Poggio JL et al44 Poggio JL, Nagorney DM, Nascimento AG, Rowland C, Kay P, Young RM, et al. Surgical treatment of adult primary hepatic sarcoma. Br J Surg 2000; 87(11):1500-5. (2000) |
13 ♂ 7 ♀ |
56 |
20 |
Abdominal mass: 10 Pain in RUQ: 10 Weight loss: 8 Fever: 8 Bleeding: Ø |
Leiomyosarcoma: 5 Malignant fibrous histiocytoma: 4 Angiosarcoma: 3 Epithelioid hemangioendothelioma: 3 Carcinosarcoma: 2 Embryonic: 1 Liposarcoma: 1 Fibrosarcoma: 1 |
Resection R0: 19 R2: 1 TX: 1 QT: 6 (Ifosfamide, Doxorubicin, Etoposide) |
Worse prognosis: Poorly differentiated (5y: 18%) |
1y: 79% 3y: 37% 5y: 37% |
Molina E et al88 Molina E, Hernandez A. Clinical manifestations of primary hepatic angiosarcoma. Dig Dis Sci. 2003;48(4):677-82. (2003) |
4 ♂ 1 ♀ |
53 |
5 |
Abdominal mass: 1 Pain in RUQ: 4 Weight loss: 1 Fever: 2 Bleeding: 2 |
Angiosarcoma |
Surgery + Chemo: 2 Surgery: 1 Biopsy: 2 |
|
Average 6 months |
Almogy G et al22 Almogy G, Lieberman S, Gips M, Pappo O, Edden Y, Jurim O, et al. Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre. Eur J Surg Oncol. 2004;30(4):421-7. (2004) |
6 ♂ 2 ♀ |
52 |
8 |
Pain in RUQ: 50% Asymptomatic: 25% Fever: 37.5% Bleeding: 25% (angiosarcoma) |
Angiosarcoma: 2 Leiomyosarcoma: 2 Embryonic: 2 Epithelioid hemangioendothelioma: 1 Neural sheath sarcoma: 1 |
Resection R0: 3 RXT: 2 QT: 5 TACE: 2 |
Worst prognosis: Positive margins |
2 patients without cancer returned at 47 mo. The others died within a year. |
Weitz J et al66 Weitz J, Klimstra DS, Cymes K, Jarnagin WR, D'Angelica M, La Quaglia MP, et al. Management of primary liver sarcomas. Cancer. 2007;109(7):1391-6. (2007) |
14 ♂ 16 ♀ |
52 |
30 |
Pain in RUQ: 19 (63%) Asymptomatic: 6 |
Epithelioid hemangioendothelioma: 10 Embryonic: 5 Angiosarcoma: 5 Malignant fibrous histiocytoma: 1 Leiomyosarcoma: 2 Others: 7 |
Resection R0: 11 R1 & R2: 13 |
Worse prognosis: Poorly differentiated Resections R1 or R2. |
Angiosarcoma Low average survival (11 months) R0: 64% (5 y) R1 or R2: 0% (3y) |
Li Y et al77 Li Y, Akbari E, Tretiakova MS, Hart J, Akbari M, Urbanski SJ, et al. Primary Hepatic Malignant Fibrous Histiocytoma. Am J Surg Pathol. 2008; 32(8):1144-58. (2008) |
21 ♂ 13 ♀ |
58 |
7 cases + 27 reviewed cases |
Abdominal mass: 0/7 Pain: 6/7 Weight loss: 4/7 Fever: 1/7 Bleeding: 0/7 |
Malignant fibrous histiocytoma |
Surgery: 4/7 & 20/27 TX:1/27 |
|
Average survival - 4 months, 6 cases:>2y |
Park YS et al1212 Park YS, Kim JH, Kim KW, Lee IS, Yoon HK, Ko GY, et al. Primary hepatic angiosarcoma: imaging findings and palliative treatment with transcatheter arterial chemoembolization or embolization. Clin Radiol. 2009;64(8):779-85. (2009) |
5 ♂ 1 ♀ |
52 |
6 |
Abdominal mass: 2 Pain in RUQ: 4 Weight loss: 0/7 Fever- 1 Bleeding: 3 |
Angiosarcoma |
TACE: 4 TAE: 2 |
|
Responsive to TACE: 8-12 months Non-responsive: 2 months |
Matthaei N et al55 Matthaei H, Krieg A, Schmelzle M, Boelke E, Poremba C, Rogiers X, et al. Long-term survival after surgery for primary hepatic sarcoma in adults. Arch Surg. 2009; 144(4):339-44. (2009) |
14 ♂ 8 ♀ |
54 |
22 |
Abdominal mass: 2 Pain in RUQ: 3 Weight loss: 2 Incidental finding: 15 |
Leiomyosarcoma: 7 Rhabdomyosarcoma: 5 Angiosarcoma: 5 Fibrous malignant histiocytoma: 1 Others: 4 |
All surgical QT: 8 (Cyclophosphamide Doxorubicin Etoposide and / or ifosfamide) |
