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Rosai–Dorfman disease is a non–Langherans cell histiocytosis typically revealed by a lymphadenopathy. Central nervous system involvement is rare, exceptionally isolated, and usually consists of dural masses mimicking meningioma. Very few reports have described non-dural-based lesions, especially with an intra-ventricular development. We report hereby the case of a Rosai–Dorfman disease in a 30-year-old man presenting as an isolated mass arising from the right cerebellar peduncle and protruding into the fourth ventricle. We provide the results of the MRI examination with a special focus on advanced MRI features. As the diagnosis relies on pathological examination, we also detail the results of the analysis that followed the surgical resection of the mass including the immunohistochemical profile. This report highlights the necessity to consider Rosai–Dorfman disease as a potential diagnosis in case of an infra–tentorial mass and/or intra-ventricular mass.

Langerhans cell histiocytosis, Rosai-Dorfman disease, and juvenile xanthogranuloma may present at birth or any time afterward. Some patients have minimal skin or lymph node involvement, but others present with life-threatening pulmonary, hepatic, bone marrow, or central nervous system lesions. There is often a delay in diagnosis because of confusing overlap with more common neonatal diseases. Many treatment regimens have been applied to these diseases, but those directed at myeloid cells, such as cytarabine and clofarabine or mutation-targeting inhibitors, are gaining favor. This article provides information on the pathophysiology, clinical presentation, evaluation guidelines, and treatment of these uncommon tumors of neonates.

Rosai Dorfman disease (RDD) also known as sinus histiocytosis with massive lymphadenopathy (SHML) is a rare non-malignant proliferative disorder of unknown etiology. Here we present a case of Rosai Dorfman disease of the spine causing lumbo-sacral radiculopathy. CT and MRI revealed lytic expansile lesion in the left half of L4 vertebra mainly involving posterior aspect involving left pedicle and transverse process. Patient underwent surgery at L3-4 level by left fenestration. Histological examination confirmed the diagnosis of Rosai Dorfman disease. Skeletal involvement is a very rare presentation seen in 2% case with involvement of long bones including tibia, femur, humerus, clavicle and bones of hand. RDD affecting the spine has been rarely reported in literature. A team approach involving the radiologist, spine surgeon, pathologist and oncologist will lead to the early diagnosis and appropriate management of this rare clinical entity.

Background Rosai-Dorfman disease (RDD) is a rare idiopathic benign proliferative disorder of histiocytes, predominantly affecting the lymph nodes. RDD can also present in extranodal tissues and is occasionally found within the central nervous system. Case Description We report the case of a 52-year-old man presenting with a short episode of dizziness. Imaging identified a right frontal, extraaxial, dural-based lesion, suspicious for a meningioma. The patient underwent a craniotomy for tumor resection and, although not entirely typical, the pathology was consistent with RDD. No other evidence of RDD was identified. Conclusions RDD should be considered as a differential diagnosis of dural-based lesions, more commonly meningiomas.

Background Rosai-Dorfman disease (RDD) is a rare, benign histiocytosis disorder with only approximately 100 reported central nervous system cases in the literature. Even less common is skull base involvement of RDD, with about 41 reported cases. Radiographically, RDD can appear similar to a meningioma; the true diagnosis is only found with histologic analysis. Although “benign,” RDD can lead to significant neurologic morbidity from the disease or unnecessary surgical and nonsurgical treatment. While rare, intracranial RDD has been treated with chemotherapy or radiotherapy, with surgery playing a limited role to relieve mass effect. Surgical approaches to the skull base are invasive and pose risk, especially for a benign and self-limiting disease like RDD. Case Description Here we present the case of a 63-year-old woman with a presumed spheno-orbital meningioma for which the patient previously underwent radiation therapy. On presentation to our facility, the patient noted pressure headaches and blurry vision, and imaging demonstrated progression of her disease. For these reasons, surgical debulking and biopsy were undertaken. Conclusions This report demonstrates the role of EEA for tissue biopsy and decompression of the affected cranial nerves and orbit. This paradigm seems to provide a safe and effective way to manage patients with compressive symptoms, while also allowing for tissue sampling.

