A 15-year-old girl was admitted to our hospital with a 9-month history of upper abdominal pain and loss of appetite. Her history showed no indication of hepatitis. Her abdomen appeared to be even and soft, and the liv...A 15-year-old girl was admitted to our hospital with a 9-month history of upper abdominal pain and loss of appetite. Her history showed no indication of hepatitis. Her abdomen appeared to be even and soft, and the liver and spleen could not be felt below the costal margin. Percussion pain in hepatic region was negative. Gastroscopy showed no abnormalities. An abdominal ultrasound examination revealed the presence of hepatic hypoechoic areas and an abdominal CT scan showed multiple roundlike low-density masses in both hepatic lobes. The edges of the focal lesions were smooth and continuous, with a heterogeneous center, the round-like edges were enhanced, but the enhancement in the focal lesions was not obvious.展开更多
Primary malignant vascular neoplasms of the liver,angiosarcoma and epithelioid hemangioendothelioma,are extremely rare entities in the pediatric population.International Society for the Study of Vascular Anomalies cla...Primary malignant vascular neoplasms of the liver,angiosarcoma and epithelioid hemangioendothelioma,are extremely rare entities in the pediatric population.International Society for the Study of Vascular Anomalies classification system is recommended for the pathologic diagnosis of hepatic vascular lesions in this age group.In this article,we highlight the clinicopathologic characteristics of hepatic angiosarcoma and epithelioid hemangioendothelioma in the pediatric population.Hepatic angiosarcoma in children shows a slight female predominance with an average age of 40 mo at diagnosis.The distinct histologic features include whorls of atypical spindled cells and eosinophilic globules,in addition to the general findings of angiosarcoma.Histologic diagnosis of pediatric hepatic angiosarcoma is not always straightforward,and the diagnostic challenges are discussed in the article.Hepatic epithelioid hemangioendothelioma also demonstrates a female predominance,but is more commonly identified in adolescents(median age at diagnosis:12 years).Histologically,the lesion is characterized by epithelioid cells and occasional intracytoplasmic lumina with a background of fibromyxoid stroma.While WWTR1-CAMTA1 and YAP1-TFE3 fusions have been associated with epithelioid hemangioendothelioma,there are currently no known signature genetic alterations seen in pediatric hepatic angiosarcoma.Advancement in molecular pathology,particularly for pediatric hepatic angiosarcoma,is necessary for a better understanding of the disease biology,diagnosis,and development of targeted therapies.展开更多
BACKGROUND: Hepatic epithelioid hemangioendothelioma (EHE) is a rare, low-grade malignant vascular tumor. Although its unusual imaging and pathologic findings are being recognized with increasing frequency, diagnosis ...BACKGROUND: Hepatic epithelioid hemangioendothelioma (EHE) is a rare, low-grade malignant vascular tumor. Although its unusual imaging and pathologic findings are being recognized with increasing frequency, diagnosis is still difficult. This study aimed to analyze the CT and MRI features of hepatic EHE with a pathological study in order to improve the diagnostic accuracy and knowledge of this disease in daily practice. METHODS: Nine patients with hepatic EHE confirmed pathologically underwent plain and dynamic contrast-enhanced multi-detector row CT examination. Of these patients, four underwent additional MRI (plain Ti-weighted imaging (T1WI), T2-weighted imaging (T2WI), and dynamic contrast-enhanced scanning) and one had selective hepatic arteriography. The imaging findings were reviewed retrospectively together with the pathological results. RESULTS: A total of 79 lesions, ranging from 3.0 to 44.6 mm in maximum diameter, with an average of 16.8 +/- 7.1 mm, were found in various segments of the liver. Thirty of the 79 lesions grew adjacent to the hepatic capsule. In the 4 patients receiving MRI, 39 lesions were found with low signal intensity on unenhanced T1WI and intermediate to high signal intensity on T2WI. The 'capsular retraction' sign was found in all the 4 patients. Nine of the 39 lesions showed the 'halo' sign after contrast enhancement on MRI. Of the 79 lesions (hypodense nodules) in the 9 patients shown by unenhanced plain CT, 26 were confluent. Calcification was found in 2 patients and the 'capsular retraction' sign in 7. Thirty-eight of the 79 lesions demonstrated the 'halo' sign after contrast enhancement on CT, and this sign was more clearly demonstrated in the portal venous phase. In one patient, selective hepatic arteriography showed patchy stain in the peripheral liver parenchyma with small vessels around them. Histology in all patients revealed proliferation of abnormal fibrous tissue and vessel-like structures scattered with irregular epithelioid cells having a signet ring-like structure. Immunohistochemically, all patients were positive for CD34, 4 were positive for CD31, and 3 were positive for factor VIII-related antigen. CONCLUSIONS: Hepatic EHE may manifest as solitary or diffuse nodular lesions with a predilection for peripheral subcapsular growth and nodular confluence, together with the 'halo' and 'capsular retraction' signs. These imaging findings can help to improve the diagnostic accuracy of this rare hepatic tumor. (Hepatobiliary Pancreat Dis Int 2010; 9: 154-158)展开更多
