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Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare, benign, and self-limiting disease. Cutaneous Rosai-Dorfman disease (CRDD) is an exceedingly rare extranodal variant, which is strictly limited to the skin and is easily misdiagnosed. We present the case of a 47-year-old Chinese woman with primary CRDD and a review of relevant literature. The patient had red-yellow papules on her left cheek and chest for more than three months with occasional tenderness. Histopathology showed phagocytic histiocytes in the dermis with the typical presentation of emperipolesis. Immunohistochemistry (IHC) staining showed S100 (+), CD68 (+), and CD1a (-). Based on these findings and the negative systemic presentation, the patient was diagnosed with primary papulonodular-type CRDD, and no treatment regimen was initiated. After three months, the skin lesions partially disappeared, confirming the spontaneous regression of CRDD.

Rosai–Dorfman disease (RDD) is also known as Sinus Histiocytosis with Massive Lymphadenopathy. It is a rare, benign, self-limiting disease of phagocytic histiocytes presenting with massive painless cervical lymphadenopathy. RDD can present as a nodal disease and also extra-nodal involvement with episodes of exacerbation and remissions and relapses after treatment. Its etiology remains poorly understood and is highly variable in its clinical presentation and response to treatment. Its treatment is poorly defined but the prognosis is usually fair. Here we are reporting a rare, unusual clinical presentation of infraorbital soft tissue mass diagnosed as RDD with cyto-histopathological correlation. Only a few such cases have been reported in the literature.

Rosai-Dorfman Disease (RDD) is also known as Sinus Histiocytosis of Massive Lymphadenopathy(SHML). Clinically Rosai-Dorfman Disease is characterized by massive, painless, bilateral cervicallymph node enlargement, often mimics lymphoma. Microscopically, it shows dilatation of lymphaticsinuses occupied by numerous lymphocytes and proliferation of histiocytes with abundant paleeosinophilic cytoplasm containing engulfed lymphocytes or plasma cells- emperipolesis.

BACKGROUND Rosai–Dorfman disease (RDD) is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents. The common manifestation of RDD is massive and painless bilateral cervical lymphadenopathy with extranodal disease. While extranodal involvement in RDD is common, the spleen is an infrequent site of disease. CASE SUMMARY We report a 10-mo-old female infant with RDD presenting multiple splenic masses without cervical lymphadenopathy. She had fever, and blood tests showed leukocytosis, anemia, and elevated erythrocyte sedimentation rate and C-reactive protein. Ultrasound, computed tomography, and magnetic resonance images demonstrated multiple splenic masses. Despite antibiotic therapy, her symptoms were not relived. She underwent diagnostic splenectomy and was discharged with recovery. CONCLUSION In pediatric patients with refractory infectious symptoms or hematological abnormalities, clinicians should suspect RDD, even in patients without significant lymphadenopathy.

Background There were very few reports of Rosai–Dorfman disease in the thymus, which known as sinus histiocytosis with massive lymphadenopathy. It usually accompanied with other systemic symptoms such as fever, malaise, night sweats, or weight loss in the short term. Case presentation We herein report a rare case of RDD of thymic origin and a review of the literature concerning the clinical and pathological features of this disease, which is often misdiagnosed as thymoma. The patient was underwent thymectomy to extirpate the lesion completely by video-assisted thoracic surgery. Conclusions To the best of our knowledge, this is the fourth thymus occurring RDD case with proof via histology and IHC. Our findings suggest the difficulty of making a diagnosis before surgery and more cases will need to be reported in order to facilitate the preoperative diagnosis of such a rare tumor.

BACKGROUND Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a benign histiocytic disorder. Extranodal involvement is common, occurring in > 40% of patients, but bone involvement occurs in < 10% of cases. In addition, primary bone RDD is extremely rare. The majority of patients are adolescents and young adults, and the mean age at onset is 20-years-old. CASE SUMMARY We report an 8-year-old Chinese girl who presented to our hospital with an insidious onset of swelling and pain in the middle shaft of her right tibia for 4 mo. We performed total surgical resection of the right tibia lesion and allograft transplantation. A good prognosis was confirmed at the 6 mo follow-up. Pain and swelling symptoms were totally relieved, range of motion of her right knee and ankle returned to normal, and there was no clinical evidence of lesion recurrence at last follow up. Our case is the second reported case of osseous RDD without lymphadenopathy in the shaft of the tibia of a child. CONCLUSION Extranodal RDD is a rare disease and can be misdiagnosed easily. Lesion resection and allograft transplantation are an option to treat extranodal RDD in children with good short term result. Pediatric orthopedist should be aware of this rare disease, especially extranodal involvement.

