Undifferentiated liver embryonal sarcoma in adults: A report of four cases and literature review

Table 1 Primary antibodies used in this study

Antibody	Clone	Source	Dilution
Vimentin (M)	Vim3B4	Dako (Glostrup, Denmark)	1:100
α-1-antitrypsin (P)		Changdao (Shanghai, China)	1:100
Cytokeratin 18 (M)	DC10	Dako (Glostrup, Denmark)	1:25
Cytokeratin 19 (M)	RCK108	Dako (Glostrup, Denmark)	1:50
CD68 (M)	PG-M1	Changdao (Shanghai, China)	1:50
CD56 (M)	123C3	Dako (Glostrup, Denmark)	1:50
Desmin (M)	D33	Dako (Glostrup, Denmark)	1:50
α-SMA (M)	1A4	Dako (Glostrup, Denmark)	1:50
Myoglobin (M)	MYO18	Dako (Glostrup, Denmark)	1:50
CD117 (P)		Changdao (Shanghai, China)	1:50
S-100 (M)	4C4.9	Changdao (Shanghai, China)	1:200
CD34 (M)	QBEnd10	Dako (Glostrup, Denmark)	1:25
Melanosome (M)	HMB-45	Dako (Glostrup, Denmark)	1:25
Hep Par 1 (M)	OCH1E5	Dako (Glostrup, Denmark)	1:50
AFP (M)	AF04	Changdao (Shanghai, China)	1:100
HBsAg (M)	3E7	Dako (Glostrup, Denmark)	1:50
p53 (M)	DO-7	Dako (Glostrup, Denmark)	1:50
pCEA (P)		Changdao (Shanghai, China)	1:200
mCEA (M)	COL-1	Changdao (Shanghai, China)	1:50
EMA (M)	E29	Dako (Glostrup, Denmark)	1:50
NSE (M)	E27	Changdao (Shanghai, China)	1:50
Chromogranin A (M)	DAK-A3	Dako (Glostrup, Denmark)	1:100
Synaptophysin (M)	SY38	Dako (Glostrup, Denmark)	1:20
Ki67 (M)	MIB-1	Dako (Glostrup, Denmark)	1:100

M: Monoclonal; P: Polyclonal; AFP: α-fetoprotein; HBsAg: Hepatitis B virus surface antigen; pCEA: Polyclonal carcinoembryonic antigen; mCEA: Monoclonal carcinoembryonic antigen; EMA: Epithelial membrane antigen; NSE: Neuron specific enolase; SMA: Smooth muscle actin.

Table 2 Clinical features of undifferentiated liver embryonal sarcoma in adults over the age of 30 years

