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.