Malignant vascular tumors of the liver include rare primary hepatic mesenchymal tumors developed in the background of a normal liver parenchyma. Most of them are detected incidentally by the increased use of performin...Malignant vascular tumors of the liver include rare primary hepatic mesenchymal tumors developed in the background of a normal liver parenchyma. Most of them are detected incidentally by the increased use of performing imaging techniques. Their diagnosis is challenging, involving clinical and imaging criteria, with final confirmation by histology and immunohistochemistry. Surgery represents the mainstay of treatment. Liver transplantation(LT) has improved substantially the prognosis of hepatic epithelioid hemangioendothelioma(HEHE), with 5-year patient survival rates of up to 81%, based on the European Liver Intestine Transplantation AssociationEuropean Liver Transplant Registry study. Unfortunately, the results of surgery and LT are dismal in cases of hepatic angiosarcoma(HAS). Due to the disappointing results of very short survival periods of approximately 6-7 mo after LT, because of tumor recurrence and rapid progression of the disease, HAS is considered an absolute contraindication to LT. Recurrences after surgical resection are high in cases of HEHE and invariably present in cases of HAS. The discovery of reliable prognostic markers and the elaboration of prognostic scores following LT are needed to provide the best therapeutic choice for each patient.Studies on a few patients have demonstrated the stabilization of the disease in a proportion of patients with hepatic vascular tumors using novel targeted antiangiogenic agents, cytokines or immunotherapy. These new approaches,alone or in combination with other therapeutic modalities, such as surgery and classical chemotherapy, need further investigation to assess their role in prolonging patient survival. Personalized therapeutic algorithms according to the histopathological features, behavior, molecular biology and genetics of the tumors should be elaborated in the near future for the management of patients diagnosed with primary malignant vascular tumors of the liver.展开更多
Background and Aims:Perivascular epithelioid cell neo-plasms(PEComas)are a rare type of mesenchymal neo-plasm and their preoperative diagnosis is challenging.In this study,we summarized the experience from a single me...Background and Aims:Perivascular epithelioid cell neo-plasms(PEComas)are a rare type of mesenchymal neo-plasm and their preoperative diagnosis is challenging.In this study,we summarized the experience from a single medical center to study the examinations,clinical presen-tations,and pathological and histological characteristics of PEComas in the liver in order to optimize overall un-derstanding of the diagnosis and treatment of these neo-plasms.Methods:We conducted a retrospective analysis to investigate the clinical and pathological characteristics as well as imaging presentations of 75 patients diagnosed with hepatic PEComa in The First Affiliated Hospital of Zhe-jiang University between April 2010 and April 2020.Re-sults:Among the 75 patients,52 were women,and the median age was 48 years.Most patients had no specific symptoms,and two were admitted to the hospital for a second time owing to relapse.All patients underwent surgi-cal resection.Histologically,38 patients had classical angio-myolipoma(AML)and 37 had epithelioid AML.The PECo-mas were accompanied by positive immunohistochemical expression of HMB45,Melan-A,and smooth muscle actin.Follow-up data were obtained from 47 of the total 75 pa-tients,through October 2020.Two patients had metastasis after surgery.Conclusions:AML is the most common type of hepatic PEComa.There are no specific symptoms of he-patic PEComa,and serological examinations and imaging modalities for accurate preoperative diagnosis are lacking.Epithelioid AML should be considered a tumor of uncertain malignant potential;however,the prognosis of PEComa af-ter resection is promising.展开更多
BACKGROUND Hepatic perivascular epithelioid cell neoplasms(PEComas)are rare.Diagnostic and treatment experience with hepatic PEComa remains insufficient.CASE SUMMARY Three hepatic PEComa cases are reported in this pap...BACKGROUND Hepatic perivascular epithelioid cell neoplasms(PEComas)are rare.Diagnostic and treatment experience with hepatic PEComa remains insufficient.CASE SUMMARY Three hepatic PEComa cases are reported in this paper:One case of primary malignant hepatic PEComa,one case of benign hepatic PEComa,and one case of hepatic PEComa with an ovarian mature cystic teratoma.During preoperative imaging and pathological assessment of intraoperative frozen samples,patients were diagnosed with hepatocellular carcinoma(HCC),while postoperative pathology and immunohistochemistry subsequently revealed hepatic PEComa.Patients with hepatic PEComa which is misdiagnosed as HCC often require a wider surgical resection.It is easy to mistake them for distant metastases of hepatic PEComa and misdiagnosed as HCC,especially when it’s combined with tumors in other organs.Three patients eventually underwent partial hepatectomy.After 1-4 years of follow-up,none of the patients experienced recurrence or metastases.CONCLUSION A clear preoperative diagnosis of hepatic PEComa can reduce the scope of resection and prevent unnecessary injuries during surgery.展开更多
Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors(PEComas)because PEComas are mainly benign tumors and may not require surgical intervention.By analyzing the...Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors(PEComas)because PEComas are mainly benign tumors and may not require surgical intervention.By analyzing the causes,properties and clinical manifestations of PEComas,we summarize the challenges and solutions in the diagnosis of PEComas.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells,has a low incidence,and can involve multiple organs.P...BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells,has a low incidence,and can involve multiple organs.PEComa originating in the liver is extremely rare,with most cases being benign,and only a few cases are malignant.Good outcomes are achieved with radical surgical resection,but there is no effective treatment for some large tumors and specific locations that are contraindicated for surgery.CASE SUMMARY A 32-year-old woman was admitted to our hospital with a palpable abdominal mass and progressive deterioration since the previous month.An ultrasoundguided percutaneous liver aspiration biopsy was performed.Postoperative pathological immunohistochemical staining was HMB45,Melan-A,and smooth muscle actin positive.Perivascular epithelioid tumor was diagnosed.The tumor was large and could not be completely resected by surgery.Further digital subtraction angiography revealed a rich tumor blood supply,and interventional embolization followed by surgery was recommended.Finally,the patient underwent transarterial embolization(TAE)combined with sorafenib for four cycles.Angiography reexamination indicated no clear vascular staining of the tumor,and the tumor had shrunk.The patient was followed up for a short period of time,achieved a stable condition,and surgery was recommended.CONCLUSION Adjuvant combination treatment with TAE and sorafenib is safe and feasible as it shrinks the tumor preoperatively and facilitates surgery.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa)represents a group of rare mesenchymal tumors.PEComa can occur in many organs but is rare in the colorectum,especially in children.Furthermore,PEComa is a rare cau...BACKGROUND Perivascular epithelioid cell tumor(PEComa)represents a group of rare mesenchymal tumors.PEComa can occur in many organs but is rare in the colorectum,especially in children.Furthermore,PEComa is a rare cause of intussusception,the telescoping of a segment of the gastrointestinal tract into an adjacent one.We describe a rare case of pediatric PEComa complicated with intussusception and anal incarceration,and conduct a review of the current literature.CASE SUMMARY A 12-year-old girl presented with abdominal pain and abdominal ultrasound suggested intussusception.Endoscopic direct-vision intussusception treatment and colonoscopy was performed.A spherical tumor was discovered in the transverse colon and removed by surgery.Postoperative pathologic analyses revealed that the tumor volume was 5.0 cm×4.5 cm×3.0 cm and the tumor tissue was located in the submucosa of the colon,arranged in an alveolar pattern.The cell morphology was regular,no neoplastic necrosis was observed,and nuclear fission was rare.The immunohistochemical staining results were as follows:Human melanoma black 45(HMB 45)(+),cluster of differentiation 31(CD31)(+),cytokeratin(-),melanoma-associated antigen recognized by T cells(-),smooth muscle actin(-),molleya(-),desmin(-),S-100(-),CD117(-),and Ki67(positive rate in hot spot<5%).Combined with the results of pathology and immunohistochemistry,we diagnosed the tumor as PEComa.Postoperative recovery was good at the 4 mo follow-up.CONCLUSION The diagnosis of PEComa mainly depends on pathology and immunohistochemistry.Radical resection is the preferred treatment method.展开更多
The hormone defined serum free conditioned medium (SFCM) of human nasopharyngeal carcinoma epithelioid cell line (CNE1) was assayed by both the 3H-thymidine incorporation test and the soft agar test. It was found that...The hormone defined serum free conditioned medium (SFCM) of human nasopharyngeal carcinoma epithelioid cell line (CNE1) was assayed by both the 3H-thymidine incorporation test and the soft agar test. It was found that the SFCM stimulated the growth of long-term serum-free cultured CNE4 cells in ac-cordence with the fact that the growth rate of long-term serum-free cultured CNE1 cells was directly proportional to the plating density. Alternatively 5% SFCM inhibited the growth of short-term serum-free cultured CNE4 cells by 51% in which the indicator cell remained the responsiveness state of growing in the serum-supplemented medium to the effector of interest. Furthermore, SFCM resulted in the inhibition of anchorage-independent growth of CNE4 cells and A431 cells. Also in soft agar test. SFCM reduced the colony formation of NRK(?),9F cells in the presence of EGF or EGF plus TGF-β. These finding suggested that CNE4 secreted autocrine growth stimulating factor(s) and growth inhibiting factor(s) in the serum-free medium, the latter strongly reverse malignant phenotypes of CNE4 and A431 cells in serum-supplemented surrounding.展开更多
