Diagnosis Of Rosai-Dorfman Disease Research Articles

Rosai-Dorfman Disease Displays a Unique Monocyte-Macrophage Phenotype Characterized by Expression of OCT2.

Rosai-Dorfman disease (RDD) is a rare histiocytosis with heterogenous clinical features. In this study, we characterized the histologic and phenotypic features in 33 RDD patients to better define the pathologic diagnosis. Cases included 24 patients with extracutaneous disease ("R" group), and 9 patients with lesions limited to the skin or subcutaneous tissue ("C" group). We identified OCT2 as a novel marker for the monocyte-macrophage phenotype of RDD, expressed in 97% of RDD cases. In contrast, OCT2 expression was seen in 0% of Erdheim-Chester disease cases and 6.7% of Langerhans cell histiocytosis cases. Other markers useful in the diagnosis of RDD included S100 (100%), CD163 (88%), and cyclin D1 (97%). In a subset of cases, RDD showed moderate to strong expression of factor 13a (30%), p16 (64%), and phosphorylated extracellular signal-regulated kinase (45%); RDD was uniformly negative for ZBTB46, CD1a, and langerin. Within the "R group" of RDD, increased expression of factor 13a or phosphorylated extracellular signal-regulated kinase showed a statistically significant association with multifocal disease (P<0.05). Identification of the unique monocyte-macrophage phenotype of RDD with OCT2 expression furthers our understanding of this complex disease and allows for more uniform classification.

Extra-nodal Rosai-Dorfman Disease Confined to Breast

Abstract Introduction/Objective First described by Rosai and Dorfman in 1969, Rosai-Dorfman Disease (RDD) is an uncommon, idiopathic, reactive lymph node process with an unknown etiology. It may involve extra-nodal organs including the skin, bone, soft tissue, and eyes. However, breast involvement is rare and RDD confined to breast without nodal involvement is extremely uncommon. Methods Here we present a case of RDD confined to breast. The patient was a 51-year-old African American woman who was found to have an irregular, solid left breast mass on routine mammogram, which had speculated irregular margins and measured of 37.0 x 32.0 x 32.0 mm. She did not have any symptoms, nor any palpable lymph nodes. The clinician stated that the lesion was highly suspicious for a breast malignancy and requested a fine needle aspiration (FNA) of the mass, followed by total excision. Results An FNA was interpreted as atypical histiocytic cells in a mixed lymphoid background. Histopathologic examination revealed an ill-defined mass with sheets of histiocytes, plasma cells, and a mixed lymphocytic population, with occasional germinal centers. Some of the histiocytes were spindle shaped and associated with storiform collagen deposition. The histiocytes had single and multiple nuclei and exhibited occasional emperipolesis. Immunohistochemical staining with S100 diffusely highlighted the histiocytes, whereas CD1a was negative. CD3 and CD20 immunostains were positive for mixed-type lymphocytic infiltration. Cytokeratin staining was performed and reassuringly stained only the benign ductal cells. A diagnosis of RDD of the breast was made, based on these histopathologic findings. Conclusion We report an example of an extra-nodal RDD involving the mammary gland that was initially suspected to be breast carcinoma. RDD may still be a diagnostic challenge, especially in a patient with suspected carcinoma. It is the pathologist’s role to lead the clinician to the proper diagnosis and render a correct histopathologic diagnosis.

Primary intraosseous Rosai-Dorfman disease: a clinicopathological analysis of fourteen cases