Background: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of vascular origin with an unknown etiology, a low incidence, and a variable natural course. We evaluated the management and prognosis of HEH ...Background: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of vascular origin with an unknown etiology, a low incidence, and a variable natural course. We evaluated the management and prognosis of HEH from the Surveillance, Epidemiology and End Results (SEER) program and changes in treatment modalities of HEH over 30 years. Methods: From 1973 to 2014 in the SEER database, we selected patients diagnosed with HEH. We analyzed the clinical characteristics, patterns of management, and clinical outcomes of patients with HEH. Results: We identi ed 79 patients with HEH (median age: 54.0 years;male to female ratio: 1:2.6). The initial extent of disease was local in 22 (27.8%) patients, regional metastasis in 22 (27.8%), distant metas-tasis in 31 (39.2%) and unknown in 4 (5.1%). The median size of primary tumor was 3.85 cm (interquartile range, 2.50 7.93 cm). Among 74 patients with available management data, the most common manage-ment was no treatment (29/74, 39.2%), followed by chemotherapy only (22/74, 29.7%), liver resection-based (13/74, 17.6%), and transplantation-based therapy (6/74, 8.1%). The 5-year cancer-speci c survival rate was 57.8%. Patients who underwent surgical treatment had signi cantly higher survival than those who underwent non-surgical treatment (5-year survival;88% vs. 49%, P=0.019). Multivariate analysis revealed that surgical therapy was the only independent prognostic factor for survival (hazard ratio: 0.20, P=0.040). Conclusions: Resection or liver transplantation is worth considering for treatment of patients with HEH.展开更多
Epithelioid hemangioendothelioma(EH)is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs.The most common visceral site is the liver,where it is often involved in a multifocal m...Epithelioid hemangioendothelioma(EH)is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs.The most common visceral site is the liver,where it is often involved in a multifocal man-ner known as hepatic EH(HEH).Surgical resection with curative intent represents the gold standard therapy.When surgery is not feasible,or in cases of metastatic disease,no standard medical treatment is currently indicated.In small series,drugs with anti-angiogenic activity(such as bevacizumab,sorafenib,thalidomide,and lenalidomide)have been proposed with promising results.We describe a 73-year-old man with multifocal non-resectable HEH treated with lenalidomide.Disease status was evaluated by abdominal ultrasound and magnetic resonance every four months.The patient was treated for a total of 39 mo with prolonged disease stabilization and,at the time of writing,is still under treatment with a good tolerance profile.During a short period of treatment discontinuation,the disease showed slight progression that immediately resolved after the reintroduction of lenalidomide.Lenalidomide may represent a valid treatment option for HEH due to its anti-angiogenic and antineoplastic activities.This preliminary result merits further study in a large series.展开更多
BACKGROUND Hepatic epithelioid hemangioendothelioma(EHE)is a rare vascular endothelial cell tumor of the liver,consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix.Imm...BACKGROUND Hepatic epithelioid hemangioendothelioma(EHE)is a rare vascular endothelial cell tumor of the liver,consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix.Immunohistochemistry is usually positive for vascular markers,such as factor VIII-related antigen,CD31,and CD34.Hepatic EHE can have a varied clinical course;treatment includes liver transplantation,liver resection,chemotherapy,and radiation therapy.CASE SUMMARY A 46-year-old woman with abdominal discomfort and elevated serum carcinoembryonic antigen was found to have multiple low-density lesions in the liver and lung on computed tomography(CT)evaluation.An ultrasound-guided fine needle aspiration biopsy revealed a fibrous stroma with dendritic cells,containing intracellular vacuoles.Immunohistochemical staining found that the tumor cells were positive for CD34,CD31,and factor VIII-related antigen.The patient received four courses of combined chemotherapy and was followed-up for 13 years,at which time the patient was in stable condition without disease progression and a confined neoplasm,as evidenced by CT scans.CONCLUSION The histology and immunohistochemical characteristics of hepatic EHE are well described.Chemotherapy may be effective in patients with extrahepatic lesions.展开更多