Poster: "ESTI 2019 / P-0109 / Lung involvement in Destombes-Rosai-Dorfman disease: clinical and radiological features, and response to the MEK inhibitor cobimetinib " by: "Q. moyon, S. Boussouar , F. cohen aubart, P. maksud, N. Aladjidi, G. Prevot, J. donadieu, Z. amoura, P. A. Grenier, J. haroche; Paris/FR"

Rosai-Dorfman disease (RDD) is a rare disease characterized by dilated lymph node sinuses with a large number of histiocytes. Extranodal disease is considered the uncommon subtype, which can be life-threatening when vital organs are involved. Here, we report a woman with a 6-month progressive dyspnea who visited our center. Computed tomographic pulmonary angiography revealed a filling defect in her bilateral pulmonary arteries. Bilateral pulmonary artery resection and reconstruction were performed, and postoperative pathologic analysis confirmed RDD. To our knowledge, this is the second case of extranodal RDD disease characterized by invasion of pulmonary artery treated with complete resection.

We report a case of a 49-year-old female diagnosed with extranodal multifocal Rosai-Dorfman disease (RDD) of the breast using mammography and ultrasound. RDD is a rare non-Langerhans cell benign proliferative disorder of histiocytes that usually involves the lymph nodes, but may involve extranodal sites. We review the clinical presentation as well as imaging features of this rare disease on multiple modalities and the importance of recognizing the diagnosis in order to direct treatment.

Background Rosai-Dorfman disease (RDD) is a rare disorder composed of a proliferation of histiocytes with varied clinical manifestations. Patients and Methods In this retrospective observational study, we obtained demographic, clinical, and outcome data from the medical records of 10 adult patients with RDD seen in the Department of Lymphoma and Myeloma at MD Anderson Cancer Center between 1995 and 2015. Results Patients had a median age of 56 years (range, 20-81 years) with equal gender distribution. Five patients were initially treated with systemic therapy. Five received cladribine as initial therapy or for relapse. Other therapeutic agents included clofarabine, lenalidomide, and steroids. The overall response rate was 80% in patients receiving cladribine, with a median progression-free survival of 29 months. Surgery, radiotherapy, and observation were also treatment options for localized disease. With a median follow-up of 65 months, none of the patients have died. Conclusion Currently, there is no standard of care for RDD. Treatment should be personalized for each patient depending on the clinical presentation, course of the disease, and prior treatment history. Purine analogues provide excellent responses; however, surgical resection and observation are also suitable for specific cases.

Background Cutaneous Rosai-Dorfman (CRD) disease is a rare entity that is characterized by histiocytic proliferation in the skin. The disease has been reported to exhibit different clinical profiles and occasionally confounding histologic features that may be challenging for a correct diagnosis. The purpose of this study was to assess the pathobiology and highlight the variance in clinical and histologic spectrum of the disease based on published literature. Methods A PUBMED search was performed to retrieve cases of cutaneous Rosai-Dorfman disease published in the literature. A PRISMA-guided review of the included articles was performed. Three interesting case reports from our institution are also described. Results A total of 263 patients, of which 220 with purely cutaneous disease were identified in 152 studies. The mean age at presentation was 45.2 years with a slight female preponderance, and East-Asian, Caucasian and African populations being largely affected. Majority of the patients presented with multiple lesions, predominantly on limbs and comprising of nodules, plaques and papules that were occasionally pigmented. The classis histologic findings included large foamy histiocytes, exhibiting emperipolesis and a specific immunophenotype (S100+, CD68+, CD1a-). Inconspicuous emperipolesis, fibrosis, increased vascularity, neutrophilic microabscesses and concurrent langerhans cell histiocytosis and lymphoma in few cases highlighted the importance of immunohistochemistry for a definitive diagnosis. The disease shows an indolent and benign course with excision and chemotherapy being most effective for extensive and refractory cases. Conclusions This review of largest cohort of CRD patients provides an updated insight into the clinicopathologic features with possible diagnostic pitfalls and effective therapeutic options that should be useful in diagnosis, management and future research opportunities.