Histiocytoses are a rare group of proliferative disorders with very similar clinical and histological pictures. We present a case report of two variants seen in an eight-month-old female and five-month-old male in a tertiary hospital in southern Nigeria. They both presented with painless neck swellings and fever, leucocytosis, neutrophilia and lymphopenia. Initial histologic examinations of the cervical lymph nodes biopsy posed a diagnostic conundrum. However, Immuno-histochemical analysis done on both sample showed CD1a, positive S100 in keeping with Langerhans cell histiocytosis in the former. While, that of the latter showed strongly positive CD68, positive S-100 in 30% cells in keeping with Sinus histiocytosis with massive lymphadenopathy (SLMH) in the latter. Clinicians should have a high index of suspicion for histiocytosis in children presenting with generalised lymphadenopathy. Also, apart from the routine histology, immunohistochemistry analysis is recommended for all cases

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare disease typically characterized by a histiocytic proliferation within lymph nodes, which is due to an unknown etiology. Extranodal involvement can occur, and it more rarely can involve the skin. RDD generally presents with an indolent nature and follows a benign disease course, although more aggressive cases have been reported. The condition predominately affects children and young adults. It is classically characterized by massive, bilateral painless lymphadenopathy and accumulation of CD68-positive, S100-positive, CD1a-negative histiocytes, with the presence of emperipolesis as a hallmark. Herein, we present an aggressive case in a 76-year-old male with past medical history significant for prostate cancer, who presented with a 7-month history of lymphadenopathy and new onset of multiple large abdominal wall, cutaneous, lymph node, liver, and lung masses, all of which were histopathologically atypical, but showed features consistent with RDD, including emperipolesis and strong S100 positivity. Molecular studies showed a KRAS 117N mutation, which has been recently reported in RDD. While most cases present as a benign tumor, this case demonstrated aggressive features clinically, showed partial response to MEK inhibitor immunotherapy in the setting of a KRAS mutation, and demonstrated atypical cytologic features on histopathology.

Rosai-Dorfman disease (RDD, also known as sinus histiocytosis with massive lymphadenopathy) is a rare and benign non-Langerhans cell histiocytosis. Skin biopsy usually shows nodular or diffuse dermatitis. Rosai-Dorfman cells (RDD cells) and emperipolesis are the key to diagnosis. RDD cells express S-100 antigen, CD68, CD163, α1-antitrypsin, α1-antichymotrypsin, and ham-56, whereas Langerhans cell markers such as CD1a and langerin are negative. We presented a case of a 55-year-old man with varying sizes of many dark red nodules and lumps over the face, trunk, and limbs for approximately 1 year but without systemic involvement. The results of the laboratory evaluations were notable for an increased level of serum IL-6 and serum IgG4. Histopathological examination showed a diffused dense nodular infiltration of "nude" epithelioid histiocytes with infiltration of minimal lymphocytes and plasm cells around the epithelioid nodules. Immunohistochemistry identified nodular histiocytes being stained strongly positive for S-100 and CD68 but negative for CD1a. Plasma cells showed focally positive for IgG, IgG4, and CD38 and with a ratio of IgG4/IgG >40%. Considering these findings, we believe that our case meets the diagnostic description of "cutaneous Rosai-Dorfman disease" and is, therefore, a rare case with clinical features of multiple tumor-like nodules, sarcoidosis-like histological features, and immunohistochemistry of IgG4-positive plasma cells.

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.

Rosai Dorfman disease is a rare cause of sinus histiocytosis with massive lymphadenopathy. In developing countries, it often mimics some infectious diseases and malignancies and is often mismanaged. A high index of suspicion is necessary for its diagnosis. It is seldom life threatening therefore observation is currently the best advocated approach for its management., Rosai Dorfman disease is a rare cause of sinus histiocytosis with massive lymphadenopathy. In developing countries, it mimics tuberculosis and malignancies and it is often mismanaged. A high index of suspicion is necessary for its diagnosis. It resolves spontaneously therefore observation is currently the advocated approach for its management.

