Department of Precision Medicine, University of Campania L. Vanvitelli, Naples, Italy.
Pathology Unit, Department of Mental and Physical Health and Preventive Medicine, University of Campania L. Vanvitelli, Naples, Italy.
Am J Case Rep. 2020 Nov 15;21:e923505. doi: 10.12659/AJCR.923505.
BACKGROUND Langerhans cell histiocytosis (LCH), also called histiocytosis X, belongs to a group of rare neoplasms and is a clonal pathology characterized by infiltration of Langerhans cells. The pathology can occur with the involvement of only 1 organ, more frequently the bone or with multi-visceral involvement, and patients frequently receive a delayed diagnosis and empirical treatments. CASE REPORT We report a case of LCH in a 60-year-old woman who presented atypical symptoms, imaging findings of lung and liver involvement. Imaging showed increased liver volume and subverted structure by multiple nodular formations. For the differential diagnosis with other neoplastic liver diseases, the patient underwent liver biopsy, with microscopic typical findings of the disease and positive immunohistochemical markers. CONCLUSIONS Liver involvement in LCH is rare and the differential diagnosis with neoplastic pathology may pose a challenge for the clinician and radiologist, also due to the possible association between LCH and malignant tumors.
朗格汉斯细胞组织细胞增生症(LCH),又称组织细胞增生症 X,属于一组罕见的肿瘤,是一种以朗格汉斯细胞浸润为特征的克隆性疾病。该病理学表现为仅累及 1 个器官,更常见的是骨骼,或多脏器受累,患者经常被延误诊断并接受经验性治疗。
我们报告了一例 60 岁女性 LCH 病例,该患者表现出非典型症状、肺部和肝脏受累的影像学表现。影像学显示肝脏体积增大,结构倒置,由多个结节形成。为了与其他肝脏肿瘤性疾病进行鉴别诊断,患者接受了肝脏活检,显微镜下具有该病的典型表现和免疫组化标志物阳性。
LCH 肝脏受累罕见,与肿瘤性病变的鉴别诊断可能对临床医生和放射科医生构成挑战,也可能与 LCH 和恶性肿瘤之间的关联有关。