Histiocytic disorders are an exceptionally rare group of diseases with diverse manifestations and a paucity of approved treatments, thereby leading to various challenges in their diagnosis and management. With the discovery of novel molecular targets and the incorporation of targeted agents in the management of various adult histiocytic disorders, their management has become increasingly complex. In an attempt to improve the understanding of the clinical features and management of common adult histiocytic disorders (Langerhans cell histiocytosis, Erdheim-Chester disease, Rosai-Dorfman disease, and hemophagocytic lymphohistiocytosis), we created this document based on existing literature and expert opinion.

Rosai-Dorfman disease (RDD) is a rare benign disorder of the histiocytes, affecting lymph nodes in its classic form. Extranodal RDD is considered the uncommon subtype and potentially impairs all tissues and intrathoracic organs. In our report, a 18F-fluorodeoxyglucose positron emission tomography scan of a mediastinal mass infiltrating the lungs and of widespread lesions suggested the presence of a metastatic disease. Open thoracic biopsy was required for immunochemistry and histopathology. Mediastinal mass with lesions disseminated throughout the body is an unusual presentation of extranodal RDD that calls for a challenging differential diagnosis to rule out the suspicion of metastatic malignancy.

Background Rosai-Dorfman disease (RDD), also called sinus histiocytosis with massive lymphadenopathy, is an idiopathic, non-neoplastic, lymphoproliferative disorder. Histologically, the disease is characterized by emperipolesis and S-100 immunoreactivity. RDD usually presents with massive painless cervical lymphadenopathy, with only 5% of cases affecting the central nervous system. Case Description We report the case of a 46-year-old woman with isolated RDD of the left cavernous sinus with left facial numbness and weakness of the left masticatory muscle. The patient was successfully treated by total resection without recurrence found at the 65-month follow-up examination. Conclusions Central nervous system RDD is a diagnostic challenge preoperatively depending on the radiologic findings. Immunohistochemical confirmation is indispensable for the definite diagnosis. Total resection remains the most effective treatment to date. Adjuvant treatment such as chemotherapy, radiotherapy, and steroids can be administrated in cases of incomplete resection, recurrence, or multiple foci.

Objective To report a case of orbital mass and proptosis, diagnosed as Rosai–Dorfman disease (RDD). Methods Clinical case report based on the review of clinical charts, radiological images, and histopathology. Results A 42-year-old male with orbital mass and proptosis of the right eye. A surgical biopsy was performed, and the diagnosis of RDD was established using microscopy and immunohistochemistry. Definitive management included open tumor cytoreduction, with good response. Discussion The RDD is a rare, benign, proliferative condition of unknown origin. It rarely affects the cranial cavities, with the orbital presentation being very unusual. Diagnostic confirmation is essential for the best surgical management.

Immunoglobulin(Ig)G4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect virtually any organ and usually presents as tumefactive lesions involving multiple sites. Characteristic histopathology of IgG4-RD consists of dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis, accompanied by tissue infiltration of IgG4-positive plasma cells with or without elevation of serum IgG4 level. Despite a general similarity in the morphologic manifestations of IgG4-RD, site-specific unique morphologic features have been described in some organs including the lung. Compared with other sites, pulmonary involvement by IgG4-RD has been recognized more recently, and lung biopsy interpretation for this condition is often challenging, as both a relative paucity of pathognomonic features and a plethora of overlapping findings with other fibroinflammatory processes of the lung. This review is focused on the morphologic spectrum of IgG4-related lung disease documented in the current literature and on the pertinent issues in the differential diagnoses with other conditions encountered in the lung.

