Extranodal Rosai-Dorfman Disease: a rare presentation involving anterior chest wall in a middle-aged female.

Rosai-Dorfman Disease is a rare benign disorder involving overproduction of immune cells, causing swollen lymph nodes and, in rare cases, the sternum. The sternal involvement may cause chest pain and masses. Diagnosis is confirmed through clinical examination, biopsy, and imaging. Treatment options may include surgery, radiation, or steroids. In this case study, we present an unusual example of extranodal Rosai-Dorfman Disease involving the sternum, bilateral clavicles and first three ribs, and pectoral muscle with no associated lymphadenopathy or systemic symptoms in a 57-year-old female. The etiology, pathology, immunohistochemistry, imaging findings, and treatment options of this unique disease are discussed.

Rosai-Dorfman Disease in a Pediatric Patient: Imaging Findings and Pathology with a brief review of the Literature.

Rosai-Dorfman Disease, otherwise known as sinus histiocytosis with massive lymphadenopathy, is a rare form of non-Langerhans cell histiocytosis with an estimated incidence of 100 cases per year in the United States. Due to its variable presentation and nonspecific clinical findings, it is particularly difficult to diagnose in pediatric patients. We report a case of an 11-month-old male who presented with a 4-day history of a right groin mass. Ultrasound of the groin and pelvis demonstrated, and MRI of the abdomen and pelvis confirmed an inguinal mass with surrounding lymphadenopathy. Pathology confirmed Rosai-Dorfman Disease and the patient improved after starting oral steroid therapy. To the best of our knowledge, this is the first case of Rosai-Dorfman Disease involving the inguinal region in an infant under 1 year of age reported in the literature. In this case report, we discuss the imaging and histology findings as well as provide a brief literature review for this diagnosis.

Rosai-Dorfman disease of the breast: a clinicoradiologic and pathologic study.

Rosai-Dorfman disease (RDD) is an uncommon histiocytic disorder typically involving lymph nodes and less frequently extranodal tissues. RDD involving the breast is rare and may clinically and radiologically mimic neoplastic and non-neoplastic disorders. We report seven patients with breast RDD, describe their clinicoradiologic and pathologic features, and discuss the differential diagnosis. Patients, ranging from 15 to 74 years of age, presented with unilateral and unifocal (5/7) or bilateral and multifocal (2/7) masses. RDD was either confined to the breast (6/7) or concurrently involved a lymph node (1/7). Masses ranged from 8 to 31 mm, categorized as Breast Imaging-Reporting and Data System (BI-RADS) 4 (6/7) or 5 (1/7). All cases showed similar morphology with many large histiocytes displaying emperipolesis with associated fibrosis and dense lymphoplasmacytic infiltrate. The abnormal histiocytes co-expressed CD68/CD163, S100, OCT2, and Cyclin D1 (7/7), and were negative for CK AE1/AE3 (7/7), CD1a (7/7), and BRAF V600E (6/6). Flow cytometry (n = 3), kappa/lambda in situ hybridization (n = 5), and IgG4/IgG immunohistochemistry (n = 1) did not reveal lymphoma or IgG4-related disease. No mycobacterial or fungal organisms were identified on acid-fast bacillus (AFB) and Grocott methenamine silver (GMS) stains (n = 5). Three patients underwent complete excision and none recurred or progressed to systemic disease during follow-up (88-151 months). In summary, breast RDD should be included in the differential diagnosis of a mass-forming breast lesion. Histopathology with ancillary studies and clinicoradiologic correlation is essential for accurate diagnosis and optimal clinical management. Patients with RDD of the breast have an excellent prognosis after complete excision.

Successful anesthesia management with inhalation anesthesia in Rosai-Dorfman disease: a case report.

Rosai-Dorfman disease (RDD) is a rare, benign, non-Langerhans cell histiocytic proliferative disease. RDD with central nervous system involvement is extremely rare. Surgical excision is generally regarded as the appropriate treatment of choice for this disease, especially when the lesion causes neurological compression. RDD can be accompanied by systemic symptoms, such as malaise, fever, weight change, leukocytosis, anemia, and hormonal disturbance, which may be challenging during general management. Little is known regarding peri-anesthesia management of this rare disease. We report a case of a patient in his 20s who had recurrent RDD and had general anesthesia with perioperative management. He was obese and hepatic insufficiency. This case report adds to the literature regarding the perioperative anesthetic management of RDD with central nervous system involvement.

Intracranial Rosai-Dorfman disease: a case report and review of the literature.

RDD involving the central nervous system is rarely reported. To better understand the disease and explore the treatments, we reported this case and review the literature.

Cerebral manifestation and diagnostic dilemma of Rosai-Dorfman disease.

