目的报道1例在鞍区精原细胞瘤放化疗后出现C型尼曼-匹克病(Niemann-Pick disease type C,NPC)的病例,加强对尼曼-匹克病(Niemann-Pick disease,NPD)的了解。方法对南部战区总医院内分泌科2021年6月收治的1例鞍区精原细胞瘤切除术、放化...目的报道1例在鞍区精原细胞瘤放化疗后出现C型尼曼-匹克病(Niemann-Pick disease type C,NPC)的病例,加强对尼曼-匹克病(Niemann-Pick disease,NPD)的了解。方法对南部战区总医院内分泌科2021年6月收治的1例鞍区精原细胞瘤切除术、放化疗后逐渐出现垂体功能减退、重度肝损害、肝脾大,激素替代治疗后引起继发性糖尿病的青少年患者长达4年的病史进行回顾性分析。结果本例患者为青少年女性,鞍区精原细胞瘤切除术及放化疗后,逐渐出现不同程度血细胞下降、脂代谢异常、肝功能异常、肝脾增大等,行骨髓穿刺,形态学发现2%尼曼-匹克细胞,最终诊断为NPC。经输血、护肝、胰岛素降糖、激素替代等对症治疗后,各项指标较治疗前好转,精神较入院时稍好转,但患者仍存在黄疸、肝脾大,偶有精神异常。目前继续门诊随访,予对症治疗。结论对于颅内肿瘤术后或放化疗后所致内分泌与代谢相关症状,且同时伴有难以解释的神经、精神、脏器功能异常时,要考虑到NPC的可能性,可通过骨髓穿刺涂片发现尼曼-匹克细胞初步诊断。目前NPC的治疗以对症为主。展开更多
BACKGROUND Solitary necrotic nodule of the liver(SNNL)is a rare benign lesion with a complete necrotic core and a clear fibrous capsule containing elastic fibers.We present the case of a patient with a radiographic co...BACKGROUND Solitary necrotic nodule of the liver(SNNL)is a rare benign lesion with a complete necrotic core and a clear fibrous capsule containing elastic fibers.We present the case of a patient with a radiographic computed tomography(CT)finding of"ring"-like annular calcification within the lesion and postoperative pathologic diagnosis of necrotic nodules wrapped by dense fibers in liver tissue,as well as the patient's subsequent management and outcome.CASE SUMMARY A 38-year-old Chinese woman with a history of systemic lupus erythematosus treated with prednisone and hydroxychloroquine,without any symptoms,was found to have hepatic space-occupying lesions by imaging examination at a health examination.A subsequent CT scan suggested a space-occupying lesion of the liver with annular calcification,which was not defined to be benign or malignant.After that,a laparoscopic hepatic space-occupying resection was performed.The postoperative pathological diagnosis was necrotic nodules wrapped by dense fibers in the liver tissue,and the final diagnosis was SNNL.The patient had an uneventful postoperative recovery.CONCLUSION There is a"ring"-like calcification in SNNL.This patient had a history of systemic lupus erythematosus,without a history of parasite infection,trauma,or tumor.Therefore,whether the etiology and pathological changes of SNNL are related to rheumatic immune diseases remains to be investigated.展开更多
文摘目的报道1例在鞍区精原细胞瘤放化疗后出现C型尼曼-匹克病(Niemann-Pick disease type C,NPC)的病例,加强对尼曼-匹克病(Niemann-Pick disease,NPD)的了解。方法对南部战区总医院内分泌科2021年6月收治的1例鞍区精原细胞瘤切除术、放化疗后逐渐出现垂体功能减退、重度肝损害、肝脾大,激素替代治疗后引起继发性糖尿病的青少年患者长达4年的病史进行回顾性分析。结果本例患者为青少年女性,鞍区精原细胞瘤切除术及放化疗后,逐渐出现不同程度血细胞下降、脂代谢异常、肝功能异常、肝脾增大等,行骨髓穿刺,形态学发现2%尼曼-匹克细胞,最终诊断为NPC。经输血、护肝、胰岛素降糖、激素替代等对症治疗后,各项指标较治疗前好转,精神较入院时稍好转,但患者仍存在黄疸、肝脾大,偶有精神异常。目前继续门诊随访,予对症治疗。结论对于颅内肿瘤术后或放化疗后所致内分泌与代谢相关症状,且同时伴有难以解释的神经、精神、脏器功能异常时,要考虑到NPC的可能性,可通过骨髓穿刺涂片发现尼曼-匹克细胞初步诊断。目前NPC的治疗以对症为主。
文摘BACKGROUND Solitary necrotic nodule of the liver(SNNL)is a rare benign lesion with a complete necrotic core and a clear fibrous capsule containing elastic fibers.We present the case of a patient with a radiographic computed tomography(CT)finding of"ring"-like annular calcification within the lesion and postoperative pathologic diagnosis of necrotic nodules wrapped by dense fibers in liver tissue,as well as the patient's subsequent management and outcome.CASE SUMMARY A 38-year-old Chinese woman with a history of systemic lupus erythematosus treated with prednisone and hydroxychloroquine,without any symptoms,was found to have hepatic space-occupying lesions by imaging examination at a health examination.A subsequent CT scan suggested a space-occupying lesion of the liver with annular calcification,which was not defined to be benign or malignant.After that,a laparoscopic hepatic space-occupying resection was performed.The postoperative pathological diagnosis was necrotic nodules wrapped by dense fibers in the liver tissue,and the final diagnosis was SNNL.The patient had an uneventful postoperative recovery.CONCLUSION There is a"ring"-like calcification in SNNL.This patient had a history of systemic lupus erythematosus,without a history of parasite infection,trauma,or tumor.Therefore,whether the etiology and pathological changes of SNNL are related to rheumatic immune diseases remains to be investigated.