Rosai-Dorfman disease (RDD) was first described by the French pathologist Paul Destombes in 1965. It frequently affects children or young adults and is characterized by the presence of large histiocytes with emperipolesis. More than 50 years after this first description, the pathogenesis of this rare disease is still poorly understood. The revised classification of histiocytoses published in 2016 identified various forms of RDD, from familial RDD to IgG4-associated RDD. Almost 90% of the patients with RDD have cervical lymph nodes involvement although all the organs may virtually be involved. Outcomes are typically favorable. Treatments may be necessary in case of compression or obstruction, and are not well codified. The main therapeutic strategies rely on surgery, radiotherapy, steroids, immunosuppressive drugs or interferon-alpha and cladribine.

Rosai–Dorfman disease (RDD) is a benign non–Langerhans cell histiocytosis with a predilection for the head and neck lymph nodes, although it may present extranodally in the skin, soft tissue, or the central nervous system. The diagnosis depends on histopathologic features, including the classic finding of emperipolesis, along with immunohistochemical characteristics: S100+ and CD68+ and CD1a– cells (Table I).1 RDD has several postulated etiologies, although none have been definitively proven. It has been reported to mimic various metastatic cancers,2 including 1 reported case of RDD simulating metastatic breast cancer.3 We present a patient with RDD of the skin and bone simulating presumed recurrent metastatic breast cancer. The correct diagnosis of RDD based on dermatologic and hematologic clues led to a significant change in her treatment. Table I Rosai–Dorfman disease: An overview

Introduction Spinal extranodal Rosai-Dorfman disease (RDD) is extremely rare. In this paper, we reported successful management of spinal extranodal RDD and reviewed medical literature. Case presentation A 19-year-old male presented with progressive bilateral leg weakness and back pain for two months before admission. He denied weight loss, fever, night sweats, and lymph node enlargement. On examination, his muscle strength of both legs was grade I with hyperreflexia. Magnetic resonance imaging of the spine (MRI) showed a thoracic extradural mass at a level of T6-T9, which was a heterogeneous hyperintense on T2W, STIR, and isointense on T1W and enhanced contrast vividly. We resected the tumor totally and decompressed the spinal cord. Pathology revealed a histiocytic tumor. Immunohistochemical staining was S100 (+), CD68 (+), CD45 (+), and CD1a (−). Postoperatively, his muscle strength improved gradually to grade IV after four months. Postoperative MRI of the spine showed no residual tumor. No further adjuvant therapy was indicated. Clinical discussion Spinal extranodal RDD has no specific symptoms and pathognomonic imaging features. CT and MRI of the spine are still the essential tools for diagnosing RDD, but biopsy is often mandatory for definitive diagnosis. There have not been consensus guidelines for treating RDD of the spine because of its rarity. Surgical resection remained the mainstay of treatment (78.8%), with or without adjuvant therapies. Conclusion Surgery is the treatment of choice for most cases, while steroid therapy, radiotherapy, and chemotherapy should be adjuvant treatment and tailored individually., Highlights • Spinal extranodal Rosai-Dorfman disease is extremely rare. • Surgery is the mainstay of treatment. • Steroid therapy, radiotherapy, and chemotherapy should be tailored individually.

This chapter describes the main features of two different diseases, Castleman Disease (CD) and Rosai-Dorfman Disease (RDD). Castleman disease (CD) is a clinical and histopathologically heterogeneous lymphoproliferative disorder that encompasses at least three distinct entities with some common overlapping morphological features: Hyaline Vascular CD (HVCD), Unicentric Plasma Cell CD and Multicentric CD. The most important feature of HVCD is the presence of abnormal germinal centers with hyaline-vascular transformation, sometimes showing multiple germinal centers within a single reactive lymphoid follicle, this outlining HVCD as a disorder of follicular dendritic cells. Unicentric and multicentric CD are, in contrast, lymphoproliferative lesions. Proinflammatory hypercytokinemia is an essential feature of multicentric CD, distinguished by a florid clinical presentation. Rosai-Dorfmann Disease is a histiocytic proliferative disorder diagnosed by the presence of tissue infiltration by S100-positive CD1a-negative histiocytes and plasma cell aggregates, often with Russell bodies. A typical, though not specific, characteristic of the disease is emperipolesis. Initially considered to be an inflammatory/reactive condition, molecular studies suggest that at least some cases of RDD could be considered as a low-grade histiocytic neoplastic process.