文摘Malignant vascular tumors of the liver include rare primary hepatic mesenchymal tumors developed in the background of a normal liver parenchyma. Most of them are detected incidentally by the increased use of performing imaging techniques. Their diagnosis is challenging, involving clinical and imaging criteria, with final confirmation by histology and immunohistochemistry. Surgery represents the mainstay of treatment. Liver transplantation(LT) has improved substantially the prognosis of hepatic epithelioid hemangioendothelioma(HEHE), with 5-year patient survival rates of up to 81%, based on the European Liver Intestine Transplantation AssociationEuropean Liver Transplant Registry study. Unfortunately, the results of surgery and LT are dismal in cases of hepatic angiosarcoma(HAS). Due to the disappointing results of very short survival periods of approximately 6-7 mo after LT, because of tumor recurrence and rapid progression of the disease, HAS is considered an absolute contraindication to LT. Recurrences after surgical resection are high in cases of HEHE and invariably present in cases of HAS. The discovery of reliable prognostic markers and the elaboration of prognostic scores following LT are needed to provide the best therapeutic choice for each patient.Studies on a few patients have demonstrated the stabilization of the disease in a proportion of patients with hepatic vascular tumors using novel targeted antiangiogenic agents, cytokines or immunotherapy. These new approaches,alone or in combination with other therapeutic modalities, such as surgery and classical chemotherapy, need further investigation to assess their role in prolonging patient survival. Personalized therapeutic algorithms according to the histopathological features, behavior, molecular biology and genetics of the tumors should be elaborated in the near future for the management of patients diagnosed with primary malignant vascular tumors of the liver.
基金This work was supported by Zhejiang Natural Science Foundation(LQ20H030005)Zhejiang Health Technology Project(2020KY126/2019RC153).
文摘Background and Aims:Perivascular epithelioid cell neo-plasms(PEComas)are a rare type of mesenchymal neo-plasm and their preoperative diagnosis is challenging.In this study,we summarized the experience from a single medical center to study the examinations,clinical presen-tations,and pathological and histological characteristics of PEComas in the liver in order to optimize overall un-derstanding of the diagnosis and treatment of these neo-plasms.Methods:We conducted a retrospective analysis to investigate the clinical and pathological characteristics as well as imaging presentations of 75 patients diagnosed with hepatic PEComa in The First Affiliated Hospital of Zhe-jiang University between April 2010 and April 2020.Re-sults:Among the 75 patients,52 were women,and the median age was 48 years.Most patients had no specific symptoms,and two were admitted to the hospital for a second time owing to relapse.All patients underwent surgi-cal resection.Histologically,38 patients had classical angio-myolipoma(AML)and 37 had epithelioid AML.The PECo-mas were accompanied by positive immunohistochemical expression of HMB45,Melan-A,and smooth muscle actin.Follow-up data were obtained from 47 of the total 75 pa-tients,through October 2020.Two patients had metastasis after surgery.Conclusions:AML is the most common type of hepatic PEComa.There are no specific symptoms of he-patic PEComa,and serological examinations and imaging modalities for accurate preoperative diagnosis are lacking.Epithelioid AML should be considered a tumor of uncertain malignant potential;however,the prognosis of PEComa af-ter resection is promising.
文摘BACKGROUND Hepatic perivascular epithelioid cell neoplasms(PEComas)are rare.Diagnostic and treatment experience with hepatic PEComa remains insufficient.CASE SUMMARY Three hepatic PEComa cases are reported in this paper:One case of primary malignant hepatic PEComa,one case of benign hepatic PEComa,and one case of hepatic PEComa with an ovarian mature cystic teratoma.During preoperative imaging and pathological assessment of intraoperative frozen samples,patients were diagnosed with hepatocellular carcinoma(HCC),while postoperative pathology and immunohistochemistry subsequently revealed hepatic PEComa.Patients with hepatic PEComa which is misdiagnosed as HCC often require a wider surgical resection.It is easy to mistake them for distant metastases of hepatic PEComa and misdiagnosed as HCC,especially when it’s combined with tumors in other organs.Three patients eventually underwent partial hepatectomy.After 1-4 years of follow-up,none of the patients experienced recurrence or metastases.CONCLUSION A clear preoperative diagnosis of hepatic PEComa can reduce the scope of resection and prevent unnecessary injuries during surgery.