No.	Ref.	Yr	Sex	Age (yr)	Symptoms and signs	Laboratory findings	Location	Size (cm)	Surgery	Adjuvanttreatment	Recurrence	Follow-up
1	Esposito et al[6]	1977	M	36	RUQA pain, hepatomegaly, jaundice	↑Bil, AST, ALT	R + L	NA	Liver biopsy	No	NA	DOD 2 mo
2	Tanner et al[7]	1978	F	66	RUQA pain, vomiting, diarrhea, weight loss, fever, hepatomegaly	↑ALP	R	NA	HA ligation	No/5-FU	v	AWD 3 yr
3	Chang et al[8]	1983	F	55	RUQA pain, weight loss, diarrhea, hepatomegaly	↑ALT	L	10 × 10 × 8	Left lob	No/CAV	No	ANED 1 mo
4	Ellis et al[9]	1983	F	86	RUQA pain and mass	Normal	R	18 × 12 × 12	Wedge resec	No	Yes	DOD 8 wk
5	Forbes et al[10]	1987	M	69	RUQA pain, nausea, weight loss, hepatomegaly	Normal	NA	NA	No	RT/RT	NA	DOD 10 mo
6	Forbes et al[10]	1987	F	49	RUQA pain, nausea, weight loss, hepatomegaly	NA	NA	NA	No	No	NA	NA
7	Zornig et al[11]	1992	M	42	RUQA pain	Normal	R	8	Biseg	adm + ifs	No	ANED 30 mo
8	Zaheer et al[12]	1994	F	44	RUQA pain and mass, weight loss, fever	↑AFP	R	10	Liver biopsy	adm	NA	DOD 1 wk
9	Grazi et al[13]	1996	F	60	Dyspepsia, RUQA mass	Normal	R	22	Rx Hep	No	Yes	DOD 11 mo
10	Nishio et al[3]	2003	F	49	RUQA pain	NA	R	14 × 8 × 8	Rx lob	No	Yes	DOD 29 mo
11	Nishio et al[3]	2003	M	62	RUQA pain	NA	L	10 × 9 × 7	Left lob	No	No	ANED 10 mo
12	Lepreux et al[14]	2005	F	51	RUQA pain	↑AFP	R	NA	No	VAC	NA	Dead 2 mo
13	Lepreux et al[14]	2005	F	49	RUQA pain	Normal	L	NA	Left lob	ifs + dtic + adm	No	ANED 6 mo
14	Agaram et al[15]	2006	F	33	NA	NA	NA	14.5	NA	NA	No	ANED 6 mo
15	Agaram et al[15]	2006	F	50	NA	NA	NA	17	NA	NA	No	ANED 8 mo
16	Scudiere et al[4]	2006	F	51	Pneumonia	↑AST, ALT	R	25 × 19 × 8.5	Rx lob	NA	NA	NA
17	Ma et al[16]	2008	F	61	RUQA pain	Normal	R	12 × 9 × 8	Rx hep	NA	NA	DOD 8 mo
18	Yang et al[17]	2009	M	46	RUQA pain and fever	↑AST, ALT, GGT	R	6.2 × 5.9 × 5.4	Rx hep	NA	NA	NA
19	Yang et al[17]	2009	M	54	RUQA pain and fever	↑AST, ALT, GGT	R	13 × 11.7 × 12.4	Rx hep	NA	NA	NA
20	Xu et al[18]	2010	F	36	RUQA pain	Normal	L	30 × 25 × 15	Rx hep	NA	NA	NA
21	Present case 1		M	63	RUQA pain and fever	↑AKP	R	15 × 15 × 20	Rx hep	NA	Yes	DOD 18 mo
22	Present case 2		M	39	weight lose and fever	↑AKP	L	16 × 13 × 9	Left lob	TACE	Yes	DOD 32 mo
23	Present case 3		M	56	RUQA pain	↑AKP, ALT, AST, GGT	R	12.5 × 11.5	Rx hep	TACE	Yes	DOD 28 mo
24	Present case 4		F	43	RUQA pain	↑AKP	R	14 × 12 × 10	Rx hep	NA	Yes	DOD 24 mo

R: Right; L: Left; NA: Not available; RUQA: Right upper quadrant abdominal; Rx hep: Right hepatectomy; Rx lob: Right lobectomy; Biseg: Bisegmentectomy; CAV: Cyclophosphamide + doxorubicin + vincristine; VAC: Vincristine + actinomycin D + cyclophosphamide; ifs: Ifosfamide; adm: Adriamycin; dtic: Dacarbazine; DOD: Dead of disease; AWD: Alive with disease; ANED: Alive with no evidence of disease; AKP: Alkaline phosphatase; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; GGT: γ-glutamyl transpeptidase; AFP: α-fetoprotein; RT: Radiation therapy; ALP: Alkaline phosphatase; HA: Hepatectomy.

Table 3 Immunohistochemical staining of undifferentiated liver embryonal sarcoma tissue in 4 adults

Antibody	Case 1	Case 2	Case 3	Case 4
				Primary	Recurrent
Age (yr)/sex	63/M	39/M	56/M	43/F	44/F
Vimentin	+	+	+	+	+
α-1-antitrypsin	+	+	+	+	+
Cytokeratin 18	+F	+F	-	+F	+F
Cytokeratin 19	+F	+F	-	+F	+F
CD68	+	+	+	+	+
CD56	+	+	+	+	+
Desmin	-	+F	+F	-	+F
α-SMA	-	-	-	-	+F
Myoglobin	-	-	-	-	+F
CD117	-	-	-	-	-
S-100	-	-	-	-	+F
CD34	-	-	-	-	+F
HMB45	-	-	-	-	-
Hep Par 1	-	-	-	-	-
AFP	-	-	-	-	-
HBsAg	-	-	-	-	-
p53	-	-	+	+	+
pCEA	-	-	-	-	-
mCEA	-	-	-	-	-
EMA	-	-	-	-	-
NSE	-	-	-	-	-
Chromogranin A	-	-	-	-	-
Synaptophysin	-	-	-	-	-
Ki67	60%	50%	45%	40%	65%

+F: Focal staining; AFP: α-fetoprotein; SMA: Smooth muscle actin; HBsAg: Hepatitis B virus surface antigen; EMA: Epithelial membrane antigen; NSE: Neuron specific enolase; pCEA: Polyclonal carcinoembryonic antigen; mCEA: Monoclonal carcinoembryonic antigen.