پديد آورندگان :
ﻣﺤـﺮاﺑﻲ، آرﻳﺎﻧﺐ داﻧﺸﮕﺎه ﻫﺎﻳﺪﻟﺒـﺮگ آﻟﻤـﺎن - ﮔـﺮوه ﺟﺮاﺣﻲ ﻋﻤـﻮﻣﻲ و ﭘﻴﻮﻧﺪ اﻋﻀﺎء , ﻛﺸﻔـﻲ، آرش داﻧﺸﮕﺎه ﻫﺎﻳﺪﻟﺒـﺮگ آﻟﻤـﺎن - ﮔـﺮوه ﺟﺮاﺣﻲ ﻋﻤـﻮﻣﻲ و ﭘﻴﻮﻧﺪ اﻋﻀﺎء , ﻓﻨـﻮﻧﻲ، ﺣﻤﻴﺪرﺿﺎ داﻧﺸﮕﺎه ﻫﺎﻳﺪﻟﺒـﺮگ آﻟﻤـﺎن - ﮔـﺮوه ﺟﺮاﺣﻲ ﻋﻤـﻮﻣﻲ و ﭘﻴﻮﻧﺪ اﻋﻀﺎء
كليدواژه :
همانژيواندوتليوماي اپيتليوييد كبدي , پيوند كبد , رزكسيون كبد , درمان
چكيده فارسي :
ﺯﻣﻴﻨﻪ ﻭ ﻫﺪﻑ: ﻫﻤﺎﻧﮋﻳﻮﺍﻧﺪﻭﺗﻠﻴـﻮﻣﺎﻱ ﺍﭘﻴﺘﻠﻴﻮﻳﻴﺪ ﻛﺒﺪﻱ )(HEH ﺑﺪﺧﻴﻢ، ﻳﻚ ﺗﻮﻣـﻮﺭ ﻋﺮﻭﻗﻲ ﺑﺪﺧﻴـﻢ ﻧﺎﺩﺭ ﺑﺎ ﻋﻠﺖ ﻧﺎﺷﻨﺎﺧﺘﻪ ﻭ ﺳﻴﺮ ﻃﺒﻴﻌﻲ ﻣﺘﻐﻴﺮ ﺍﺳﺖ.
ﻧﻮﻳﺴﻨﺪﮔﺎﻥ ﺍﻳﻦ ﻣﻘﺎﻟﻪ، ﻣـﺮﻭﺭ ﺟﺎﻣﻌﻲ ﺍﺯ ﻣﺘـﻮﻥ ﺗﺤﻘﻴﻘﻲ ﺩﺭ ﻣـﻮﺭﺩ HEH ﺭﺍ ﺑﺎ ﺗﻤـﺮﻛﺰ ﺑﺮ ﭘﻴﺎﻣﺪﻫﺎﻱ ﺑﺎﻟﻴﻨﻲ ﭘﺲ ﺍﺯ ﺭﺍﻫﺒـﺮﺩﻫﺎﻱ ﺩﺭﻣﺎﻧﻲ ﻣﺘﻔﺎﻭﺕ، ﺍﺭﺍﺋﻪ ﻣﻲ ﺩﻫﻨﺪ.
ﻣﻮﺍﺩ ﻭ ﺭﻭﺵ ﻫﺎ: ﺩﺭ ﺍﻳﻦ ﻣـﺮﻭﺭ، ﺗﻤﺎﻣﻲ ﻣﺠﻤـﻮﻋﻪ ﻫﺎﻱ ﻣﻨﺘﺸﺮ ﺷﺪﻩ ﺩﺭ ﻣﻮﺭﺩ ﺑﻴﻤﺎﺭﺍﻥ ﻣﺒﺘﻼ ﺑﻪ HEH )ﺗﻌﺪﺍﺩ 434 ﺑﻴﻤـﺎﺭ( ﺍﺯ ﻧﺨﺴﺘﻴﻦ ﺗﻮﺻﻴﻒ ﺍﻳﻦ ﺑﻴﻤـﺎﺭﺍﻥ ﺩﺭ ﺳﺎﻝ 1984 ﺗﺎ ﻣﻘﺎﻟﺔ ﺣﺎﺿﺮ ﻣـﻮﺭﺩ ﺗﺤﻠﻴﻞ ﻗـﺮﺍﺭ ﮔﺮﻓﺖ. ﭘﺎﺭﺍﻣﺘـﺮﻫﺎﻱ ﻣﺮﻭﺭ ﺷﺪﻩ ﺷﺎﻣﻞ: ﺩﺍﺩﻩ ﻫﺎﻱ ﺟﻤﻌﻴﺖ ـ ﺷﻨﺎﺧﺘﻲ، ﺗﻈﺎﻫـﺮﺍﺕ ﺑﺎﻟﻴﻨﻲ، ﺭﻭﺵ ﻫﺎﻱ ﺩﺭﻣﺎﻧﻲ ﻭ ﭘﻴﺎﻣـﺪﻫﺎﻱ
ﺑﺎﻟﻴﻨﻲ ﺑﻮﺩ.