Rosai–Dorfman disease (RDD) is a non-malignant disease. Pathologically it is characterised by histiocytic proliferation. A typical clinical presentation is painless generalised lymphadenopathy. Extra nodal involvement can be seen in 20–40% of cases. Cardiac involvement is rare and suggests a poor prognosis. We report a case of RDD with extensive cardiac involvement, initially masquerading as multiple cardiac thrombi in the background of underlying antiphospholipid antibody syndrome.

Rosai Dorfman disease is rare benign disease of phagocytic histiocytosis usually present as painless massive lymphadenopathy. Extra nodal presentation of Rosai Dorfman disease is rare but also described. We hereby describe an unusual presentation of the extra nodal Rosai Dorfman disease with rapidly growing soft tissue lesion in the left thigh with associated osseous lesions in the right distal femur, left distal femur, left proximal tibia and presence of solitary pulmonary nodule. This case is unique as clinical presentation highly suspicious of soft tissue sarcoma but excision biopsy of thigh lesion showed Rosai Dorfman disease.Though RDD in extremities has been described in limited number of cases, to the best of our knowledge this is first case of lower extremity RDD associated with osseous lesions and pulmonary nodule but without lymphadenopathy.

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a nonmalignant disease of unknown etiology. It may present as bilateral, massive, painless cervical lymphadenopathy; however, one third of the cases have extranodal involvement. Extranodal sites include soft tissue, skin, upper respiratory and gastrointestinal tracts, and central nervous system, with head and neck being the most common. Here we report a rare case of intracardiac RDD in a 46-year-old, African-American female who presented with worsening shortness of breath and fatigue for 7 weeks. Comprehensive physical examination was within normal limits; the patient had no lymphadenopathy. Echocardiogram revealed a large mass in the left atrium extending into the mitral valve. The mass was resected under the pretext of clinical diagnosis of myxoma, and the patient underwent a radical extended interatrial septectomy with bovine pericardial patch reconstruction. The 8-cm resected mass consisted of areas of fibrosis alternating with sheets of histiocytes enmeshed in a mixed inflammatory infiltrate. Histiocytes contained intact lymphocytes, plasma cells, neutrophils, and erythrocytes suggesting emperipolesis. The diagnosis of RDD was confirmed by immunohistochemistry. This case highlights the extranodal manifestation of RDD in the heart which could be easily mistaken for a myxoma on clinical and radiologic evaluation. RDD must be considered in the differential diagnosis of any patient who presents with a cardiac mass.

Background Rosai-Dorfman disease (RDD) is a rare entity characterized by lymphadenopathy with dilated lymph node sinuses containing lymphocytes, plasma cells, and histiocytes exhibiting emperipolesis. IgG4-related disease (IgG4-RD) is relatively more common and is characterized by mass-forming lesions with dense lymphoplasmacytic infiltration, numerous IgG4 positive cells, fibrosis which is at least focally storiform, and obliterative phlebitis. Concomitant RDD and IgG4-RD is rare, and the significance of this association remains unclear. Case presentation A 64-year-old female presented with the gradual onset of a painful right gluteal mass. The mass was clinically suspected to represent an infected sebaceous cyst and was surgically resected. Histological examination revealed a mixed inflammatory reaction in adipose tissue. There were areas of histiocytic proliferation with phagocytosis of inflammatory cells (emperipolesis), predominantly lymphocytes and plasma cells. There was also prominent storiform sclerosis with dense collections of plasma cells. No obliterative thrombophlebitis was seen. Immunohistochemical staining highlighted S100-positive cells with emperipolesis, greater than 50 IgG4 positive cells per high power field, and an IgG4/IgG ratio of greater than 40%. The histologic and immunohistochemical findings were those of RDD with concomitant IgG4-RD. Conclusions There is limited literature on RDD with increased IgG4-positive plasma cells; and the exact relationship and clinical significance of this finding remains undetermined. We present here a case with combined RDD and IgG4-RD and review the relevant literature.