Rosai-Dorfman disease (RDD) is a rare, S100-positive histiocytic proliferation, that can cause both nodal and extranodal illness. We present a case of a 53-year-old male patient. Magnetic resonance imaging described a plaque-like meningeal lesion, and the preoperative diagnosis was meningioma. Histologically, dense infiltration of lymphocytes, plasma cells, and histiocytes was seen, furthermore, the presence of emperipolesis in the sample was pronounced. In the histiocytes nuclear and cytoplasmic positivity with S100 protein, and nuclear positivity with Cyclin D1 was observed. The case was concluded as RDD. Morphological appearance of intracranial RDD with imaging procedures can present a differential diagnostic challenge. The correct diagnosis is based on the presence of histiocytes with emperipolesis, and properly defined immunohistochemical characteristics.

Temporal bone manifestation of primary extranodal Rosai-Dorfman disease: a case report.

BACKGROUND: Rosai-Dorfman disease is a rare benign histiocytic disorder characterized in most cases by painless cervical adenopathy. Less than 10% of extranodal cases involve bony lesions. Primary bone Rosai-Dorfman disease in the absence of nodal disease is extremely rare. CASE PRESENTATION: A 48 year-old Caucasian male presented with progressive right-sided otalgia, tinnitus, vertigo, and hearing loss. A right temporal bone lytic lesion was detected on diagnostic imaging. Resection of the lesion and histopathological examination revealed Rosai-Dorfman disease. CONCLUSIONS: Rosai-Dorfman disease primary bone lesions are an atypical presentation of a rare disease. This is the second reported case of Rosai-Dorfman disease arising within the temporal bone. This case study reveals that Rosai-Dorfman disease should be considered for patients presenting with inflammatory/lytic lesions of the temporal bone, in cases where infection and malignancy have been excluded.

The value of 18F-FDG PET/CT in the systemic evaluation of patients with Rosai-Dorfman disease: a retrospective study and literature review.

BACKGROUND: Rosai-Dorfman disease (RDD) is a rare form of non-Langerhans cell histiocytic disease. The aim of this study was to review the characteristics of RDD using 18F-FDG PET/CT and determine its efficacy in the disease management. RESULTS: A total of 28 RDD patients underwent 33 18F-FDG PET/CT scans for systematic assessment and follow-up. The common involved sites included the lymph nodes (17, 60.7%), upper respiratory tract (11, 39.3%), and skin (9, 32.1%). Five patients had more lesions detected in PET/CT images than in CT and/or MRI, including inapparent nodules (n = 5) and bone destruction (n = 3). After thorough treatment evaluation using PET/CT, the treatment strategies of 14 patients (14/16, 87.5%) were changed. Five patients underwent PET/CT twice during follow-up and the SUVs were significantly decreased (15.3 ± 3.4 vs. 4.4 ± 1.0, p = 0.02), which demonstrated disease improvement. CONCLUSIONS: 18F-FDG PET/CT contributed to displaying the holistic characteristics of RDD, in particular during initial assessment, treatment strategy adjustment, or efficacy evaluation, and could compensate for some disadvantages of CT and MRI images.

Characterizing Rosai-Dorfman disease with [18F]FDG PET/CT: a retrospective analysis of a single-center study.

OBJECTIVES: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytic proliferative disorder. We performed a retrospective study to characterize the clinical and [18F]FDG PET/CT features of RDD. METHODS: We retrospectively recruited 38 RDD patients with [18F]FDG PET/CT scan in our center. [18F]FDG PET/CT features were assessed, and clinical and follow-up data were recorded. RESULTS: In the recruited patients, 20/38 (52.6%) patients had single-system disease, while others (18/38, 47.4%) had disease affecting multiple system. RDD most commonly involved the upper respiratory tract (47.4%), followed by cutaneous/subcutaneous lesion (39.5%), lymph node (36.8%), bone (31.6%), central nervous system (28.9%), and cardiovascular system (13.2%) in the recruited patients. In PET/CT, the RDD lesions were FDG-avid, and the SUVmax of the hottest lesion in an individual patient was positively correlated with C-reactive protein levels (r = 0.418, p = 0.014), and negatively correlated with hemoglobin levels (r = -0.359, p = 0.036). The overall response rate of the first-line treatment was 80.8% in newly diagnosed RDD patients, and for patients with relapsed/progressive RDD, the overall response rate was 72.7%. CONCLUSION: [18F]FDG PET/CT could be a useful tool for evaluating RDD. KEY POINTS: • About half of the patients with Rosai-Dorfman disease had single-system disease, while others had disease affecting multiple system. Rosai-Dorfman disease most commonly involved the upper respiratory tract, followed by cutaneous/subcutaneous lesion, lymph node, bone, central nervous system, and cardiovascular system. • In [18F]FDG PET/CT, Rosai-Dorfman disease was usually hypermetabolic, and the SUVmax of the hottest lesion in an individual patient was positively correlated with C-reactive protein levels. • Rosai-Dorfman disease usually has a high overall response rate after treatment.