AIM： To investigate and review the contrast-enhanced multiple-phase computed tomography （CEMP CT） and magnetic resonance imaging （MRI） findings in patients with pathologically confirmed hepatic epithelioid hemang...AIM： To investigate and review the contrast-enhanced multiple-phase computed tomography （CEMP CT） and magnetic resonance imaging （MRI） findings in patients with pathologically confirmed hepatic epithelioid hemangioendothelioma （HEHE）. METHODS： Findings from imaging examinations in 8 patients （5 women and 3 men） with pathologically confirmed HEHE were retrospectively reviewed （CT images obtained from 7 patients and MR images obtained from 6 patients）. The age of presentation varied from 27 years to 60 years （average age 39.8 years）. RESULTS： There were two types of HEHE： multifocal type （n = 7） and diffuse type （n = 1）. Tn the multifocal-type cases, there were 74 lesions on CT and 28 lesions on MRI with 7 lesions found with diffusion weighted imaging; 18 （24.3%） of 74 lesions on plain CT and 26 （92.9%） of 28 lesions on pre-contrast MRI showed the target sign. On CEMP CT, 28 （37.8%） of 74 lesions appeared with the target sign and a progressive-enhancement rim and 9 （12.2%） of 74 lesions displayed progressive enhancement, maintaining a state of persistent enhancement. On CEMP MRI, 27 （96.4%） of 28 lesions appeared with the target sign with a progressive-enhancement rim and 28 （100%） of 28 lesions displayed progressive-enhancement, maintaining a state of persistent enhancement. In the diffuse-type cases, an enlarged liver was observed with a large nodule appearing with persistent enhancement on CEMP CT and MRI. CONCLUSION： The most important imaging features of HEHE are the target sign and/or progressive en- hancement with persistent enhancement on CEMP CT and MRI. MRI is advantageous over CT in displaying these imaging features.展开更多
A new case of epithelioid hemangioendothelioma is reported to have occurred to a 67-year-old patient who consulted for rightsided chest pain. The work-up showed multiple right pulmonary lesions associated with bilater...A new case of epithelioid hemangioendothelioma is reported to have occurred to a 67-year-old patient who consulted for rightsided chest pain. The work-up showed multiple right pulmonary lesions associated with bilateral moderate pleural effusion and left-sided pleural thickening and three hypodense nodules in the right lobe of the liver, peritoneal thickening, ascites, and multiple vertebral lytic lesions. The diagnosis of an epithelioid hemangioendothelioma was concluded through a histological examination of a computed tomography scan guided biopsy of the liver. The patient received a primary mono-chemotherapy with Adriamycin(75 mg/m^2 every three weeks) and intravenous bisphosphonates without response and general status impairment. The patient died after 16 months of follow-up.展开更多
Benign liver tumors are common lesions that are usually asymptomatic and are often found incidentally due to recent advances in imaging techniques and their widespread use.Although most of these tumors can be managed ...Benign liver tumors are common lesions that are usually asymptomatic and are often found incidentally due to recent advances in imaging techniques and their widespread use.Although most of these tumors can be managed conservatively or treated by surgical resection,liver transplantation(LT)is the only treatment option in selected patients.LT is usually indicated in patients that present with life-threatening complications,when the lesions are diffuse in the hepatic parenchyma or when malignant transformation cannot be ruled out.However,due to the significant postoperative morbidity of the procedure,scarcity of available donor liver grafts,and the benign course of the disease,the indications for LT are still not standardized.Hepatic adenoma and adenomatosis,hepatic hemangioma,and hepatic epithelioid hemangioendothelioma are among the most common benign liver tumors treated by LT.This article reviews the role of LT in patients with benign liver tumors.The indications for LT and long-term outcomes of LT are presented.展开更多
Liver transplantation for malignant disease has gained increasing attention as part of transplant oncology.Following the implementation of the Milan criteria,hepatocellular carcinoma(HCC)was the first generally accept...Liver transplantation for malignant disease has gained increasing attention as part of transplant oncology.Following the implementation of the Milan criteria,hepatocellular carcinoma(HCC)was the first generally accepted indication for transplantation in patients with cancer.Subsequently,more liberal criteria for HCC have been developed,and research on this topic is still ongoing.The evident success of liver transplantation for HCC has led to the attempt to extend its indication to other malignancies.Regarding perihilar cholangiocarcinoma,more and more evidence supports