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
Pulmonary lymphangioleiomyomatosis(LAM)is a rare cystic lung disease that targets women during their reproductive years.A confident diagnosis can often be based on clinical grounds,but diagnostic certainty requires pa...Pulmonary lymphangioleiomyomatosis(LAM)is a rare cystic lung disease that targets women during their reproductive years.A confident diagnosis can often be based on clinical grounds,but diagnostic certainty requires pathological analysis.Although surgical lung biopsy is considered the gold standard for obtaining tissue in patients with diffuse lung disease,it is also associated with higher morbidity and mortality than alternative,less invasive techniques.The objective of our study was to examine the utility of transbronchial biopsy in the diagnosis of LAM.We conducted two online surveys of over 1000 LAM patients registered with the LAM Foundation who were accessible by email.Transbronchial biopsy specimens were subsequently collected and reviewed by an expert pathologist to validate the diagnosis.We found that transbronchial biopsy has a yield of approximately 60% in patients with LAM.We conclude that transbronchial biopsy may be a safe and effective method for establishing the diagnosis of LAM,obviating the need for surgical lung biopsy in more than half of LAM patients.展开更多
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.
文摘Pulmonary lymphangioleiomyomatosis(LAM)is a rare cystic lung disease that targets women during their reproductive years.A confident diagnosis can often be based on clinical grounds,but diagnostic certainty requires pathological analysis.Although surgical lung biopsy is considered the gold standard for obtaining tissue in patients with diffuse lung disease,it is also associated with higher morbidity and mortality than alternative,less invasive techniques.The objective of our study was to examine the utility of transbronchial biopsy in the diagnosis of LAM.We conducted two online surveys of over 1000 LAM patients registered with the LAM Foundation who were accessible by email.Transbronchial biopsy specimens were subsequently collected and reviewed by an expert pathologist to validate the diagnosis.We found that transbronchial biopsy has a yield of approximately 60% in patients with LAM.We conclude that transbronchial biopsy may be a safe and effective method for establishing the diagnosis of LAM,obviating the need for surgical lung biopsy in more than half of LAM patients.
