lesion	Extended left hepatic lobe resection	99, favorable prognosis
15	F/54	Right lobe	8.0	Right hepatic hemangioma	Extended Rright hepatic lobe resection	84, favorable prognosis
16	F/41	Caudate lobe	6.0	Caudate lobe occupying lesion	Extended caudate hepatic lobe resection	87, hepatic pain often occurs after discharge
17	M/45	Right lobe	0.5	Right hepatic hemangioma	Extended hepatic lobe resection	85, favorable prognosis
18	F/66	Right lobe	5.5	Right lobe occupying lesion	Extended hepatic lobe resection	59, favorable prognosis
19	F/43	Right lobe	2.8	Right lobe occupying lesion	Extended hepatic lobe resection	47, favorable prognosis
20	F/41	Left lobe	5.0	Left lobe occupying lesion	Extended hepatic lobe resection	49, reoperation for liver cancer in 2017
21	M/52	Left lobe	7.5	Left lobe occupying lesion	Left hepatic tumor simple resection	48, favorable prognosis
22	F/48	Right lobe	9.5	Right lobe occupying lesion	Extended right hepatic lobe resection	71, favorable prognosis
23	M/58	Left lobe	4.0	Left lobe occupying lesion	Left hepatic tumor simple resection	70, favorable prognosis
24	M/77	Left lobe	4.0	Left lobe occupying lesion	Extended left hepatic lobe resection	47, favorable prognosis
25	M/62	Left lobe	6.5	Left lobe occupying lesion	Extended left hepatic lobe resection	36, favorable prognosis
26	F/45	Left lobe	3.0	Left lobe occupying lesion	Extended left hepatic lobe resection	35, favorable prognosis

Table 3 Immunohistochemical features of the 26 cases of hepatic PEComa

Target protein	Positive cases (n)/total	% Positive
HMB45	25/26	96.2
Melan-A	23/26	88.5
SMA	22/26	83.6
Desmin	1/26*	3.8
S100	14/26	53.8
Hepatocyte	9/26	34.6
Vimentin	20/26	76.9
CD34	18/26	69.2
TFE3	0/26	0
Ki-67 (> 10%)	1/26	3.8

Weakly positive (1%-5%), only scattered cells.