the use of liver transplantation,especially after neoadjuvant therapy.In addition,some data also show a benefit for selected patients with very early stage intrahepatic cholangiocarcinoma.Hepatic epithelioid hemangioendothelioma is a very rare but nonetheless established indication for liver transplantation in primary liver cancer.In contrast,patients with hepatic angiosarcoma are currently not considered to be optimal candidates.In secondary liver tumors,neuroendocrine cancer liver metastases are an accepted but comparability rare indication for liver transplantation.Recently,some evidence has been published supporting the use of liver transplantation even for colorectal liver metastases.This review summarizes the current evidence for liver transplantation for primary and secondary liver cancer.展开更多
BACKGROUND Mesenchymal tumors such as perivascular epithelioid cell neoplasm(PEComa)and inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDC sarcoma)are relatively uncommon in the liver and are...BACKGROUND Mesenchymal tumors such as perivascular epithelioid cell neoplasm(PEComa)and inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDC sarcoma)are relatively uncommon in the liver and are particularly rare in the caudate lobe.The clinical manifestations and available imaging tests lack specificity for hepatic mesenchymal tumors.To the best of our knowledge,no caudate PEComa or IPT-like FDC sarcoma has been completely resected by laparoscopy.The standard laparoscopic technique,surgical approaches,and tumor margins for potentially malignant or malignant caudate mesenchymal tumors are still being explored.AIM To assess both the safety and feasibility of laparoscopic resection for rare caudate mesenchymal neoplasms.METHODS Eleven patients who underwent isolated caudate lobe resection from 2003 to 2017 were identified from a prospective database.Three consecutive patients with rare caudate mesenchymal tumors underwent laparoscopic resection.Patient demographic data,intraoperative parameters,and postoperative outcomes were assessed and compared with the open surgery group.RESULTS All procedures for the three resection patients with caudate mesenchymal tumors were completed using a total laparoscopic technique by two different approaches.The average operative time was 226 min,and the estimated blood loss was 133 mL.The average length of postoperative hospital stay was 6.3±0.3 d for the laparoscopy group and 15.5±2.3 d for the open surgery group(P<0.05).There were no perioperative complications or patient deaths in this series.CONCLUSION Laparoscopic isolated caudate lobe resection for rare mesenchymal neoplasms is a feasible and curative surgical option in selected patients.展开更多
文摘A 15-year-old girl was admitted to our hospital with a 9-month history of upper abdominal pain and loss of appetite. Her history showed no indication of hepatitis. Her abdomen appeared to be even and soft, and the liver and spleen could not be felt below the costal margin. Percussion pain in hepatic region was negative. Gastroscopy showed no abnormalities. An abdominal ultrasound examination revealed the presence of hepatic hypoechoic areas and an abdominal CT scan showed multiple roundlike low-density masses in both hepatic lobes. The edges of the focal lesions were smooth and continuous, with a heterogeneous center, the round-like edges were enhanced, but the enhancement in the focal lesions was not obvious.
文摘Primary malignant vascular neoplasms of the liver,angiosarcoma and epithelioid hemangioendothelioma,are extremely rare entities in the pediatric population.International Society for the Study of Vascular Anomalies classification system is recommended for the pathologic diagnosis of hepatic vascular lesions in this age group.In this article,we highlight the clinicopathologic characteristics of hepatic angiosarcoma and epithelioid hemangioendothelioma in the pediatric population.Hepatic angiosarcoma in children shows a slight female predominance with an average age of 40 mo at diagnosis.The distinct histologic features include whorls of atypical spindled cells and eosinophilic globules,in addition to the general findings of angiosarcoma.Histologic diagnosis of pediatric hepatic angiosarcoma is not always straightforward,and the diagnostic challenges are discussed in the article.Hepatic epithelioid hemangioendothelioma also demonstrates a female predominance,but is more commonly identified in adolescents(median age at diagnosis:12 years).Histologically,the lesion is characterized by epithelioid cells and occasional intracytoplasmic lumina with a background of fibromyxoid stroma.While WWTR1-CAMTA1 and YAP1-TFE3 fusions have been associated with epithelioid hemangioendothelioma,there are currently no known signature genetic alterations seen in pediatric hepatic angiosarcoma.Advancement in molecular pathology,particularly for pediatric hepatic angiosarcoma,is necessary for a better understanding of the disease biology,diagnosis,and development of targeted therapies.
