PDF(Pubmed)
Abstract：
UNASSIGNED: Hepatic Inflammatory Pseudotumor (IPT) is an infrequent condition often masquerading as a malignant tumor, resulting in misdiagnosis and unnecessary surgical resection. The emerging concept of IgG4-related diseases (IgG4-RD) has gained widespread recognition, encompassing entities like IgG4-related hepatic IPT. Clinically and radiologically, corticosteroids and immunosuppressive therapies have proven effective in managing this condition.
UNASSIGNED: A 3-year-old Chinese boy presented to the clinic with an 11-month history of anemia, fever of unknown origin, and a tender hepatic mass. Blood examinations revealed chronic anemia (Hb: 6.4 g/L, MCV: 68.6 fl, MCH: 19.5 pg, reticulocytes: 1.7%) accompanied by an inflammatory reaction and an elevated serum IgG4 level (1542.2 mg/L). Abdominal contrast-enhanced computed tomography unveiled a 7.6 cm low-density mass in the right lateral lobe, while magnetic resonance imaging demonstrated slight hypointensity on T1-weighted images and slight hyperintensity on T2-weighted images, prompting suspicion of hepatic malignancy. A subsequent liver biopsy revealed a mass characterized by fibrous stroma and dense lymphoplasmacytic infiltration. Immunohistochemical analysis confirmed the presence of IgG4-positive plasma cells, leading to the diagnosis of IgG4-related hepatic IPT. Swift resolution occurred upon initiation of corticosteroid and mycophenolate mofetil therapies.
UNASSIGNED: This study underscores the diagnostic approach to hepatic IPT, utilizing histopathology, immunostaining, imaging, serology, organ involvement, and therapeutic response. Early histological examination plays a pivotal role in clinical guidance, averting misdiagnosis as a liver tumor and unnecessary surgical interventions.
UNASSIGNED: A 3-year-old Chinese boy presented to the clinic with an 11-month history of anemia, fever of unknown origin, and a tender hepatic mass. Blood examinations revealed chronic anemia (Hb: 6.4 g/L, MCV: 68.6 fl, MCH: 19.5 pg, reticulocytes: 1.7%) accompanied by an inflammatory reaction and an elevated serum IgG4 level (1542.2 mg/L). Abdominal contrast-enhanced computed tomography unveiled a 7.6 cm low-density mass in the right lateral lobe, while magnetic resonance imaging demonstrated slight hypointensity on T1-weighted images and slight hyperintensity on T2-weighted images, prompting suspicion of hepatic malignancy. A subsequent liver biopsy revealed a mass characterized by fibrous stroma and dense lymphoplasmacytic infiltration. Immunohistochemical analysis confirmed the presence of IgG4-positive plasma cells, leading to the diagnosis of IgG4-related hepatic IPT. Swift resolution occurred upon initiation of corticosteroid and mycophenolate mofetil therapies.
UNASSIGNED: This study underscores the diagnostic approach to hepatic IPT, utilizing histopathology, immunostaining, imaging, serology, organ involvement, and therapeutic response. Early histological examination plays a pivotal role in clinical guidance, averting misdiagnosis as a liver tumor and unnecessary surgical interventions.
摘要：
■肝脏炎性假瘤（IPT）是一种罕见的疾病，通常伪装成恶性肿瘤，导致误诊和不必要的手术切除。IgG4相关疾病（IgG4-RD）的新兴概念已获得广泛认可，涵盖IgG4相关肝IPT等实体。临床和放射学，皮质类固醇和免疫抑制疗法已被证明可以有效控制这种情况。
■一个3岁的中国男孩出现在诊所，有11个月的贫血史，不明原因的发烧，还有一个细嫩的肝脏肿块.血液检查显示慢性贫血（Hb：6.4g/L，MCV:68.6fl,MCH:19.5pg,网织红细胞：1.7％）伴有炎症反应和血清IgG4水平升高（1542.2mg/L）。腹部对比增强计算机断层扫描显示右侧叶有一个7.6厘米的低密度肿块，磁共振成像在T1加权图像上显示出轻微的低强度，在T2加权图像上显示出轻微的高强度，提示怀疑肝脏恶性肿瘤。随后的肝活检显示肿块，其特征是纤维基质和密集的淋巴浆细胞浸润。免疫组织化学分析证实了IgG4阳性浆细胞的存在，导致IgG4相关性肝IPT的诊断。在开始使用皮质类固醇和霉酚酸酯治疗后迅速消退。
■本研究强调了肝IPT的诊断方法，利用组织病理学，免疫染色，成像,血清学,器官受累,和治疗反应。早期组织学检查在临床指导中起着举足轻重的作用,避免误诊为肝肿瘤和不必要的手术干预。
■一个3岁的中国男孩出现在诊所，有11个月的贫血史，不明原因的发烧，还有一个细嫩的肝脏肿块.血液检查显示慢性贫血（Hb：6.4g/L，MCV:68.6fl,MCH:19.5pg,网织红细胞：1.7％）伴有炎症反应和血清IgG4水平升高（1542.2mg/L）。腹部对比增强计算机断层扫描显示右侧叶有一个7.6厘米的低密度肿块，磁共振成像在T1加权图像上显示出轻微的低强度，在T2加权图像上显示出轻微的高强度，提示怀疑肝脏恶性肿瘤。随后的肝活检显示肿块，其特征是纤维基质和密集的淋巴浆细胞浸润。免疫组织化学分析证实了IgG4阳性浆细胞的存在，导致IgG4相关性肝IPT的诊断。在开始使用皮质类固醇和霉酚酸酯治疗后迅速消退。
■本研究强调了肝IPT的诊断方法，利用组织病理学，免疫染色，成像,血清学,器官受累,和治疗反应。早期组织学检查在临床指导中起着举足轻重的作用,避免误诊为肝肿瘤和不必要的手术干预。