Objective: To investigate the clinicopathological characteristics, histogenesis, immunophenotypes and molecular genetic features of primary intraosseous Rosai-Dorfman disease (RDD) for improving diagnostic accuracy and differential diagnosis. Methods: This retrospective study included 14 RDD cases diagnosed from January 2009 to January 2019 at Beijing Jishuitan Hospital, China. The immunohistochemical staining for S-100, cyclin D1, CD1a and CD207 expression was analyzed. The BRAF V600E and KRAS mutation analyses were performed using the Scorpions amplification refractory mutation system (ARMS) fluorescence quantitative PCR. Results: There were 6 female and 8 male patients, aged from 2 to 64 years (mean 31.4 years). All of the 14 cases occurred in the bone without lymph node disease, while one patient developed additional lesions within vertebra and nasal cavity. Radiographically, the lesions were lytic with sclerotic margins. Histologically, the lesions percolated through the medullary cavity in an infiltrative fashion and alternating hyper- and hypo-cellular regions of histiocytic clusters (seen as alternating dark and light zones at low magnification). Large histiocytes also showed emperipolesis. Some cases had areas of fibrosis and dense lymphoplasmacytic infiltrates. There were vasculitis and an increased number of plasma cells in the cases involving multiple sites. One case showed concurrence of RDD and Langerhans cell histiocytosis(LCH) with inconspicuous increase of Langerhans histiocytes. Immunohistochemical staining showed that the large histiocytes were positive for S-100, CD68 and CD163 in all cases. The nuclear immunoreactivity for cyclin D1 was observed in 13 of the 14 cases. S-100, CD1a and CD207 were positive in the case with concurrence of RDD and LCH. ARMS-PCR results showed that BRAF V600E mutation was observed in the cases with concurrence of RDD and LCH, while there were no KRAS mutations (7/7). Follow-up information was available for 12 patients and ranged from 9 to 49 months. Three of the 12 patients experienced recurrences after the first surgery. Conclusions: Primary intraosseous RDD is rare, and its concurrence with LCH is a very rare phenomenon. Its clinical symptoms, imaging, and pathological manifestations need to be distinguished from other bone lesions. The molecular detection of BRAF V600E and the nuclear expression of cyclin D1 mutations can be used for the diagnosis and differential diagnosis of RDD.

Cytological Diagnosis of Rosai-Dorfman Disease: A Study of Twelve Cases with Emphasis on Diagnostic Challenges.

Context:Rosai–Dorfman disease, also known as Sinus histiocytosis with massive lymphadenopathy, is a benign proliferative disorder of histiocytes. It typically affects lymph nodes; however, extranodal disease is being increasingly reported. The latter entity poses exceptional diagnostic challenge clinico-radiologically by forming mass lesions. Fine needle aspiration cytology (FNAC) is the investigation of choice to avoid unnecessary surgery as the majority are self-limiting.Aims:The objective is to assess the utility of FNAC in the diagnosis of Rosai–Dorfman disease and to highlight the diagnostic difficulties.Material and Methods:The cytology features of 12 cases of Rosai–Dorfman disease were analyzed and correlated with histopathology and immunohistochemistry.Results:The present study included six nodal and six extranodal Rosai–Dorfman disease. The cytology smears showed a variable number of large histiocytes exhibiting characteristic emperipolesis. Ten cases diagnosed as Rosai–Dorfman disease on cytology were confirmed on histopathology. The presence of granulomas, atypical histiocytes, insignificant emperipolesis, and eosinophil infiltration were the challenges we faced.Conclusions:FNAC, a simple and cost-effective method with its unique cytology features is the first line of investigation in the diagnosis of Rosai–Dorfman disease.

ORAL SIGNS IN A PATIENT WITH UNDIAGNOSED ROSAI-DORFMAN DISEASE: A CASE REPORT

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.

Enfermedad de Rosai-Dorfman con presentación cutánea y ausencia de mutaciones BRAF-V600, KRAS y NRAS: ¿trastorno neoplásico o reactivo

Non-Langerhans cell histiocytosis, including Rosai-Dorfman disease (RDD) and xanthogranuloma are rare disorders with occasional overlapping in the histopathological and immunohistochemical (IHC) findings. We report the case of a 53-year-old woman with erythematous-violaceous plaques on the cheeks and edema in the auricular pavilions. A biopsy was performed and the histopathological examination revealed a histiocytic proliferation with emperipolesis characteristic of RDD and lymphoplasmocitic infiltrate. IHC analysis showed S100 and CD68 positivity in the histiocytes but was negative for CD1a, supporting the diagnosis of RDD. Molecular analysis failed to detect BRAF-V600, NRAS or KRAS mutation. We discuss the differential diagnosis of cutaneous non-Langerhans cell histiocytosis. Pathologist must be aware of unusual presentations of RDD and further treatment options must be explored for patients with unresectable lesions and/or resistance to the classical management of RDD.

Thirty Year Experience with Rosai Dorfman Disease treated with Radiation, Chemotherapy, and/or Surgery.