文摘BACKGROUND: Hepatic epithelioid hemangioendothelioma (EHE) is a rare, low-grade malignant vascular tumor. Although its unusual imaging and pathologic findings are being recognized with increasing frequency, diagnosis is still difficult. This study aimed to analyze the CT and MRI features of hepatic EHE with a pathological study in order to improve the diagnostic accuracy and knowledge of this disease in daily practice. METHODS: Nine patients with hepatic EHE confirmed pathologically underwent plain and dynamic contrast-enhanced multi-detector row CT examination. Of these patients, four underwent additional MRI (plain Ti-weighted imaging (T1WI), T2-weighted imaging (T2WI), and dynamic contrast-enhanced scanning) and one had selective hepatic arteriography. The imaging findings were reviewed retrospectively together with the pathological results. RESULTS: A total of 79 lesions, ranging from 3.0 to 44.6 mm in maximum diameter, with an average of 16.8 +/- 7.1 mm, were found in various segments of the liver. Thirty of the 79 lesions grew adjacent to the hepatic capsule. In the 4 patients receiving MRI, 39 lesions were found with low signal intensity on unenhanced T1WI and intermediate to high signal intensity on T2WI. The 'capsular retraction' sign was found in all the 4 patients. Nine of the 39 lesions showed the 'halo' sign after contrast enhancement on MRI. Of the 79 lesions (hypodense nodules) in the 9 patients shown by unenhanced plain CT, 26 were confluent. Calcification was found in 2 patients and the 'capsular retraction' sign in 7. Thirty-eight of the 79 lesions demonstrated the 'halo' sign after contrast enhancement on CT, and this sign was more clearly demonstrated in the portal venous phase. In one patient, selective hepatic arteriography showed patchy stain in the peripheral liver parenchyma with small vessels around them. Histology in all patients revealed proliferation of abnormal fibrous tissue and vessel-like structures scattered with irregular epithelioid cells having a signet ring-like structure. Immunohistochemically, all patients were positive for CD34, 4 were positive for CD31, and 3 were positive for factor VIII-related antigen. CONCLUSIONS: Hepatic EHE may manifest as solitary or diffuse nodular lesions with a predilection for peripheral subcapsular growth and nodular confluence, together with the 'halo' and 'capsular retraction' signs. These imaging findings can help to improve the diagnostic accuracy of this rare hepatic tumor. (Hepatobiliary Pancreat Dis Int 2010; 9: 154-158)
基金supported by the Bio&Medical Tech-nology Development Program of the National Research Foun-dation(NRF)funded by the Korean government(MSIT)(NRF-2018M3A9E8023861)by a grant from the Korean Health R&D Project.Ministry of Health Welfare,Korea(HI18C0531)
文摘Background: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of vascular origin with an unknown etiology, a low incidence, and a variable natural course. We evaluated the management and prognosis of HEH from the Surveillance, Epidemiology and End Results (SEER) program and changes in treatment modalities of HEH over 30 years. Methods: From 1973 to 2014 in the SEER database, we selected patients diagnosed with HEH. We analyzed the clinical characteristics, patterns of management, and clinical outcomes of patients with HEH. Results: We identi ed 79 patients with HEH (median age: 54.0 years;male to female ratio: 1:2.6). The initial extent of disease was local in 22 (27.8%) patients, regional metastasis in 22 (27.8%), distant metas-tasis in 31 (39.2%) and unknown in 4 (5.1%). The median size of primary tumor was 3.85 cm (interquartile range, 2.50 7.93 cm). Among 74 patients with available management data, the most common manage-ment was no treatment (29/74, 39.2%), followed by chemotherapy only (22/74, 29.7%), liver resection-based (13/74, 17.6%), and transplantation-based therapy (6/74, 8.1%). The 5-year cancer-speci c survival rate was 57.8%. Patients who underwent surgical treatment had signi cantly higher survival than those who underwent non-surgical treatment (5-year survival;88% vs. 49%, P=0.019). Multivariate analysis revealed that surgical therapy was the only independent prognostic factor for survival (hazard ratio: 0.20, P=0.040). Conclusions: Resection or liver transplantation is worth considering for treatment of patients with HEH.