ﻳﺎﻓﺘﻪ ﻫﺎ: ﻣﻴﺎﻧﮕﻴﻦ ﺳﻨﻲ ﺑﻴﻤﺎﺭﺍﻥ ﻣﺒﺘﻼ ﺑﻪ HEH، 41/7 ﺳﺎﻝ ﻭ ﻧﺴﺒﺖ ﻣﺮﺩ ﺑﻪ ﺯﻥ، 2 ﺑﻪ 3 ﺑﻮﺩ. ﺷﺎﻳﻌﺘﺮﻳﻦ ﺗﻈﺎﻫـﺮﺍﺕ ﺑﺎﻟﻴﻨﻲ: ﺩﺭﺩ ﺭﺑﻊ ﻓـﻮﻗﺎﻧﻲ ﺭﺍﺳﺖ ﺷﻜﻢ، ﻫﭙﺎﺗﻮﻣﮕﺎﻟﻲ ﻭ ﻛﺎﻫﺶ ﻭﺯﻥ ﺑﻮﺩ. ﺍﻏﻠـﺐ ﺑﻴﻤﺎﺭﺍﻥ ﺑﺎ ﺗﻮﻣﻮﺭ ﭼﻨﺪ ﻛﺎﻧﻮﻧﻲ ﻛﻪ ﻫﺮ ﺩﻭ ﻟـﻮﺏ ﺭﺍ ﺩﺭﮔﻴـﺮ ﻛﺮﺩﻩ، ﻣـﺮﺍﺟﻌﻪ ﻛﺮﺩﻧﺪ. ﺷﺎﻳﻌﺘﺮﻳﻦ ﻣﻨﺎﻃﻖ ﺩﺭﮔﻴﺮﻱ ﺧﺎﺭﺝ ﻛﺒﺪﻱ ﺩﺭ ﺯﻣﺎﻥ ﺗﺸﺨﻴﺺ: ﺭﻳﻪ، ﺻﻔﺎﻕ، ﮔـﺮﻩ ﻫﺎﻱ ﻟﻨﻔـﺎﻭﻱ ﻭ ﺍﺳﺘﺨﻮﺍﻥ ﺑﻮﺩ. ﺷﺎﻳﻌﺘـﺮﻳﻦ ﺗﺪﺍﺑﻴـﺮ ﺩﺭﻣﺎﻧﻲ: ﭘﻴـﻮﻧﺪ ﻛﺒﺪ (LTx) )44/8 ﺍﺯ ﺑﻴﻤـﺎﺭﺍﻥ(، ﭘﻴﮕﻴـﺮﻱ ﺑﺪﻭﻥ ﺩﺭﻣﺎﻥ 24/8% ﺍﺯ ﺑﻴﻤﺎﺭﺍﻥ(، ﺷﻴﻤﻲ ﺩﺭﻣﺎﻧﻲ ﻳﺎ ﭘﺮﺗﻮ ﺩﺭﻣﺎﻧﻲ ﺍﺯ ﺑﻴﻤﺎﺭﺍﻥ(، ﻭ ﺭﺯﻛﺴﻴـﻮﻥ ﻛﺒﺪ )(LRx )9/4 ﺍﺯ ﺑﻴﻤـﺎﺭﺍﻥ( ﺑﻮﺩ. ﻣﻴـﺰﺍﻥ ﺑﻘـﺎﻱ ﻳﻚ ﻭ ﭘﻨـﺞ ﺳﺎﻟﺔ ﭘﺲ ﺍﺯ LTx ﺑﻪ ﺗﺮﺗﻴﺐ 96 ﻭ 54/5%، ﭘﺲ ﺍﺯ ﻋـﺪﻡ ﺩﺭﻣﺎﻥ ﺑﻪ ﺗﺮﺗﻴﺐ 39/3 ﻭ 4/5 ﭘﺲ ﺍﺯ ﺷﻴﻤـﻲ ﺩﺭﻣﺎﻧﻲ ﻳﺎ ﭘﺮﺗﻮﺩﺭﻣﺎﻧﻲ ﺑﻪ ﺗﺮﺗﻴﺐ 73/3 ﻭ 30 ﻭ ﭘﺲ ﺍﺯ LRx ﺑﻪ ﺗﺮﺗﻴﺐ 100 ﻭ
75 ﺑﻮﺩ.
