Radiological Characteristics and Pathological Correlation Analysis of Extranodal Rosai-Dorfman Disease of liver

Xingxing Zheng ^1,2 Hongzhe Tian ¹ Wei Li ¹ Meihong Yang ¹ Chenwang Jin ^1* Yuhui Pang ^1*

• ¹ Baoji Central Hospital, Baoji, China
• ² Xi'an Jiaotong University, Xi'an, China

Background: Rosai Dorfman disease (RDD), also known as sinus histiocytosis with extensive lymph node involvement, is a rare histiocytosis with unknown etiology.Liver RDD is relatively rare and is often reported in individual cases. Exploring the imaging and pathological features of extrahepatic Rosai Dorfman disease (RDD) and conducting a comprehensive analysis of reported cases in domestic and foreign literature to enhance understanding of this rare disease.We collected data from a patient diagnosed with liver RDD in our hospital.In addition, we searched for liver RDD cases through PubMed and conducted a literature review.Of the patient data used in this study, 1 patient's data was obtained from our hospital records and 16 were retrieved from literature. There were 6 males and 11 females aged between 2-72 years, with an average age of 33.55 ± 19.38 years. Four patients presented with isolated liver nodules without the involvement of lymph nodes or extranodal organs, two patients presented with a case that involved extranodal organs but not lymph nodes, two patients presented with a case involving lymph nodes but not extranodal organs, eight patients presented with a case involving both lymph nodes and extranodal organs and lastly one patient presented with a case that did not involve either lymph nodes or extranodal organs. Four of the patients underwent enhanced scanning. The results revealed that 2 of them showed low enhancement, and the other 2 showed no significant enhancement. Only 1 case underwent PET-CT, and the results indicated a high uptake. Immunohistochemistry was performed on all 17 cases of liver RDD, and the results were positive for CD1a (-), CD68 (+), and S-100 (+).Conclusions: Liver RDD can manifest at any age. It presents with nonspecific imaging findings and a challenging preoperative diagnosis. Patients with extranodal hepatic RDD present with typical RDD characteristic immunohistochemistry features such as, high uptake on PET-CT, and no to mild enhancement on contrast-enhanced scans. For an early diagnosis, it is beneficial to fully comprehend these traits.