文摘Epithelioid hemangioendothelioma(EH)is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs.The most common visceral site is the liver,where it is often involved in a multifocal man-ner known as hepatic EH(HEH).Surgical resection with curative intent represents the gold standard therapy.When surgery is not feasible,or in cases of metastatic disease,no standard medical treatment is currently indicated.In small series,drugs with anti-angiogenic activity(such as bevacizumab,sorafenib,thalidomide,and lenalidomide)have been proposed with promising results.We describe a 73-year-old man with multifocal non-resectable HEH treated with lenalidomide.Disease status was evaluated by abdominal ultrasound and magnetic resonance every four months.The patient was treated for a total of 39 mo with prolonged disease stabilization and,at the time of writing,is still under treatment with a good tolerance profile.During a short period of treatment discontinuation,the disease showed slight progression that immediately resolved after the reintroduction of lenalidomide.Lenalidomide may represent a valid treatment option for HEH due to its anti-angiogenic and antineoplastic activities.This preliminary result merits further study in a large series.
基金Supported by Zhejiang Medical and Health Science and Technology Project,No.2021429795Scientific Research Project of Zhejiang Education Department,No.Y202043306。
文摘BACKGROUND Hepatic epithelioid hemangioendothelioma(EHE)is a rare vascular endothelial cell tumor of the liver,consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix.Immunohistochemistry is usually positive for vascular markers,such as factor VIII-related antigen,CD31,and CD34.Hepatic EHE can have a varied clinical course;treatment includes liver transplantation,liver resection,chemotherapy,and radiation therapy.CASE SUMMARY A 46-year-old woman with abdominal discomfort and elevated serum carcinoembryonic antigen was found to have multiple low-density lesions in the liver and lung on computed tomography(CT)evaluation.An ultrasound-guided fine needle aspiration biopsy revealed a fibrous stroma with dendritic cells,containing intracellular vacuoles.Immunohistochemical staining found that the tumor cells were positive for CD34,CD31,and factor VIII-related antigen.The patient received four courses of combined chemotherapy and was followed-up for 13 years,at which time the patient was in stable condition without disease progression and a confined neoplasm,as evidenced by CT scans.CONCLUSION The histology and immunohistochemical characteristics of hepatic EHE are well described.Chemotherapy may be effective in patients with extrahepatic lesions.
文摘AIM： To investigate and review the contrast-enhanced multiple-phase computed tomography （CEMP CT） and magnetic resonance imaging （MRI） findings in patients with pathologically confirmed hepatic epithelioid hemangioendothelioma （HEHE）. METHODS： Findings from imaging examinations in 8 patients （5 women and 3 men） with pathologically confirmed HEHE were retrospectively reviewed （CT images obtained from 7 patients and MR images obtained from 6 patients）. The age of presentation varied from 27 years to 60 years （average age 39.8 years）. RESULTS： There were two types of HEHE： multifocal type （n = 7） and diffuse type （n = 1）. Tn the multifocal-type cases, there were 74 lesions on CT and 28 lesions on MRI with 7 lesions found with diffusion weighted imaging; 18 （24.3%） of 74 lesions on plain CT and 26 （92.9%） of 28 lesions on pre-contrast MRI showed the target sign. On CEMP CT, 28 （37.8%） of 74 lesions appeared with the target sign and a progressive-enhancement rim and 9 （12.2%） of 74 lesions displayed progressive enhancement, maintaining a state of persistent enhancement. On CEMP MRI, 27 （96.4%） of 28 lesions appeared with the target sign with a progressive-enhancement rim and 28 （100%） of 28 lesions displayed progressive-enhancement, maintaining a state of persistent enhancement. In the diffuse-type cases, an enlarged liver was observed with a large nodule appearing with persistent enhancement on CEMP CT and MRI. CONCLUSION： The most important imaging features of HEHE are the target sign and/or progressive en- hancement with persistent enhancement on CEMP CT and MRI. MRI is advantageous over CT in displaying these imaging features.