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare and benign disease that usually presents as massive and painless cervical lymphadenopathy. We are reporting this rare disease with systemic manifestations and causing internal jugular vein thrombosis and middle lobe collapse-consolidation.

Rosai-Dorfman disease (RDD) is a rare and self-limiting disease process that presents most commonly in young patients as massive, painless, cervical lymphadenopathy. Extranodal involvement may also occur. Histopathologic evaluation is the main diagnostic modality. We report an unusual presentation of RDD with cervical lymphadenopathy and an incidentally discovered sinonasal mass, clinically worrisome for malignancy. We emphasize that a high index of clinical suspicion is critical for accurate diagnosis of RDD. Clinicians and pathologists should consider RDD in a differential diagnosis of cervical lymphadenopathy, especially in young patients.

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.

Rosai-Dorfman-Destombes (RDD) disease also known as sinus histiocytosis with massive lymphadenopathy is a rare disease characterized by disorder of histiocytes within lymph nodes or other affected tissues. Extranodal forms of disease are even more uncommon. Only small percent of patients present with intracranial extranodal form of disease. Lesion biopsy and characteristic histopathological findings are crucial for definitive diagnosis. Histological findings typically show emperipolesis of affected histiocytes or hyperplastic lymph node sinuses containing enlarged histiocytes with phagocyted intact lymphocytes with specific immunoassay findings of the characteristic histiocytic non-Langerhans cells. Therapy includes various modalities such as surgery, steroids, radiotherapy, and/or chemotherapy. In case of extranodal intracranial form of disease, resection (single or combined with other treatment) was reported as effective. There are only few cases of extranodal intracranial disease in pediatric population described in the world so far. We aimed to provide brief comprehensive review of published cases referring on pediatric extranodal intracranial RDD.

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.

Rosai-Dorfman disease is a rare proliferative histiocytic disorder of lymph nodes that is descriptively known as sinus histiocytosis with massive lymphadenopathy. Extranodal involvement of the parenchyma of the breast is uncommonly reported, with fewer than 50 cases of mammary extranodal disease detailed in the English-language literature. We characterize a retrospective series of adult female patients from a single institution with Rosai-Dorfman disease of the breast and axillary lymph nodes. Because Rosai-Dorfman disease of the breast and axillary lymph nodes may clinically, radiographically, and histologically mimic breast carcinoma and other conditions, we present an illustrated review of the disease and its relevant differential diagnoses in hopes of raising awareness and allowing for accurate management of affected patients.

Rosai- Dorfman disease (RDD) or Sinus Histiocytosis with massive lymphadenopathy (SHML) is a rare benign disease with infiltration of lymph nodes or extranodal sites by non malignant histiocytes. It generally presents with fever, painless cervical lymphadenopathy and eleveated inflammaotory markers. Sometimes, it may be associated with night sweats and weight loss mimicking lymphoma. Forty per cent, present with extra nodal manifestations and 10 % have systemic involvement. Though the disease has a predilection for lymph nodes in head and neck region, it can affect any organ. The common extra nodal sites are skin and soft tissue, central nervous system, upper respiratory tract and gastrointestinal tract. Immune dysfunction and viral infections like HPV 6, EBV, parvo virus B19 are reported to be associated with the disease. RDD typically occurs in childhood and early adulthood. Histologially, characteristic finding of emperipolesis is seen. Diagnois is confirmed by Immunohistochemistry with S100 and CD68 positivity. Most of them do not require treatment as it is usually self-limiting. Steroids, surgery, chemotherapy and radiotherapy are considered in refractory disease with systemic involvement or vital organ involvement. We report a case of 4 year old boy who presented with massive bilateral cervical and mediastinal lymphadenopapthy and had complaints of breathing difficulty while lying down since last 3 months. Biopsy was confirmative of Rosai-Dorfman disease and he had a near complete response after 3 months of steroids and is symptom free for the past 1 year.

Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman Disease [RDD]), is a rare, benign but clinically heterogeneous histiocytic disorder. Our aims were to analyze the clinical characteristics of the disease and explore the outcomes of patients with RDD followed at our institution. Between January 2000 and February 2019, there were 15 patients with a pathologically confirmed diagnosis of RDD. Median age at diagnosis was 48 years old (range 26-78). The majority (87%

Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) being a rare benign proliferative self-limiting disease of the cells of macrophage-histiocyte family is of unknown etiology and presents with massive lymphadenopathy. We are hereby reporting a case of RDD presenting with massive bilateral cervical and submandibular lymphadenopathy, diagnosed by histocytopathology.

Rosai-Dorfman disease, also defined as sinus histiocytosis with massive lymphadenopathy (SHML), is represented by a distorted lymph node architecture with marked dilation of lymphatic sinuses occupied by numerous lymphocytes, as well as histiocytes with vesicular nuclei and abundant clear cytoplasm with phagocytized lymphocytes or plasma cells, also known as 'emperipolesis.' This disease of unknown cause develops with a favourable prognosis only if and when it is diagnosed and treated early. A dismal prognosis is exacerbated by a late diagnosis and widespread lymph node involvement. A 29-year-old Indian woman with a 4-month history of a painful unilateral cervical tumour and low-grade fever was diagnosed with Rosai-Dorfman disease. Fine needle aspiration (FNA) biopsy of the cervical lymph node was used to make the final diagnosis. Finally, due to the specific cytological features of SHML, FNA biopsy may be sufficient to make the diagnosis in the majority of patients, eliminating potentially unnecessary invasive procedures. The disease can be diagnosed on the histopathologically and immunohistochemically.

Background Rosai-Dorfman-Destombes (RDD) disease, is a rare proliferative and inflammatory disorder of non-Langerhans cell histiocytes. Case presentation We report a 35-year-old woman, who originally presented with recurrent episodes of lower extremity joint/bone pain and chronic nasal stuffiness and congestion. Her worsening nasal congestion was due to an obstructing nasal cavity lesion which was subsequently biopsied. Pathology was consistent with RDD. 18F-FDG PET images demonstrated intense uptake in the paranasal sinuses and a large pelvic lymph node. Focal osseous lesions with intense 18F-FDG uptake were also observed in the lower extremity, corresponding to areas of peri-articular pain. Rheumatologic work-up was consistent with palindromic rheumatism. She was diagnosed with immune-related disseminated RDD, presenting as palindromic rheumatism. Conclusions This is the first case of RDD presenting as palindromic rheumatism. RDD should be considered as a possible but rare diagnosis in young patients with sinus-related symptoms and lymphadenopathy. The disease can on rare occasions be disseminated and can also present as immune-related RDD, such as in this patient.

Background Rosai–Dorfman–Destombes (RDD) is also known as sinus histiocytosis with massive lymphadenopathy (SHML). It is a benign proliferative disorder of histiocytes, affecting lymph nodes, rarely with extra-nodal involvement. Rapid on-site evaluation (ROSE) with fine-needle aspiration cytology (FNAC) can be utilized as a minimally invasive investigation to avoid unnecessary surgery of this self-limiting disease. Case presentation A 65-year-old female presented with complaints of bilateral cervical lymphadenopathy since 1 year. Rapid on-site stain with FNAC from bilateral cervical lymph nodes revealed features of Rosai–Dorfman–Destombes (RDD) disease. Conclusion FNAC with rapid on-site evaluation can provide a simple and cost-effective method for looking at the unique cytological features of the disease and act as a first-line investigation.

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Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Syndrome, a non-malignant variant of histiocytosis (1) is a chronic reactive disorder. It presents as a benign, chronic, massive enlargement of cervical lymph nodes (LN) accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate (ESR) and hyperglobulinamia (2). There is no malignant proliferation of the class II histiocytes (i.e. normal macrophages and monocytes). The etiology of this disease is currently unclear. Sinus histiocytosis with massive lymphadenopathy may be of two types – either familial or infection induced (2). With respect to the latter, increased antibody titers to Epstein Barr and measles viruses have been observed; however, etiological evidence is lacking (1). Immune disturbances are likely to be a feature in some patients. Subtle undefined immunological defects are also considered as a causal factor. Human Herpes Virus-6 DNA has been detected in biopsy specimens and is considered as a contributing factor (6). Sinuous histiocytosis with massive lymphadenopathy (SHML) most frequently occurs in first and second decades of life, with a peak incidence at twenty years of age (1). There is a higher prevalence in males, and among Afro-Caribbeans as compared to Caucasians and Asians. Also, a familial association has been observed in some cases (3).