Rosai-Dorfman sinusal histiocytosis is a benign histiocytic disorder presenting as large lymphadenopathies, mostly in the cervical area. Visceral involvement is not unusual. The diagnosis is based on histologic findings, showing intrasinusoidal histiocytic infiltration with pathognomonic emperipolesis features. Histiocytes display a normal activated phenotype. It probably represents a heterogeneous group of diseases with some common histopathologic findings. An association with immunologic abnormalities or auto-immune diseases, mostly auto-immunes cytopenias is possible. This is a criterion of poor prognosis. The evolution can sometimes be spontaneously favorable. Nonetheless, there is a risk of compression, due to the huge volume of tumoral masses, especially in case of orbital or epidural involvement. Therapeutic abstention is usually justified. Treatments are discussed in threatening forms, evolving forms or in case of poor prognosis factors. Treatment, when indicated, is not codified. Surgery, corticosteroids, immunosuppressive agents and/or alpha interferon can be used.

Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder that is characterized by persistent massive lymphadenopathy mimicking malignant tumors. Extranodal RDD is uncommon, and more severe fibrosis and fewer histiocytes in lesions make the diagnosis of RDD more difficult than that of nodal RDD. We herein described a 31-year-old male patient with isolated soft tissue RDD of the head and neck. The patient was referred to our hospital with a right neck mass. Computed tomography (CT) scans showed a diffuse enhanced tumor with an unclear border in the right side of the neck. 18F-fluorodeoxyglucose positron emission tomography (PET)/CT revealed high uptake in the corresponding lesion. Second wide local excisional biopsy led to a diagnosis of RDD, and immunohistochemistry was useful for diagnosing RDD. A systemic treatment with steroids improved his symptoms, including the neck mass. Physicians need to consider the diagnostic difficulty associated with extranodal soft tissue RDD as well as its rarity.

Various hematolymphoid lesions involve the sinonasal tract, including aggressive B, T, and NK-cell neoplasms; myeloid sarcoma; low-grade lymphomas; indolent T-lymphoblastic proliferations; and Rosai-Dorfman disease. Differentiating aggressive lymphomas from non-hematopoietic neoplasms such as poorly differentiated squamous cell carcinoma, olfactory neuroblastoma, or sinonasal undifferentiated carcinoma may pose diagnostic challenges. In addition, the necrosis, vascular damage, and inflammatory infiltrates that are associated with some hematolymphoid disorders can result in misdiagnosis as infectious, autoimmune, or inflammatory conditions. Here, we review hematolymphoid disorders involving the sinonasal tract including their key clinical and histopathologic features.

Rosai Dorfman disease (RDD) is a rare, benign disease of unknown aetiology. It typically presents with massive, painless cervical lymphadenopathy but may have a varied presentation. We report a paediatric case of RDD with initial isolated involvement of nasal cavity and PNS with subsequent involvement of cervical lymph nodes. Endoscopic biopsy confirmed the pathological diagnosis and he was managed successfully with medical therapy. At 20 month follow up there was no evidence of recurrence. The ideal protocol for the treatment is still debated. A long term follow-up is warranted to detect relapses. This case report is illustrated aiming at developing insights into management and diagnosis of such rare clinical entity in a paediatric patient.

