目的探讨肺具有血管周上皮样细胞分化的肿瘤(Neoplasms with perivascular epithelioid celldifferentiation,PEComa)的临床病理特征、免疫组化表达特征、鉴别诊断和预后。方法回顾我院今年年初收治的1例肺PEComa的临床病理组织学及免...目的探讨肺具有血管周上皮样细胞分化的肿瘤(Neoplasms with perivascular epithelioid celldifferentiation,PEComa)的临床病理特征、免疫组化表达特征、鉴别诊断和预后。方法回顾我院今年年初收治的1例肺PEComa的临床病理组织学及免疫表型进行分析,并复习相关文献。结果肺PEComa属间叶肿瘤,由具有组织学和免疫组化特征的血管周上皮样细胞组成,瘤细胞特征性表达黑色素生成标记物,瘤细胞胞浆PAS染色强阳性。结论肺PEComa临床治疗以手术完整切除肿块为主,多数病例预后良好,但部分病例可复发。展开更多
We describe a young female patient suffering from a PEComa (perivascular epithelioid cell tumor) of the cecum, incidentally found at an examination made by her family physician. The perivascular epithelioid cell tumor...We describe a young female patient suffering from a PEComa (perivascular epithelioid cell tumor) of the cecum, incidentally found at an examination made by her family physician. The perivascular epithelioid cell tumor is a very rare tumor, until today reported in a few cases in falciform ligament, uterus, jejunum, terminal ileum, rectum, liver, kidney, lung, pancreas, prostate, and soft tissue of the thigh. This tumor is part of a new group of tumors, comprised of angiomyolipoma, lymphangiomyolipoma, and clear cell myomelanocytic “sugar”tumor. Defined by coexpression of melanocytic (HMB-45)and muscle markers (smooth muscle actin and desmin) the perivascular epithelioid cell tumor does not have predictable histopathologic behavior. Some cases of metastasis are described, comorbidities such as tuberous sclerosis of the brain “Bourneville”and lymphangioleiomyomatosis have to be excluded. The therapy consists of the radical resection. An adjuvant therapy is not known. Recommended is a close and long term follow up clinically and by CT scan.展开更多
In 2013,the World Health Organization defined perivascular epithelioid cell tumor(PEComa)as“a mesenchymal tumor which shows a local association with vessel walls and usually expresses melanocyte and smooth muscle mar...In 2013,the World Health Organization defined perivascular epithelioid cell tumor(PEComa)as“a mesenchymal tumor which shows a local association with vessel walls and usually expresses melanocyte and smooth muscle markers.”This generic definition seems to better fit the PEComa family,which includes angiomyolipoma,clear cell sugar tumor of the lung,lymphangioleiomyomatosis,and a group of histologically and immunophenotypically similar tumors that include primary extrapulmonary sugar tumor and clear cell myomelanocytic tumor.Clear cell tumors with this immunophenotypic pattern have also had their malignant variants described.When localizing to the liver,preoperative radiological diagnosis has proven to be very difficult,and most patients have been diagnosed with hepatocellular carcinoma,focal nodular hyperplasia,hemangioma,or hepatic adenoma based on imaging findings.Examples of a malignant variant of the liver have been described.Finally,reports of malignant variants of these lesions have increased in recent years.Therefore,we support the use of the Folpe criteria,which in 2005 established the criteria for categorizing a PEComa as benign,malignant,or of uncertain malignant potential.Although they are not considered ideal,they currently seem to be the best approach and could be used for the categorization of liver tumors.展开更多
文摘目的探讨肺具有血管周上皮样细胞分化的肿瘤(Neoplasms with perivascular epithelioid celldifferentiation,PEComa)的临床病理特征、免疫组化表达特征、鉴别诊断和预后。方法回顾我院今年年初收治的1例肺PEComa的临床病理组织学及免疫表型进行分析,并复习相关文献。结果肺PEComa属间叶肿瘤,由具有组织学和免疫组化特征的血管周上皮样细胞组成,瘤细胞特征性表达黑色素生成标记物,瘤细胞胞浆PAS染色强阳性。结论肺PEComa临床治疗以手术完整切除肿块为主,多数病例预后良好,但部分病例可复发。
文摘We describe a young female patient suffering from a PEComa (perivascular epithelioid cell tumor) of the cecum, incidentally found at an examination made by her family physician. The perivascular epithelioid cell tumor is a very rare tumor, until today reported in a few cases in falciform ligament, uterus, jejunum, terminal ileum, rectum, liver, kidney, lung, pancreas, prostate, and soft tissue of the thigh. This tumor is part of a new group of tumors, comprised of angiomyolipoma, lymphangiomyolipoma, and clear cell myomelanocytic “sugar”tumor. Defined by coexpression of melanocytic (HMB-45)and muscle markers (smooth muscle actin and desmin) the perivascular epithelioid cell tumor does not have predictable histopathologic behavior. Some cases of metastasis are described, comorbidities such as tuberous sclerosis of the brain “Bourneville”and lymphangioleiomyomatosis have to be excluded. The therapy consists of the radical resection. An adjuvant therapy is not known. Recommended is a close and long term follow up clinically and by CT scan.
文摘In 2013,the World Health Organization defined perivascular epithelioid cell tumor(PEComa)as“a mesenchymal tumor which shows a local association with vessel walls and usually expresses melanocyte and smooth muscle markers.”This generic definition seems to better fit the PEComa family,which includes angiomyolipoma,clear cell sugar tumor of the lung,lymphangioleiomyomatosis,and a group of histologically and immunophenotypically similar tumors that include primary extrapulmonary sugar tumor and clear cell myomelanocytic tumor.Clear cell tumors with this immunophenotypic pattern have also had their malignant variants described.When localizing to the liver,preoperative radiological diagnosis has proven to be very difficult,and most patients have been diagnosed with hepatocellular carcinoma,focal nodular hyperplasia,hemangioma,or hepatic adenoma based on imaging findings.Examples of a malignant variant of the liver have been described.Finally,reports of malignant variants of these lesions have increased in recent years.Therefore,we support the use of the Folpe criteria,which in 2005 established the criteria for categorizing a PEComa as benign,malignant,or of uncertain malignant potential.Although they are not considered ideal,they currently seem to be the best approach and could be used for the categorization of liver tumors.