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is a rare idiopathic, non-neoplastic proliferative disorder of histiocytes with an unknown etiology that most often presents as bilateral painless cervical lymphadenopathy. In this report, we highlight an unusual case of RDD pathology in a 7-month-old immunocompetent male that presented with a two-week history of cervical lymphadenopathy and fevers. Our case is an indication that while RDD in general is rare, the disease should be considered in the differential diagnosis of a cervical lymphadenopathy associated with rare pathogens such as Serratia marcescens.

Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare non-Langerhans cell histiocytosis. We report a case of a 69-year-old male with concurrent appendiceal and rectal masses who underwent CT-guided percutaneous biopsy. Histopathology confirmed a diagnosis of RDD with IgG4-positive plasma cells. It is believed to be a subset of RDD that shares similar features with IgG4-related disease suggesting some overlap of the two diseases. Because gastrointestinal RDD accounts for less than 1% of extranodal disease, it is important to recognize this entity in order to guide management. We review the presentation, diagnosis, and treatment of gastrointestinal RDD and discuss the possible overlap with IgG4-related disease.

INTRODUCTION: Rosai–Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a nonneoplastic lymphoproliferative disorder which commonly presents with painless massive cervical lymphadenopathy along with fever and weight loss. Isolated extra-nodal disease involvement, especially isolated central nervous system disease without lymph node involvement is rare. CASE PRESENTATION: A 35-year-old man presented with upper back pain and paraparesis of 2 months duration. Contrast-MRI revealed an enhancing epidural mass lesion from C6-T7 suggestive of a primary epidural spinal-cord tumor. The lesion was resected and posterolateral fusion C6-T8 was performed. Histopathological examination revealed an isolated extra-nodal case of RDD. He was started on chemotherapy after the healing of surgical site in 2 weeks post operatively. Complete resolution of neurological deficits with significant improvement in gait was seen at the last follow-up 2 years after surgery. Repeat MRI revealed complete resolution of the disease. DISCUSSION: RDD with spinal involvement is rare and it is often a challenge to arrive at the correct diagnosis. Histopathological characteristics and immunohistochemical findings are necessary for diagnosis. Resection of the lesion takes compression off the cord and therefore, is the primary line of management of this disease. Our case presentation serves as a reference when diagnosing and managing a patient of RDD.

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic disorder, which is rare and typically occurs in lymph nodes. However, one third of the cases manifest as extranodal disease. The cardiac involvement of this disease is extremely rare. We reported here the FDG PET/CT findings of a pathologically confirmed Rosai-Dorfman disease occurring in the right atrium in a 60-year-old man.

Purpose To review and summarize the clinical features, diagnosis, treatment strategies, and prognosis of spinal Rosai-Dorfman disease (RDD). Methods RDD is also termed as sinus histiocytosis with massive lymphadenopathy. We searched the databases of PubMed, Elsevier ScienceDirect, SpringerLink, and OVID. The keywords were Rosai-Dorfman disease and spine/central nervous system. Research articles and case reports with accessibility to full texts regarding spinal RDD were eligible for the inclusion. A total of 62 articles were included, and they contained 69 cases. We extracted the information of interest and analyzed them using SPSS statistics package. Results The average age was 33.1 ± 18.3 years. The ratio of males to females was 1.9/1. Overall, 63 cases presented with spine-related symptoms. A total of 27 cases (39.1%) had multi-organ lesions, and 12 cases had records of massive lymphadenopathy. Among 47 cases who first manifested spine-related symptoms, 93.6% were preoperatively misdiagnosed. The disease had a predilection for cervical spine (38.8%) and thoracic spine (40.3%). 62.9% of lesions were dura-based. Surgery remained the mainstream treatment option (78.8%), with or without adjuvant therapies. Total lesion resection was achieved in 34.8% of cases. The rate of lesion recurrence/progression was 19.5%, which was marginally lower for total resection than for non-total resection. Conclusion Spinal RDD has no pathognomonic clinical and imaging features. Most cases first present with spine-relevant symptoms. Massive lymphadenopathy is not common, but a tendency for multi-organ involvement should be considered. Spinal RDD has a high recurrence rate; thus, total resection is the treatment of choice. Adjuvant therapies are indicated for multi-organ lesions and residual lesions. A wait and watch strategy is recommended for asymptomatic patients. Herein, a workflow of diagnosis and treatment of the spinal RDD is established.