Purpose Cutaneous Rosai-Dorfman disease (C-RDD) is a rare, benign, non-Langerhans cell histiocytosis that can affect any skin area. The purpose of this study was to characterize C-RDD of the face through a literature review of published case reports of this lesion and present a new case of C-RDD. Materials and Methods An English-language search of 3 databases (PubMed, Scopus, and EBSCO Search) was conducted for cases of C-RDD of any skin area that had been published since 1969. Repeated citations of the same article in more than 1 database were deleted. Cases of C-RDD with solely facial involvement or involving the face and other skin parts were included and analyzed. Details of C-RDD cases (demographic features, regional distribution, clinical presentation, treatment, and follow-up course) were methodically reviewed and collected in Excel spreadsheets. Simple statistical analyses were conducted using Excel. Results The literature search yielded 578 published cases of C-RDD affecting any skin area; of these, 65 cases (11.2%) had facial skin involvement. The male-to-female ratio was 1:1.5, and the average age at presentation was 43.5 years (standard deviation, 12.4 yr). The racial distribution of facial C-RDD was, in descending order, 74.5% in Asians, 20% in Caucasians, and 5.5% in blacks. The most commonly affected facial skin regions were the cheeks and periorbital area, and most lesions were multiple in number and bilaterally distributed. The vast majority of facial C-RDD lesions presented as asymptomatic, nonulcerative, red, nodular plaques with durations ranging from 1 month to a few years. Many methods have been attempted for the treatment of facial C-RDD. However, the combined cure rate for all published treatment methods was only 28.6%. Surgical excision was the most effective treatment method, and corticosteroids were the least effective. Conclusion This article has tried to characterize facial C-RDD lesions for easier management by maxillofacial surgeons.

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare multisystemic disorder that was first reported by Rosai and Dorfman in 1969. It is a distinct histioproliferative disorder due to overproduction of histiocytes, which accumulate in lymph nodes. The cardiac involvement of this disease is extremely rare, and until now, only 18 cases have been reported. We report the case of a 53-year-old woman with right atrial mass mimicking myxoma, which the histopathologic evaluation revealed to be Rosai-Dorfman disease of the right atrium.

Surgical resection is the optimal treatment of intracranial Rosai-Dorfman disease (RDD). However, data on the long-term outcomes after total resection are extremely rare. We herein describe a 39-year-old man in whom de novo RDD appeared twice during the long-term follow-up after total resection in the lateral ventricle region. The first de novo lesion developed in the left sphenoid ridge 3 years after surgical resection. The sphenoid ridge lesion was completely resected. The second de novo lesion developed in the bilateral anterior clinoid processes 12 years after initial treatment. The patient underwent intensity-modulated radiotherapy and continued corticosteroid therapy. Long-term follow-up should be performed for intracranial RDD because repeated remission and recurrence are possible.

A 47-year-old woman with Rosai–Dorfman disease (RDD) presented with an electrical storm with sustained ventricular tachycardia. Echocardiography and computed tomography revealed an intracardiac mass involving the right atrium and left ventricle. Positron emission tomography demonstrated fluorodeoxyglucose avidity of the mass and myocardial infiltration; the electrical storm was attributed to this feature. Cardiac involvement in RDD is extraordinarily rare, and this appears to be the first report of an association of this disease with an electrical storm.

Primary cutaneous Rosai-Dorfman disease is a rare form of Rosai-Dorfman disease limited to the skin. The diagnosis of primary cutaneous disease is based on a combination of clinical presentation, histopathology, and the detection of S100+, CD68+, and CD1a- histiocytic immunophenotyping. However, the diagnosis of primary cutaneous disease is often difficult and significantly delayed due to the non-specific nature of its histologic and clinical features. In this review, we describe four cases in order to familiarize pathologists and dermatopathologists with the clinicopathologic correlation of primary cutaneous Rosai-Dorfman disease and to help facilitate early diagnosis. In addition, we discuss the proposed pathophysiology and molecular etiology of this tumor, and its relationship with IgG4 sclerosing disease.

This case report discusses the presentation of Rosai–Dorfman disease in a 4 year old female patient, who presented with bilateral level V lymphadenopathy of the cervical neck. The histopathology demonstrated numerous histiocytes, with characteristic emperipolesis. No granulomas or necrosis were identified. Micro-organisms were not identified on special stains, nor on culture. The patient was under observation and spontaneous remission occurred after six months.