ﻧﺘﻴﺠﻪ ﮔﻴـﺮﻱ: LRx ﺩﺭﻣﺎﻥ ﺍﻧﺘﺨـﺎﺑﻲ ﺑﺮﺍﻱ ﺑﻴﻤـﺎﺭﺍﻥ ﻣﺒﺘﻼ ﺑﻪ HEH ﻗﺎﺑﻞ ﺭﺯﻛﺴﻴـﻮﻥ ﺍﺳﺖ، ﺑﺎ ﺍﻳﻦ ﻭﺟـﻮﺩ، ﺑﻪ ﺩﻟﻴﻞ ﭼﻨﺪ ﻣـﺮﻛﺰﻱ ﺑﻮﺩﻥ HEH ﻛﺒـﺪﻱ، LTx ﺑﻪ ﻋﻨﻮﺍﻥ ﺩﺭﻣـﺎﻥ ﺍﻧﺘﺨـﺎﺑﻲ ﭘﻴﺸﻨﻬـﺎﺩ ﺷﺪﻩ ﺍﺳﺖ. ﻋﻼﻭﻩ ﺑﺮ ﺍﻳﻦ، LTx ﮔﺰﻳﻨﺔ ﻗﺎﺑﻞ ﻗﺒـﻮﻟﻲ ﺑﺮﺍﻱ ﺑﻴﻤﺎﺭﺍﻧﻲ ﺍﺳﺖ ﻛﻪ HEH ﺑﺎ ﺗﻈﺎﻫـﺮﺍﺕ ﺧﺎﺭﺝ ﻛﺒﺪﻱ ﺩﺍﺭﻧﺪ. ﺷﺎﻳﺪ ﺑﺘﻮﺍﻥ ﺑﻴﻤﺎﺭﺍﻥ ﻛﺎﻣﻼﹰ ﮔﺰﻳﻨﺶ ﺷﺪﻩ ﺭﺍ ﺗﺤﺖ LTx ﺍﺯ ﺍﻫـﺪﺍﺀ ﻛﻨﻨﺪﺓ ﺯﻧﺪﻩ ﺑﺎ ﺣﻔـﻆ ﻣﻨﺒﻊ ﺍﻫـﺪﺍﺀ( ﻗـﺮﺍﺭ ﺩﺍﺩ. ﻧﻘﺶ ﺩﺭﻣﺎﻥ ﻫﺎﻱ ﻛﻤﻜﻲ ﻣﺨﺘﻠﻒ ﺑﺮﺍﻱ ﺑﻴﻤﺎﺭﻥ ﻣﺒﺘﻼ ﺑﻪ heh ﻫﻤﭽﻨﺎﻥ ﻧﺎﻣﻌﻠـﻮﻡ ﺍﺳﺖ.
چكيده لاتين :
tumor of vascular origin with unknown etiology and a variable natural course. The authors present a
comprehensive review of the literature on HER with a focus on clinical outcome after different therapeutic
strategies.
Materials & Methods: All published series on patients with HEH (n = 434 patients) were: analyzed
from the first description in 1984 to the current literature. The reviewed parameters included demographic
data clinical manifestations, therapeutic modalities, and clinical outcome.
Results: The mean age of patients with HER was 41.7 years and the male-to-female ratio was 2:3. The
most common clinical manifestations were right upper quadrant pain, hepatomegaly, and weight loss. Most
patients presented with multifocal tumor that involved both lobes of the liver. Lung peritoneum, lymph nodes,
and bone were the most common sites of extrahepatic involvement at the time of diagnosis. The most
common management has been liver transplantation (LTx) (44.8% of patients), followed by no treatment
(24.8% of patients), chemo-therapy or radiotherapy (21% of patients), and liver resection (LRx) (9.4% of
patients). The 1-year and 5-year patient survival rates were 96% and 54.5%, respectively, after LTx; 39.3%
and 4.5%, respectively; after no treatment, 73.3% and 30%, respectively, after chemotherapy or radiotherapy;
and 100% and 75%, respectively, after LRx.
Conclusions: LRx has been the treatment of chosen in patients with resectable HER. LTx has been
proposed as the treatment of choice because of the hepatic muIticentricity of HEH. In addition, LTx is an
acceptable option for patients, who have HEH with extrahepatic manifestation. Highly selected patients may
be able to undergo living-donor LTx, preserving the donor pool. The role of different adjuvant therapies for
patients with HEH remains to be determined.