Worse prognosis: Angiosarcoma |
Average survival 153 months 2y: 85.2% 3y: 70.2% 5y: 65.2% |
Zhou Y et al1010 Zhou YM, Li B, Yin ZM, Xu F, Wang B, Xu W, et al. Results of hepatic resection for primary hepatic angiosarcoma in adults. Med Sci Monit. 2010;16(2):61-66. (2010) |
5 ♂ 1 ♀ |
55 |
6 |
Abdominal mass: 1 Pain in RUQ: 4 Weight loss: 4 Asymptomatic: 1 Fever: 0/7 Bleeding: 0/7 |
Angiosarcoma |
Resection R0: 5 TACE: 5 (Lipiodol + adriamycin + carboplatin) |
|
5 recurrences within 2 years. |
Chi M et al3 (2012) |
60 ♂ 49 ♀ |
58 |
109 |
Abdominal mass: 56.8% Pain in RUQ: 50.5% Weight loss: 34.7% Fever: 12.6% Bleeding: 7.4% |
Leiomyosarcoma |
Surgery: 61% QT: 13.7% TX : 5.3% RXT: 2.1% |
|
Average survival 19 months 1y: 100%, 2y: 41.1% 5y: 14.5% |
Duan XF et al1111 Duan XF, Li Q. Primary hepatic angiosarcoma: A retrospective analysis of 6 cases. J Dig Dis. 2012;13(7):381-5. (2012) |
5 ♂ 1 ♀ |
49 |
6 |
Abdominal mass: 1 Pain in RUQ: 2 Weight loss: 1 Fever: 2 Bleeding: 0/7 |
Angiosarcoma |
Resection R0: 5 R2: 1 QT: 1 |
|
R0: 1y: 100% 3y: 80% 5y: 40% |
Zheng Y et al99 Zheng Y, Zhang X, Zhang J, Hui Z, Du W, Li R, et al. Primary hepatic angiosarcoma and potential treatment options. J Gastroenterol Hepatol. 2014;29(5):906-11 (2014) |
49 ♂ 15 ♀ |
59 |
64 |
Rupture 26.6% |
Angiosarcoma |
Surgery: 30 Palliative: 15 TACE: 10 TX: 5 QT: 4 |
|
Average survival: 5 months Surgery + QT: 17 months |
Lin YH et al11 Lin YH, Lin CC, Concejero AM, Yong CC, Kuo FY, Wang CC. Surgical experience of adult primary hepatic sarcomas. World J Surg Oncol. 2015;13:87. (2015) |
8 ♂ 5 ♀ |
48 |
13 |
Pain in RUQ: 9 Anemia: 7 Thrombocytopenia: 2 |
Angiosarcoma: 6 Undifferentiated Sarcoma: 4 Epithelioid Hemangioendothelioma: 1 Embryonic sarcoma: 1 Leiomyosarcoma: 1 |
Surgery + QT: 4 Surgery: 4 TAE + Surgery: 3 TX: 2 Resection: R0: 9 R1: 1 |
Non- angiosarcoma longer survival than angiosarcoma: 1y: 85.7% x 33.3%; 3y: 71.4% x 16.7%; 5y: 57.1% x 0% |
Average survival: 1y: 61.5%, 2y: 53.8%, 5y: 30.8%; Surgical patients: 1y: 72.7%, 2y: 63.6%, 5y: 36.4% |
Konstantinidis et al1515 Konstantinidis IT, Nota C, Jutric Z, Ituarte P, Chow W, Chu P, et al. Primary liver sarcomas in the modern era: Resection or transplantation? J Surg Oncol. 2018;117(5):886-91. (2019) |
93 ♂ 144♀ |
52 |
237 |
|
Angiosarcoma: 64 Undifferentiated sarcoma: 4 Epithelioid Hemangioendothelioma: 67 Embryonic sarcoma: 31 Leiomyosarcoma: 33 |
Resection: 40.9% TX: 17.3% |
R0 Well-differentiated Epithelioid hemangioendothelioma |
5 years: 25% Angiosarcoma: <1 year |
Tripke et al13 (2019) |
5♂ 4♀ |
61 |
9 |
5 symptomatic (pain) |
Angiosarcoma |
Resection R0: 8 R1 + QT: 1 (Doxorubicin + Ifosfamide) |
Poorly differentiated |
Overall survival: 18 months R0: 59 months Disease free survival: 10 months , R0: 11months |
Zhou et al1414 Zhou F, Huang HZ, Zhou MT, Han SL. Surgical Treatment and Chemotherapy of Adult Primary Liver Sarcoma: Experiences from a Single Hospital in China. Dig Surg. 2019;36(1):46-52. (2019) |
12♂ 5♀ |
57 |
17 |
Pain: 9 Palpable mass: 7 Anorexia: 4 |
Liposarcoma: 6 Leiomyosarcoma: 4 Epithelioid hemangioendothelioma: 3 Fibrosarcoma: 2 Hepatoblastoma: 2 |
Biopsy: 5, Surgery: 12 (R0: 10), TACE: 5, QT: 17 (All) |
R0 |
Overall 5y: 12% (R0: 20%) |