基金Supported by Nantong Municipal Health Commission,No.MSZ2022036.
文摘Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors(PEComas)because PEComas are mainly benign tumors and may not require surgical intervention.By analyzing the causes,properties and clinical manifestations of PEComas,we summarize the challenges and solutions in the diagnosis of PEComas.
基金Supported by Gansu Provincial Natural Science Foundation,No.21JR7RA417Lanzhou Science and Technology Development guiding Plan Project,No.2019-ZD-72.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells,has a low incidence,and can involve multiple organs.PEComa originating in the liver is extremely rare,with most cases being benign,and only a few cases are malignant.Good outcomes are achieved with radical surgical resection,but there is no effective treatment for some large tumors and specific locations that are contraindicated for surgery.CASE SUMMARY A 32-year-old woman was admitted to our hospital with a palpable abdominal mass and progressive deterioration since the previous month.An ultrasoundguided percutaneous liver aspiration biopsy was performed.Postoperative pathological immunohistochemical staining was HMB45,Melan-A,and smooth muscle actin positive.Perivascular epithelioid tumor was diagnosed.The tumor was large and could not be completely resected by surgery.Further digital subtraction angiography revealed a rich tumor blood supply,and interventional embolization followed by surgery was recommended.Finally,the patient underwent transarterial embolization(TAE)combined with sorafenib for four cycles.Angiography reexamination indicated no clear vascular staining of the tumor,and the tumor had shrunk.The patient was followed up for a short period of time,achieved a stable condition,and surgery was recommended.CONCLUSION Adjuvant combination treatment with TAE and sorafenib is safe and feasible as it shrinks the tumor preoperatively and facilitates surgery.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)represents a group of rare mesenchymal tumors.PEComa can occur in many organs but is rare in the colorectum,especially in children.Furthermore,PEComa is a rare cause of intussusception,the telescoping of a segment of the gastrointestinal tract into an adjacent one.We describe a rare case of pediatric PEComa complicated with intussusception and anal incarceration,and conduct a review of the current literature.CASE SUMMARY A 12-year-old girl presented with abdominal pain and abdominal ultrasound suggested intussusception.Endoscopic direct-vision intussusception treatment and colonoscopy was performed.A spherical tumor was discovered in the transverse colon and removed by surgery.Postoperative pathologic analyses revealed that the tumor volume was 5.0 cm×4.5 cm×3.0 cm and the tumor tissue was located in the submucosa of the colon,arranged in an alveolar pattern.The cell morphology was regular,no neoplastic necrosis was observed,and nuclear fission was rare.The immunohistochemical staining results were as follows:Human melanoma black 45(HMB 45)(+),cluster of differentiation 31(CD31)(+),cytokeratin(-),melanoma-associated antigen recognized by T cells(-),smooth muscle actin(-),molleya(-),desmin(-),S-100(-),CD117(-),and Ki67(positive rate in hot spot<5%).Combined with the results of pathology and immunohistochemistry,we diagnosed the tumor as PEComa.Postoperative recovery was good at the 4 mo follow-up.CONCLUSION The diagnosis of PEComa mainly depends on pathology and immunohistochemistry.Radical resection is the preferred treatment method.
文摘The hormone defined serum free conditioned medium (SFCM) of human nasopharyngeal carcinoma epithelioid cell line (CNE1) was assayed by both the 3H-thymidine incorporation test and the soft agar test. It was found that the SFCM stimulated the growth of long-term serum-free cultured CNE4 cells in ac-cordence with the fact that the growth rate of long-term serum-free cultured CNE1 cells was directly proportional to the plating density. Alternatively 5% SFCM inhibited the growth of short-term serum-free cultured CNE4 cells by 51% in which the indicator cell remained the responsiveness state of growing in the serum-supplemented medium to the effector of interest. Furthermore, SFCM resulted in the inhibition of anchorage-independent growth of CNE4 cells and A431 cells. Also in soft agar test. SFCM reduced the colony formation of NRK(?),9F cells in the presence of EGF or EGF plus TGF-β. These finding suggested that CNE4 secreted autocrine growth stimulating factor(s) and growth inhibiting factor(s) in the serum-free medium, the latter strongly reverse malignant phenotypes of CNE4 and A431 cells in serum-supplemented surrounding.
