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Rosai–Dorfman disease is the most frequent variant of non-Langerhans cell histiocytosis. Local forms can be resected or irradiated. If the process involves multiple organs, systemic chemotherapy can cure some patients. This article includes literature review and a case report of a 34-year-old patient with multifocal, multisystemic form of Rosai–Dorfman disease with bone and pleural involvement. The diagnosis was based on histological, immunohistochemical, and molecular studies of tumor tissue. Since November 2021, 6 courses of chemotherapy with cladribine and 8 infusions of zolendronic acid were carried out with achievement of durable remission. The tolerance was acceptable.

Introduction and purpose: ​Rosai-Dorfman Disease (RDD), known also as sinus histiocytosis with massive lymphadenopathy(SHML) is a benign histiocytic proliferative syndrome. The etiology and pathogenesis of RDD remains unclear. Central nervous system involvement is a rare event and concerns approximately 7.8% of RDD cases, whereas intracranial lesions constitute almost 90% of CNS-RDD cases. The aim of this literature review was to summarize current knowledge about the diagnosis and treatment of intracranial manifestation of RDD. We also described possible hypotheses regarding the pathophysiology of this disorder. State of knowledge: ​Even though Rosai-Dorfman disease was thought to be a reactive process, recent evidencedemonstrate the presence of clonality, which means that in this histiocytosis the process that underlies the pathology is neoplastic. Intracranial lesions caused by RDD can be easily misdiagnosed with many diseases such as meningiomas, malignant gliomas or metastatic tumors. The final diagnosis of Rosai-Dorfman disease should be made based on histologic and immunohistochemical examinations. Current therapeutic options for this condition include surgery, radiotherapy, chemotherapy, corticosteroids and immunotherapy. Surgical treatment often constitutes the first-line treatment for intracranial RDD and is the most beneficial treatment option. However, the implementation of adjuvant therapies is very important to avoid the recurrence of lesions, which appear in approximately 14% of subjects after about 10 years from surgery. Conclusion: ​This literature review presents current data about pathophysiology, diagnosis and treatment of intracranial involvement of Rosai-Dorfman disease. Further studies on this topic should focus on exploring etiologic mechanisms underlying on this pathology and comparing available treatment methods.

Rosai–Dorfman disease or sinus histiocytosis is a rare entity which may affect various lymph node groups. Extranodal form is observed in 45 % of cases, facial bone involvement is especially rare. A 62-year old male presented with complaints on dumb sensation of the chin and lower lip. After clinical assessment and surgical exploration an intraosseous extranodal Rosai–Dorfman disease was diagnosed. The patient under-went free fibula osseous graft reconstruction. After 2 years the patient is recurrent free. The purpose of this publication is to describe a rare case of lower jaw involvement in an intraosseous form with significant bone destruction. The clinical and microscopic features of the process are discussed.

The paper describes a case of diffuse large B-cell lymphoma detected in a patient 13 months after sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) being diagnosed together with active hepatitis B virus infection. Analysis of their observations of patients with sinus histiocytosis and the data available in the literature allowed the authors to identify a few tens of cases with SHML associated with lymphomas. This case and previously described ones in the literature on the association of RDD and lymphomas may suggest with a high degree of probability that patients diagnosed as having RDD in evident lymphadenopathy and the non-typical clinical course of RDD may develop blood cancer.

In maxillofacial surgery, there are periodic cases of long-lasting chronic inflammation of the soft tissues without the positive effect of the conservative treatment. In these cases, questions arise of a detailed analysis of the etiology and pathogenesis of the disease, differential diagnosis of the pathological process, including those rarely encountered in the practice of a maxillofacial surgeon. One of these diseases is Rosai Dorfman's disease, which refers to extranodal histiocytosis. In domestic and foreign literature, single clinical observations are found without a detailed analysis of the diagnostic algorithm and the choice of treatment tactics. This article presents a clinical case of Rosai-Dorfman disease of a 55-year-old patient with a lesion of the soft tissues of the maxillofacial region. The described case took place in the clinic of maxillofacial surgery of the Research Institute of Dentistry of Surgical and Maxillofacial Surgery PSPbGMU named after academician I.P. Pavlova in the period from 2016 to 2019. Given the complexity of the diagnosis of this rare nosological form, we offer a specific examination algorithm that leads to a multidisciplinary comprehensive approach from disease verification to the choice of treatment tactics.

Rosai-Dorfman disease originally known as sinus histiocytosis with massive lymphadenopathy is a rare non-neoplastic condition of unexplored etiology characterized by a benign clinical picture. The present article summarizes the results of the literature publications and clinical observations of the extranodal form of Rosai-Dorfman disease manivfested as the lesions of mucous membranes of the nose and paranasal sinuses.

The paper describes a case of solitary epithelioid hemangioendothelioma concurrent with nodular parenchymal AL amyloidosis of the lung and Rosai-Dorfman disease in a 70-year-old woman. The core of the tumor was represented by bone tissue with dendriform ossification, as well as by amyloid that showed green apple birefringence at polarized light microscopy. The peripheral portions of the tumor and the myxohyaline stroma exhibited slit-like structures, epithelioid and fusiform cells with small cytoplasmic vacuoles. These cells expressed CD31, CD34, factor VIII, and cytokeratins 7 and 18. The Ki-67 proliferation index was 10%. S100- and CD68-positive histiocytes with the phenomenon of emperipolesis were revealed in the tumor and in the lymph nodes of the mediastinum and lung hilum. There was a positive reaction to immunoglobulin lambda light chains in the lymphocytic infiltration around amyloid clumps. The frequency of epithelioid hemangioendothelioma was less than 1 case per million people annually. We found only one case of its concurrence with pulmonary amyloidosis in the English-language literature. No relationship could be revealed between this tumor and Rosai-Dorfman disease.