Rosai-Dorfman disease (RDD), also called sinus histiocytosis with massive lymphadenopathy, is a rare, benign and self-limited histiocytosis. We present a case of cutaneous Rosai-Dorfman disease (CRDD) on the right cheek that responded well to combination of subtotal resection and ALA-PDT. We believe that the combination of subtotal resection and ALA-PDT treatment for large cutaneous facial Rosai-Dorfman disease patients is a more effective, highly satisfying, and quick therapy worth promoting.

Rosai Dorfman disease is a rare cause of sinus histiocytosis with massive lymphadenopathy, in developing countries it mimics tuberculosis and malignancies like lymphoma and its often mismanaged. A high index of suspicion is necessary for its diagnosis. It resolves spontaneously therefore observation is currently the advocated approach for its management.

Rosai-Dorfman disease (RDD), sometimes known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder that most commonly presents as painless, massive cervical lymphadenopathy in young adults. Extranodal disease can occur in up to 40% of patients but primary involvement of bone is rare. We present two cases of primary RDD of bone: one case of multifocal osseous RDD presenting as a painful lesion in the elbow, and one case of a solitary osseous lesion presenting as a painful lesion in the wrist.

Patient: Male, 40 Final Diagnosis: Rosai-Dorfman disease Symptoms: Dry cough • dry mouth • dry skin • general fatigue • itchy eyes • joint pain • neck swelling • shortness of breath Medication: — Clinical Procedure: Complete blood cell, C reactive protein, creatinine, antibody and rheumatological studies • histopathological and radiological study Specialty: General and Intrenal Medicine Objective: Unusual clinical course Background: Rosai-Dorfman disease is a rare disorder that was previously described as sinus histiocytosis with massive lymphadenopathy. The disease is derived from overproduction of monocytes, which play an important role in immunity. The overproduction of macrophages will lead to accumulation of the cells in the affected tissue. Case Report: A 40-year-old Saudi male presented with shortness of breath with joint pain starting 3 months prior. His main complaints were a dry cough, bilateral neck swelling, dry mouth, dry skin, itchy eyes, and general fatigability. Physical examination showed that the patient had prominent bilateral parotid swelling that measured roughly 5 by 3 cm, which was firm and tender, with a smooth surface and no local signs of inflammation. Joint examination revealed non-tender and non-swollen joints, with mild limitations of movement. Eye examination revealed dry eyes after having a positive Schirmer test. For diagnosis, the patient underwent complete blood count, autoantibody, histopathology, immunohistochemistry, and radiological assessment. The histopathological study confirmed Rosai-Dorfman disease. Rosai-Dorfman disease can involve various presentations, as in this patient, who exhibited a highly unusual presentation in association with autoimmune disease. Conclusions: Rosai-Dorfman disease must be considered as differential diagnosis in patients who present with bilateral lymphadenopathy with multisystem complaints, as the disease can present with various characteristics.

Rosai-Dorfman disease (RDD) is a rare medical condition with bilateral painless lymphadenopathy. We present the case of a young man diagnosed with a very unique presentation of Rosai-Dorfman disease. A 40-year-old African-American man presented with a firm, non-tender, progressive chest and neck mass appeared three months ago. Imaging of the neck demonstrated an 8.6-cm anterior neck subcutaneous soft tissue mass extending into the anterior mediastinum through the sternum with erosive changes in the sternum and the lesion is abutting the right common carotid artery and innominate vein and surrounds the medial aspect of the clavicles bilaterally. Ultrasound (US)-guided biopsy showed marked polytypic-appearing plasma cell proliferation associated with relatively prominent histiocytes with hemophagocytosis/emperipolesis and focal neutrophils. There were S100+ histiocytes; however, findings were not typical for RDD. As that biopsy was not diagnostic, incisional biopsy with adequate sampling was performed. Surgical pathology demonstrated a very abnormal infiltrate with prominent histiocytes including areas with the features of extranodal RDD. BRAF V600E immunohistochemistry (IHC) was negative. Modified radical neck dissection, proximal sternal resection and superior mediastinal nodal dissection surgery was recommended. However, the patient refused the procedure. Typical manifestations are lymphadenopathy with fever that our patient did not experience. Bone involvement happens in 5-10% of cases. There is not enough data about blood vessel invasion which make our case unique. Treatment plan is still controversial. Clinical monitoring is recommended if the symptoms are tolerable as regression has been reported in many cases (20-50%). Surgery is reserved for patients with vital organ involvement or extra-nodal disease.