Rosai-Dorfman Disease (RDD) is a rare disorder with histiocytosis and massive painless lymphadenopathy. It usually develops from the accumulation of non-Langerhans type histiocytic cells inside lymph nodes. Because the etiology of RDD is not well understood, the treatment is still nonspecific and controversial. Current data on the optimal management of RDD is limited to small case reviews/series. Herein we aim to describe treatment and outcomes, including the use of and response to radiation, in a large cohort of patients with RDD. We report on 27 patients diagnosed with Rosai-Dorfman Disease, who were evaluated at a single institution. Patients were diagnosed with RDD between 1980 and 2016. All patients had pathologic confirmation of RDD. We included patients who had a minimum of 3 months of follow up after diagnosis of RDD. We included patients of any age, with unifocal or multifocal disease, lymphatic and/or extranodal disease, and patients with previous treatment for RDD at outside institutions. We report descriptive outcomes for these patients. Median length of diagnosis was 73 months (IQR: 23- 138). Median age at diagnosis was 43 (range: 3 – 81). Fifty-two percent of patients were female. Three patients (11%) had nodal involvement alone, 17 patients (63%) had extranodal disease alone, and 7 patients (26%) had nodal disease and extranodal involvement at presentation. The most common site of disease involved the head and neck in 13 patients (48%). The most common therapeutic approaches included local or systemic steroids (n=11, 41%), chemotherapy (n=11, 41%), radiotherapy (n=9, 33%), and surgery (n=15, 55%). Eighteen patients were treated with 2 or more modalities (67%). Four of nine (44%) patients treated with radiation experienced a sustained response in the irradiated field. Median dose of RT was 36 Gy (range: 20 – 50 Gy). Intensity modulated radiation therapy (IMRT) was used for 5 patients. Various chemotherapy regimens were employed over the years, Clofarabine and Cladrabine were the most common systemic treatments. Seven (out of 11) patients receiving chemotherapy had stabilization, improvement, or resolution of disease after chemotherapy. At time of last follow up, 13 patients (48%) were with no evidence of disease, 11 patients had short interval stable disease (41%), and 3 patients (11%) had progressive disease. The optimal management of RDD remains elusive. Based on our review it seems that multiple treatment options may benefit patients. Radiation could be useful as an alternative option to avoid systemic therapy. Keeping in mind that avoiding long-term treatment related side effects is paramount, clinical factors such as age, location of the lesion(s), and symptoms, must be considered when recommending therapy for this benign condition.

Orbital presentation of Rosai–Dorfman disease

Abstract 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.

Presentación orbitaria de enfermedad de Rosai-Dorfman

ObjectiveTo report a case of orbital mass and proptosis, diagnosed as Rosai-Dorfman disease (RDD). MethodsClinical case report based on the review of clinical charts, radiological images, and histopathology. ResultsA 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 tumour cytoreduction, with good response. DiscussionThe 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.

Histology of Rosai-Dorfman disease in a subset of patients with Erdheim-Chester disease: A distinct entity mainly driven by MAP2K1.

e24180Background: Diagnosis of Rosai-Dorfman disease (RDD) is based on histology which is characterized by infiltration by CD68+ CD1a- S100+ histiocytes with large nuclei and abundant lesions of em...

A Study on Clinical Characteristics and Magnetic Resonance Imaging Manifestations on Systemic Rosai-Dorfman Disease.

Background:Rosai-Dorfman disease (RDD) is typically characterized by painless bilateral and symmetrical cervical lymphadenopathy, with associated fever and leukocytosis. The aim of the current study was to summarize the clinical features and imaging characteristics of RDD, in an effort to improve its diagnostic accuracy.Methods:The study was analyzed from 32 patients between January 2011 and December 2017; of these, 16 patients had pathologically diagnosed RDD, eight had pathologically diagnosed meningioma, and eight pathologically diagnosed lymphoma. All patients underwent computed tomography and magnetic resonance imaging (MRI). Clinical features and imaging characteristics of RDD were analyzed retrospectively. The mean apparent diffusion coefficient (ADC) values of lesions at different sites were measured, and one-way analysis of variance and the least significant difference t-test were used to compare the differences between groups and draw receiver operating characteristic curves. The tumors were excised for biopsy and analyzed using immunohistochemistry.Results:The mean ADCs were (0.81 ± 0.10) × 10−3 mm2/s for intercranial RDD, (0.73 ± 0.05) × 10−3 mm2/s for nasopharyngeal RDD, (0.74 ± 0.11) × 10−3 mm2/s for bone RDD, and (0.71 ± 0.04) × 10−3 mm2/s for soft-tissue RDD. The optimum ADC to distinguish intracranial RDD from lymphoma was 0.79 × 10−3 mm2/s (62.5% sensitivity and 100% specificity) and to distinguish meningioma from intracranial RDD was 0.92 × 10−3 mm2/s (62.5% sensitivity and 100% specificity). Levels of C-reactive protein, erythrocyte sediment rate and D-dimer were significantly elevated (81%, 87%, and 75%, respectively). On immunohistochemistry, RDD was positive for both S-100 and CD68 proteins but negative for CD1a.Conclusions:Conventional MRI, combined with diffusion-weighted imaging and ADC mapping, is an important diagnostic tool in evaluating RDD patients. An accurate diagnosis of RDD should consider the clinical features, imaging characteristics, and the pathological findings.

