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 rare mesenchymal neoplasm that predominantly affects the kidney and uterus.The occurrence of this tumor in the liver,particularly with simultaneous involvemen...BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus.The occurrence of this tumor in the liver,particularly with simultaneous involvement of the liver and kidney,is exceedingly uncommon.Pathological diagnosis is the gold standard.PEComas usually show positive immunohistochemical staining for melanocytic(HMB-45,Melan-A)and myoid(SMA,muscle-specific actin)markers.CASE SUMMARY We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex(TSC).FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45,Melan A,and TFE3 were detected.In addition,we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa,while surgery remained the most effective approach.Everolimus showed an excellent efficacy in the postoperative treatment of the tumor.CONCLUSION Surgical treatment is preferred for malignant PEComa affecting liver and kidney,especially with TSC;everolimus is effective postoperatively.展开更多
Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hep...Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues including smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant （double-positive for CD34 and Ki-67） and recurred 3 months after surgery. In addition, malignant hepatic PECo- mas were reviewed in the literature, indicating that the majority of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients （〉50 years of age） with abdominal discomfort and pain, larger tumor size （〉10 cm）, or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohistochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa)is an uncommon tumor of mesenchymal origin.Cases of PEComa in the liver are extremely rare.AIM To analyze the clinicopathological features and treatment of hepatic...BACKGROUND Perivascular epithelioid cell tumor(PEComa)is an uncommon tumor of mesenchymal origin.Cases of PEComa in the liver are extremely rare.AIM To analyze the clinicopathological features and treatment of hepatic PEComa and to evaluate the prognosis after different treatments.METHODS Clinical and pathological data of 26 patients with hepatic PEComa were collected.All cases were analyzed by immunohistochemistry and clinical follow-up.RESULTS This study included 17 females and 9 males,with a median age of 50 years.Lesions were located in the left hepatic lobe in 13 cases,in the right lobe in 11,and in the caudate lobe in 2.The median tumor diameter was 6.5 cm.Light microscopy revealed that the tumor cells were mainly composed of epithelioid cells.The cytoplasm contained heterogeneous eosinophilic granules.There were thick-walled blood vessels,around which tumor cells were radially arranged.Immunohistochemical analysis of pigment-derived and myogenic markers in PEComas revealed that 25 cases were HMB45(+),23 were Melan-A(+),and 22 SMA(+).TFE3 and Desmin were negative in all cases.All the fluorescence in situ hybridization samples were negative for TFE3 gene break-apart probe.Tumor tissues were collected by extended hepatic lobe resection or simple hepatic tumor resection as the main treatments.Median follow-up was 62.5 mo.None of the patients had metastasis or recurrence,and there were no deaths due to the disease.CONCLUSION Hepatic PEComa highly expresses melanin and smooth muscle markers,and generally exhibits an inert biological behavior.The prognosis after extended hepatic lobe resection and simple hepatic tumor resection is semblable.展开更多
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 Cuiying Scientific and Technological Innovation Program of Lanzhou University Second Hospital,No.CY2022-QN-A13 and No.CY2023-QN-A02Lanzhou Science and Technology Plan Project,No.2023-4-26+1 种基金Gansu Provincial Natural Science Foundation Project,No.23JRRA1630 and No.24JRRA331Gansu Provincial Health Research Program Project,No.GSWSKY-2019-32.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus.The occurrence of this tumor in the liver,particularly with simultaneous involvement of the liver and kidney,is exceedingly uncommon.Pathological diagnosis is the gold standard.PEComas usually show positive immunohistochemical staining for melanocytic(HMB-45,Melan-A)and myoid(SMA,muscle-specific actin)markers.CASE SUMMARY We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex(TSC).FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45,Melan A,and TFE3 were detected.In addition,we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa,while surgery remained the most effective approach.Everolimus showed an excellent efficacy in the postoperative treatment of the tumor.CONCLUSION Surgical treatment is preferred for malignant PEComa affecting liver and kidney,especially with TSC;everolimus is effective postoperatively.
基金supported by grants from the Foundation of Jiangsu Collaborative Innovation Center of Biomedical Functional Materials,Basic Research Program-Youth Fund Project of Jiangsu Province(BK20140092)the National Natural Science Foundation of China(81400650,81273261 and 81270583)
文摘Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues including smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant （double-positive for CD34 and Ki-67） and recurred 3 months after surgery. In addition, malignant hepatic PECo- mas were reviewed in the literature, indicating that the majority of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients （〉50 years of age） with abdominal discomfort and pain, larger tumor size （〉10 cm）, or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohistochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.
基金Supported by the Anhui Provincial Natural Science Foundation,No.1908085MH275Bengbu Medical College Key projects of Natural Science Foundation,No.BYKF1710Bengbu City-Bengbu Medical College Joint Science and Technology Project,No.BYLK201812.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)is an uncommon tumor of mesenchymal origin.Cases of PEComa in the liver are extremely rare.AIM To analyze the clinicopathological features and treatment of hepatic PEComa and to evaluate the prognosis after different treatments.METHODS Clinical and pathological data of 26 patients with hepatic PEComa were collected.All cases were analyzed by immunohistochemistry and clinical follow-up.RESULTS This study included 17 females and 9 males,with a median age of 50 years.Lesions were located in the left hepatic lobe in 13 cases,in the right lobe in 11,and in the caudate lobe in 2.The median tumor diameter was 6.5 cm.Light microscopy revealed that the tumor cells were mainly composed of epithelioid cells.The cytoplasm contained heterogeneous eosinophilic granules.There were thick-walled blood vessels,around which tumor cells were radially arranged.Immunohistochemical analysis of pigment-derived and myogenic markers in PEComas revealed that 25 cases were HMB45(+),23 were Melan-A(+),and 22 SMA(+).TFE3 and Desmin were negative in all cases.All the fluorescence in situ hybridization samples were negative for TFE3 gene break-apart probe.Tumor tissues were collected by extended hepatic lobe resection or simple hepatic tumor resection as the main treatments.Median follow-up was 62.5 mo.None of the patients had metastasis or recurrence,and there were no deaths due to the disease.CONCLUSION Hepatic PEComa highly expresses melanin and smooth muscle markers,and generally exhibits an inert biological behavior.The prognosis after extended hepatic lobe resection and simple hepatic tumor resection is semblable.