Rosai-Dorfman Disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare entity characterized by proliferating S100-positive histiocytes. It is most commonly found in lymph nodes with extranodal involvement usually occurring in the head and neck. Pancreatic involvement is extremely rare. The pathology department archives were searched for fine needle aspirations and pancreatic resections showing evidence of RDD. Clinicopathologic features, cytologic smears, cell blocks, immunocytochemical stains and surgical resections were reviewed. Three cases were identified. They were all females, aged 65, 69 and 75, with involvement of the pancreatic tail or head by solid masses of median size 2.3 cm (range 2.1-4.5 cm). Cytologic findings on smears included multiple histiocyte clusters resembling loosely cohesive epithelioid granulomas, singly dispersed histiocytes with moderate to marked nuclear atypia and characteristic emperipolesis. These atypical histiocytes stained positively for CD68, CD163 and S100. Smear background contained variable mixed inflammatory cells, necrotic debris and stromal fragments. The RDD diagnosis was further confirmed on pancreatic resection in two patients and core biopsy in one. The latter patient required three separate procedures before a definitive diagnosis was made. RDD of pancreas is a rare benign inflammatory condition that is diagnostically challenging on cytology. This can cause delays in cytologic diagnosis and/or misdiagnosis. Identification of characteristic cytologic features, primarily histiocytes with emperipolesis, and matching immunocytochemical profile can ensure accurate diagnosis and distinction from mimics.

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.

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a disease of histiocytic proliferation with no known pathogenesis. This disease is defined by histological and cytological characteristics, including emperipolesis and positive S100 and CD68 markers. Although the disease typically presents clinically with massive bilateral lymphadenopathy due to sinus expansion from excessive histiocytosis, only unilateral lymphadenopathy was observed in this patient. The case involves a 40-year-old Caucasian male from the Netherlands presenting with unilateral lymphadenopathy suspicious for malignancy. Subsequent histological and laboratory testing led to the rare diagnosis of RDD. The lymphadenopathy resolved spontaneously over the course of several weeks following the initial presentation. Rosai-Dorfman disease reportedly has a benign prognosis with approximately 20% of patients experiencing spontaneous disease resolution (as was the case for this patient) with 70% experiencing chronic symptoms that may last years but not require intervention. We therefore advocate observation as a mainstay of treatment for most cases of this rare disease with intervention only being pursued in symptomatic cases. A review of recent literature regarding pathogenesis, epidemiology, diagnostic factors, prognosis, and treatment is provided and discussed.

Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare, benign type of non-Langerhans cell histiocytosis. The radiological findings are often nonspecific, potentially mimicking malignancies. The diagnosis is ultimately made based on pathology, in which the lymph nodes are characterized by a dilated subcapsular sinus filled with histiocytes that can exhibit emperipolesis. Immunohistochemically, the histiocytes are variably CD68 positive and reliably negative for CD1a. The features of head and neck sinus histiocytosis with massive lymphadenopathy are exemplified in this radiology-pathology correlation sine qua non article.