Background Rosai–Dorfman disease (sinus histiocytosis with massive lymphadenopathy (SHML)) is a rare, histiocytic, lymphoproliferative disease of unknown etiology affecting young people with male predominance. It is characterized by massive, painless cervical lymphadenopathy with fever and malaise and varying extra-nodal involvement. Isolated spinal canal Rosai–Dorfman disease is extremely rare. We describe a case of isolated Rosai–Dorfman disease with both intradural extramedullary and epidural components. Clinical presentation 52 year man presented with 2 week history of progressive lower limb weakness and 2 days of urinary and fecal incontinence. He showed bilateral lower limbs weakness with normal muscle tone, exaggerated deep tendon reflexes and no sensory loss. CT and MRI showed large enhancing soft tissue mass lesion with both epidural and intradural extramedullary components opposite to T2–T5 vertebrae causing spinal cord compression associated with marrow changes of the body of T4 vertebra. Intervention A T3–T5 laminectomy and excision of the epidural lesion was performed. We opened the dura and found a large extramedullary well circumscribed mass engulfing the cord. Careful dissection and total resection of the intradural mass was done. The mass was histopathologically proved to be sinus histiocytosis conforming to Rosai–Dorfman disease. Postoperatively the patient showed improvement in the motor power and regained control over urine and stool. Conclusion This is a rare case of spinal Rosai–Dorfman Disease with epidural and intradural components causing cord compression. To our knowledge, this represents the first case of combined epidural and intradural extramedullary lesions in the literature.

Rosai-Dorfman disease (RDD) is a benign histiocytic proliferation that most commonly presents with painless bilateral lymphadenopathy and constitutional symptoms such as fever, fatigue, and night sweats.1 RDD is considered by many to be a reaction pattern with several different manifestations, especially as clonality has not been documented to support it representing a neoplasm per se. Classic histologic features include histiocytes that are S100 protein positive, are CD1a−, and demonstrate emperipolesis. Cutaneous lesions can occur in about 10% of patients, however, RDD limited only to cutaneous involvement is particularly rare.2, 3 Moreover, concomitant cutaneous RDD (CRDD) and bone RDD has rarely been reported in the English-language literature.4, 5 Here, we presented a case of CRDD on the scalp with underlying bony involvement.

Highlights • Rosai-Dorfman Disease is a benign disease of cervical adenopathy. • Extranodal disease can present rarely in the breast. • Histiocytes with emperipolesis is the hallmark pathological finding. • Surgical excision with post-op observation is the treatment for breast lesions. • Minimal amount of cases found in the male breast., Rosai-Dorfman Disease (RDD) is a rare disease that normally presents with bilateral cervical lymph node enlargement. Systemic symptoms of fever and weight loss may be present but patients are usually asymptomatic. This benign disease tends to regress on its own without treatment but there have been cases that required treatment with steroids or chemotherapeutic regimes. Extranodal disease in the breast is extremely rare, with only three cases identified in the male breast. The patient in this study presented with an asymptomatic right breast lump identified incidentally. After excisional biopsy, a diagnosis of RDD was confirmed on immunochemistry and histopathology.

Rosai-Dorfman disease (RDD) is a rare, nonneoplastic, idiopathic histiocytic proliferation disorder. Massive lymphadenopathy is one of the main clinical findings, although it can occur without nodal involvement, mimicking other conditions and making diagnosis difficult. Oral manifestations are extremely rare. A 65-year-old female patient was attended for evaluation in a stomatology service at a cancer hospital. Physical examination revealed a reddish macule with irregular contour and telangiectasia and a 4-cm, nodular, sessile, well-delimited lesion with normal coloration that softened to palpation. Both lesions were asymptomatic and localized in the hard palate. Furthermore, multiple papular lesions with bluish coloration in the face were observed. An incisional biopsy of both intraoral lesions was performed, and after the histopathologic and immunohistochemical analysis (S100), the diagnosis of RDD was obtained. Therapy was initially instituted with oral prednisone 60 mg/d resulting in significant regression of clinical signs. Currently, the patient is under monthly follow-up in stomatology and hematology services.