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
Background:Pancreatic perivascular epithelioid cell tumors(PEComas)are exceedingly uncommon mesenchymal tumors.Herein we have mainly focused on the radiologic features of pancreatic PEComas to achieve a better knowled...Background:Pancreatic perivascular epithelioid cell tumors(PEComas)are exceedingly uncommon mesenchymal tumors.Herein we have mainly focused on the radiologic features of pancreatic PEComas to achieve a better knowledge of the disease.Case report:We present the case of a 41-year-old woman who had a benign pancreatic PEComa that was misinterpreted as a malignant tumor based on a preoperative CT scan before undergoing surgery for a hysteromyoma.Laboratory tests revealed no significant abnormal findings except an elevated CA-125(428 U/L).CT and MRI images showed a solid,well-defined,hypovascular nodule approximately 10 mm in diameter in the pancreatic uncinate process that was hyperintense on diffusion-weighted imaging(DWI)and hypointense on apparent diffusion coefficient(ADC)maps.Because of a suspected malignant pancreatic neoplasm,she underwent a pancreaticoduodenectomy.The pathologic examination confirmed the diagnosis of a pancreatic PEComa.Conclusions:Because of the rarity of pancreatic PEComas,we highlighted the radiologic characteristics to facilitate the diagnosis and therapy.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Background:Pancreatic perivascular epithelioid cell tumors(PEComas)are exceedingly uncommon mesenchymal tumors.Herein we have mainly focused on the radiologic features of pancreatic PEComas to achieve a better knowledge of the disease.Case report:We present the case of a 41-year-old woman who had a benign pancreatic PEComa that was misinterpreted as a malignant tumor based on a preoperative CT scan before undergoing surgery for a hysteromyoma.Laboratory tests revealed no significant abnormal findings except an elevated CA-125(428 U/L).CT and MRI images showed a solid,well-defined,hypovascular nodule approximately 10 mm in diameter in the pancreatic uncinate process that was hyperintense on diffusion-weighted imaging(DWI)and hypointense on apparent diffusion coefficient(ADC)maps.Because of a suspected malignant pancreatic neoplasm,she underwent a pancreaticoduodenectomy.The pathologic examination confirmed the diagnosis of a pancreatic PEComa.Conclusions:Because of the rarity of pancreatic PEComas,we highlighted the radiologic characteristics to facilitate the diagnosis and therapy.