Multimodality imaging-based evaluation of Rosai-Dorfman disease in the head and neck: A retrospective observational study.

Rosai–Dorfman disease (RDD) is an uncommon benign entity characterized histologically by lymphatic sinus dilatation due to histiocyte proliferation. This study was performed to delineate its imaging features, reviewed retrospectively in 12 patients (8 women and 4 men, mean age 58.2 years [range 27–84]) with pathologically confirmed RDD in the head and neck. The location, involvement, and imaging characteristics (CT, magnetic resonance imaging (MRI), and PET/CT) of all lesions were evaluated. Signal intensity on MRI images was compared to gray matter (orbital RDD) and adjacent muscle (sinonasal and cervical RDD). RDD in the head and neck involved multiple sites, primarily the sinonasal cavity (n = 7), neck (n = 3), and orbit (n = 1), with one case of simultaneous involvement of the sinonasal cavity, orbit, and neck. With sinonasal involvement, MRI signal intensity of the involved areas was isointense or slightly hyperintense relative to adjacent muscle on T1WI images and heterogeneous on T2WI images; with lacrimal involvement, it was isointense relative to gray matter on T1- and T2-weighted images; and with neck involvement, it was isointense relative to muscle on T1WI images and relatively hyperintense on T2WI images, with homogenous postcontrast enhancement in all sites of involvement. The lesions on CT were observed as enhancing masses with or without bony destruction. PET/CT showed hypermetabolism in one lesion in the neck. RDD is a rare disorder with multiple sites of involvement in the head and neck. Concomitant cervical lymphadenopathy with extranodal masses assisted by multimodal imaging may be useful in the diagnosis of RDD.

Extranodal Rosai–Dorfman disease arising in the heart: clinical course and review of literature

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.

Rosai-Dorfman Disease: Manifestations Outside of the Head and Neck.

Rosai-Dorfman disease (RDD) is an uncommon benign histiocytic disorder. Extranodal involvement occurs in 43% and most commonly involves the head and neck, skin, and bones. We review less common imaging findings outside the head and neck. The imaging manifestations of RDD overlap with a variety of neoplastic and nonneoplastic conditions. Confirmation of an RDD diagnosis is based on specific histologic features. Radiologic assessment is used to rule out multifocality on initial workup and to evaluate treatment response.

Endobronchial Rosai-Dorfmann disease mimicking lung carcinoma: case presentation

Rosai-Dorfman disease (RDD) is a rare nonmalignant disorder which seems to be a proliferation of histiocytes of unknown etiology that mainly affects lymph nodes but most other organs of the body. Our case is a diagnostic dilemma due to its atypical presentation. We report a history of Moroccan female 57 years old who was thought to have recurrent hemoptysis with endobronchial obstructive tumor and atelectasis of the lung. The diagnosis of Rosai-Dorfman disease was eventually made following a range of investigations including CT scans and histological analysis of specimens after bronchial debulking through rigid bronchoscopy. She has an upper right destroyed lobe and lobectomy was performed with histologically a definitive diagnosis of RDD. She remains well with no further progression of the disease. We think that Rosai-Dorfman disease can mimic endobronchialcarcinoma, so histological confirmation should be sought in all cases.

Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): A Case Report with Review of Literature

Sinus Histiocytosis with Massive lymphadenopathy, also commonly known as Rosai-Dorfman Disease is a rare non neoplastic histiocytic disorder first described by Rosai and Dorfman, seen predominantly in childhood and young adults. It is characterized by fever, painless massive cervical lymphadenopathy and elevated ESR levels. RDD is often misdiagnosed as lymphoma, tuberculosis or metastatic malignancy, so it is important to distinguish it from these conditions as the modes of treatment are different. Here we report a case of 18 years old male presenting with massive lymphadenopathy. Morphological findings demonstrated Emperipolesis consistent with a diagnosis of Rosai-Dorfman disease. Clinical and cytological aspects of the

Rosai-Dorfman Disease Presenting with Recurrent, Unilateral Submandibular Lymph Adenitis: a Case Report.