文摘A new case of epithelioid hemangioendothelioma is reported to have occurred to a 67-year-old patient who consulted for rightsided chest pain. The work-up showed multiple right pulmonary lesions associated with bilateral moderate pleural effusion and left-sided pleural thickening and three hypodense nodules in the right lobe of the liver, peritoneal thickening, ascites, and multiple vertebral lytic lesions. The diagnosis of an epithelioid hemangioendothelioma was concluded through a histological examination of a computed tomography scan guided biopsy of the liver. The patient received a primary mono-chemotherapy with Adriamycin(75 mg/m^2 every three weeks) and intravenous bisphosphonates without response and general status impairment. The patient died after 16 months of follow-up.
文摘Benign liver tumors are common lesions that are usually asymptomatic and are often found incidentally due to recent advances in imaging techniques and their widespread use.Although most of these tumors can be managed conservatively or treated by surgical resection,liver transplantation(LT)is the only treatment option in selected patients.LT is usually indicated in patients that present with life-threatening complications,when the lesions are diffuse in the hepatic parenchyma or when malignant transformation cannot be ruled out.However,due to the significant postoperative morbidity of the procedure,scarcity of available donor liver grafts,and the benign course of the disease,the indications for LT are still not standardized.Hepatic adenoma and adenomatosis,hepatic hemangioma,and hepatic epithelioid hemangioendothelioma are among the most common benign liver tumors treated by LT.This article reviews the role of LT in patients with benign liver tumors.The indications for LT and long-term outcomes of LT are presented.
文摘Liver transplantation for malignant disease has gained increasing attention as part of transplant oncology.Following the implementation of the Milan criteria,hepatocellular carcinoma(HCC)was the first generally accepted indication for transplantation in patients with cancer.Subsequently,more liberal criteria for HCC have been developed,and research on this topic is still ongoing.The evident success of liver transplantation for HCC has led to the attempt to extend its indication to other malignancies.Regarding perihilar cholangiocarcinoma,more and more evidence supports the use of liver transplantation,especially after neoadjuvant therapy.In addition,some data also show a benefit for selected patients with very early stage intrahepatic cholangiocarcinoma.Hepatic epithelioid hemangioendothelioma is a very rare but nonetheless established indication for liver transplantation in primary liver cancer.In contrast,patients with hepatic angiosarcoma are currently not considered to be optimal candidates.In secondary liver tumors,neuroendocrine cancer liver metastases are an accepted but comparability rare indication for liver transplantation.Recently,some evidence has been published supporting the use of liver transplantation even for colorectal liver metastases.This review summarizes the current evidence for liver transplantation for primary and secondary liver cancer.
基金Supported by the National Natural Science Foundation of China,No.81600505Medical Scientific Research Foundation of Guangdong Province,No.A2017370
文摘BACKGROUND Mesenchymal tumors such as perivascular epithelioid cell neoplasm(PEComa)and inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDC sarcoma)are relatively uncommon in the liver and are particularly rare in the caudate lobe.The clinical manifestations and available imaging tests lack specificity for hepatic mesenchymal tumors.To the best of our knowledge,no caudate PEComa or IPT-like FDC sarcoma has been completely resected by laparoscopy.The standard laparoscopic technique,surgical approaches,and tumor margins for potentially malignant or malignant caudate mesenchymal tumors are still being explored.AIM To assess both the safety and feasibility of laparoscopic resection for rare caudate mesenchymal neoplasms.METHODS Eleven patients who underwent isolated caudate lobe resection from 2003 to 2017 were identified from a prospective database.Three consecutive patients with rare caudate mesenchymal tumors underwent laparoscopic resection.Patient demographic data,intraoperative parameters,and postoperative outcomes were assessed and compared with the open surgery group.RESULTS All procedures for the three resection patients with caudate mesenchymal tumors were completed using a total laparoscopic technique by two different approaches.The average operative time was 226 min,and the estimated blood loss was 133 mL.The average length of postoperative hospital stay was 6.3±0.3 d for the laparoscopy group and 15.5±2.3 d for the open surgery group(P<0.05).There were no perioperative complications or patient deaths in this series.CONCLUSION Laparoscopic isolated caudate lobe resection for rare mesenchymal neoplasms is a feasible and curative surgical option in selected patients.