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy is a rare non-Langerhans' cell histiocytic disease resulting from the proliferation and accumulation of sinus histiocytes within lymph nodes. Extranodal involvement frequently occurs, which increases the morbidity and mortality of the disease. There is no clear consensus with regard to the most effective diagnostic and treatment modalities. This report will focus on the diagnostic imaging, treatment, and outcomes for 3 cases of Rosai-Dorfman disease. Imaging has typically utilized computed tomography (CT)/magnetic resonance imaging to detect extranodal involvement. However, the addition of fluorodeoxyglucose positron emission tomography/CT scans has shown value in identifying lesions unidentified or ambiguous on other modalities. Fluorodeoxyglucose positron emission tomography/CT detected disease involvement in 2 instances either not reported or not felt to be significant on correlative CT imaging. Areas of involvement included the stomach/liver in case 1, and the paranasal sinus in case 3. In addition, previously utilized chemotherapy regimens have not consistently displayed regression of the disease, which lends credence to the pursuit of more successful treatment. Notably, Clofarabine has shown promise in its use against histiocytic disorders. Our study concluded that Clofarabine demonstrates the ability to decrease lesion size and should be considered as an effective chemotherapeutic treatment method.

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is an uncommon histiocytic disease of the lymph nodes. Extranodal presentation, especially in breast parenchymal tissue, is rare. A 54-year-old woman presented with a painful and palpable lump in the right breast. Ultrasonography revealed an irregular, indistinct, hypoechoic mass with a hyperechoic halo. Pathological analysis revealed proliferation of large histiocytes and stromal fibrosis with emperipolesis and positive immunoreactivity for S-100 and CD68. The clinical and radiologic manifestations of Rosai-Dorfman disease may vary, and differentiation from other inflammatory diseases and malignancies is challenging; thus, accurate pathological diagnosis plays an important role in appropriate management.

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare and benign proliferative disease that affects histiocytes. Its etiology remains unclear. Rosai-Dorfman disease primarily affects lymph nodes with many extranodal manifestations present, including cutaneous, pulmonary, ophthalmic, and gastrointestinal. Diagnosis is mainly histologic with the presence of Rosai-Dorfman cells, which demonstrate emperipolesis. We report a case of a 30-year-old man who presented with a facial cutaneous mass and was diagnosed with RDD; he underwent surgical excision. The patient experienced recurrent lesions on the surgical scar and parotid gland as well as the lower back. Treatment consisted of systemic steroids and surgical excision.

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare, benign clinical entity of unknown cause. RDD is characterised by the overproduction and accumulation of histiocytes, primarily in the lymph nodes, although it may affect every organ and system. It predominantly affects children and young adults. Typically, patients are in good general condition, with massive cervical lymphadenopathy and fever. In about 40% of cases extranodal localisation of RDD is diagnosed. In laboratory tests the most common abnormalities are increased erythrocyte sedimentation rate (ESR), leukocytosis with neutrophilia, normocytic anaemia, and hypergammaglobulinaemia. Histopathological examination remains the mainstay of diagnosis - lymph nodes have massive sinusoidal dilation, containing histiocytes positive for S-100 and CD68, and negative for CD1a. Most patients do not require treatment as spontaneous remissions are observed. We present a brief review of the literature and the case of a six-year-old boy with cervical lymphadenopathy diagnosed with RDD. So far, the patient has not required systemic treatment and has been kept under observation.

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment.

Rosai Dorfman disease known as sinus histiocytosis with massive lymphadenopathy is an uncommon benign systemic histo-proliferative disease which affects the cervical lymph nodes commonly. The characteristic histological feature is lymphophagocytosis (emperipolesis) which is the hallmark feature. Here, we report a case of 35‑year‑old Indian female presenting as a mass on medial aspect of thigh appearing as soft tissue mass, which on cytological examination was misdiagnosed as malignant mesenchymal neoplasm but histopathological examination and immunohistochemistry confirmed the diagnosis. According to the literature reviewed no case of Rosai Dorfman disease with only ingunal lymphadenopathy was reported till date which was seen in present case. RDD is a rare inflammatory non neoplastic process that should be considered in the differential diagnosis of lympadenopathy and soft tissue tumor such as soft tissue sarcoma. The diagnosis of RDD is made on the basis of clinical suspicion and is confirmed by histopathology and immunohistochemistry.DOI:10.21276/APALM.1464

Rosai–Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is a self-limiting, benign histiocytic disorder. We report a case of RDD mimicking lymphoma clinically and cytologically in a 3-year-old male child. The present case highlights the importance of cytology in the diagnosis of RDD along with cytological features of other differential diagnoses.