Rosai-Dorfman Disease (RDD) is a rare disease of unknown aetiology, initially described as a separate entity in 1969 by Rosai and Dorfman. We describe here a case of RDD presenting with acute, recurrent lymphadenitis and massive lymphadenopathy. The patient's good response to the antibiotics was a false pointer towards infective pathology. The repeated fine needle aspiration cytology was inconclusive or suggested nonspecific reactive hyperplasia. The excision biopsy confirmed the diagnosis of RDD and was supported by immune-histochemistry for S-100 antigen. No other lymph node group was involved clinically or radiologically. The patient was treated with long-term antibiotic and low-dose predenisolone. The residual lymph node mass was excised after 3months of first surgery. The patient is on regular follow-up for last 2years and no recurrence has been reported.

Rosai-Dorfman Disease Involving Thyroid and Parotid Glands in Male with 48-Year-Old

Rosai-Dorfman disease (RDD) is a rare and self-limiting non-Langherhans histiocytosis with lymph node and cutaneous manifestations. This sinus histiocytosis is very extremely usual in children that progress to massive lymphadenopathy. Emperipolesis is a common Histopathological findings and protein S-100 is positive in most cases of RDD. Here, report a case of a 48-year-old with adult with RDD that exhibited cervical lymphadenopathy without fever or Osteolyticlesions. Histopathological and immunohistochemistry studies of lymph node showed emperipolesis, S-100 protein, CD68 positive and macrophages negative for CD1a. After that the diagnosis of RDD was done. However, cervical lymphadenopathy was not solved spontaneously, and the treatment with corticosteroid therapy was initiated and the follow-up showed a clinical improvement condition of the patient.

Cerebral Rosai-Dorfman disease.

Sinus histiocytosis (Rosai-Dorfman disease) with massive lymphadenopathy is a rare nonneoplastic and nonlangerhans cell proliferation disorder of the histiocytes. Extranodal location with or without lymphadenopathy occurs in about 40 % of the cases. Intracranial location is rare in children often mimicking meningiomas. The parasphenoidal region is more frequently involved though intraxial or intraventricular locations were described as well. Rarely, the surgical treatment allows the complete excision of the lesion; however, in symptomatic cases, partial resections of the tumor allow to counteract its mass effect. Long survivals are possible, even without radiotherapy or chemotherapy, due to the frequent spontaneous benign evolution of the lesions. A 2-year-10-month-old girl presented with high fever and vomiting. One year ago, she had a period of muscular weakness in both legs that recovered completely. MRI of the brain revealed an axial enhancing lesion with ventricular spreading mainly to the left occipital horn and bilateral frontal periventricular infiltration. After steroid therapy, all the symptoms recovered. Partial removal of the occipital intraventricular lesion was performed and the diagnosis of Rosai-Dorfman disease was established and confirmed by the reference center. At the latest follow-up (16 months), the girl is without any neurological symptoms and without any treatment.

Rosai-dorfman disease with cervical lymphadenopathy and orbital involvement: a case report

Rosai-Dorfman disease (RDD), which is also called as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare histiocytic disorder which occurs due to the over-production of non Langerhans sinus histiocytes. It is a nonmalignant disorder that most frequently affects children and young adults and typically presents with fever, night sweats, nonpainful cervical lymphadenopathy, leukocytosis and an elevated ESR. Extranodal involvement may also occur, thus a variety of organs in the body can be affected. Although some viral etiology has been implicated, the disease generally is considered to have an unknown cause. RDD can often be misdiagnosed as lymphoma, leukemia or tuberculosis, so it is imperative to distinguish it from these conditions as well as other forms of histiocytosis because of difference in the modes of treatment. Diagnosis of Rosai-Dorfman disease is based on biopsy of affected tissue. Biopsy showing the presence of emperipolesis, or the engulfment of lymphocytes and other immune cells by histiocytes that express S-100 antigen is diagnostic of Rosai-Dorfman disease. Once diagnosed, further workup including imaging studies are undertaken in order to determine the extent of the disease. In majority of cases, the disease resolves on its own however, treatments including corticosteroids, chemotherapy, surgical treatment or radiotherapy are carried out in severe or persistent disease or when organ function is at stake (e.g. breathing obstruction, kidney failure, visual problems). The case we report is that of a 16 year old girl who presented with a 6 month history of gradual onset drooping of left upper eyelid with mild proptosis of the left eye alongwith mild drooping of right upper eyelid, low grade fever, night sweats and cervical lymphadenopathy. Blood workup showed increased ESR, CT scan of orbits showed superior orbital masses and diagnostic biopsy revealed Rosai-Dorfman disease.