Rosai-Dorfman Disease in the Skull Base: A Case Series Study.

OBJECTIVE: Rosai-Dorfman disease (RDD) is a rare, idiopathic, and non-neoplastic histio-proliferative disease that is uncommon in the central nervous system. Hence, reports of management of RDD in the skull base are scarce and only a few studies on skull base RDD are available. The objective of this study was to analyze the diagnosis, treatment, and prognosis of RDD in the skull base and explore an appropriate treatment strategy thereof. METHODS: Nine patients with clinical characteristics and follow-up data from our department between 2017 and 2022 were included in this study. From this information, the clinical profiles, imaging, treatment, and prognosis data were collected. RESULTS: There were 6 male and 3 female patients with skull base RDD. These patients ranged in age from 13 to 61 years, with a median age of 41 years. The locations included 1 anterior skull base orbital apex, 1 parasellar region, 2 sellar regions, 1 petroclivus, and 4 foramen magnum regions. Six patients underwent total resection and 3 underwent subtotal resection. Patient follow-up lasted 11-65 months, with a median duration of 24 months. One patient died, 2 experienced recurrence, and the other patients' lesions were stable. The symptoms worsened and new complications occurred in 5 patients. CONCLUSIONS: Skull base RDDs are intractable diseases with a high rate of complications. Some patients are at risk of recurrence and death. Surgery may be the basic treatment for this disease, and combined therapy including targeted therapy or radiation therapy may also be a valuable therapeutic strategy.

Rosai-Dorfman disease with spinal and multiple intracranial involvement: a case report and literature review.

Rosai-Dorfman disease (RDD) is a condition of unknown etiology, and characterized by the proliferation of histiocytes. RDD most commonly affects lymph nodes, and central nervous system (CNS) involvement is rare. Here, we describe the case of a 43-year-old man who presented with an intradural tumour of the thoracic spine. The patient underwent a laminectomy for tumour resection and pathology results diagnosed the tumour as a RDD. Two years later, brain magnetic resonance imaging (MRI) revealed multiple intracranial dural-based lesions. Prednisolone treatment was initiated and led to resolution of the disease. We reviewed the literature to the investigate clinical characteristics, imaging features, diagnosis and treatment protocols pertaining to such cases.

Rosai Dorfman Disease With Extensive Bony Involvement- A Diagnostic Dilemma.

Previously classified as Non Langerhan cell histiocytosis by the Working Group of Histiocytic Society in 1987 Rosai Dorfman Destombes disease was first described by Destombes in 1965 and later in 1969 by Rosai and Dorfman as a rare histiocytic disorder with sinus histiocytosis and massive lymphadenopathy. They exist in both nodal and extranodal forms. Immunohistochemistry is an essential part of diagnosis to differentiate between Langerhans cell histiocytosis and another malignant histiocytosis. Some overlap has also been reported with IgG4-related diseases. We hereby reflect upon a patient who presented to our facility with pyrexia of unknown origin, the challenges faced to reach a diagnosis and the management offered.

A Rare Case of Thymic Rosai-Dorfman Disease Mimicking Malignancy on 18F-FDG PET/CT.

Background Rosai-Dorfman disease (RDD), the massive lymphadenopathy characterized by the proliferation of sinus histiocytosis, is a relatively idiopathic benign disease with unknown etiology. We reported a rare case of thymic RDD detected by 18F-FDG PET/CT. A 23-year-old man with right-sided chest pain underwent 18F-FDG PET/CT scan, showing increased 18F-FDG uptake in an anterior mediastinal mass corresponding to a thymic lesion at an enhanced CT scan. The patient was referred to surgery with the clinical suspicion of thymic malignancy. The histological examination and immunohistochemical results confirmed RDD. Conclusions This was the first case report of RDD isolated to the thymus and initially presented with chest pain. Moreover, there was no characteristic painless neck lymphadenopathy at any stage of the disease course. Thus, for young patients with thymus mass, RDD should be considered a rare but possible diagnosis.

68Ga-FAPI and 18F-FDG PET/CT Images of a Patient With Rosai-Dorfman Disease With Liver Involvement.

ABSTRACT: Rosai-Dorfman disease is a rare non-Langerhans cell histiocytosis that is histopathologically characterized by the accumulation of CD68-positive and S100-positive histiocytes. The pathological changes are mostly discovered in lymph nodes. We report a case of Rosai-Dorfman disease with liver and bone marrow involvement. In this case, 18F-FDG PET/CT revealed FDG avidity in multiple lymph nodes, liver, and bone marrow. 68Ga-FAPI PET/CT showed higher uptake of 68Ga-FAPI than that of 18F-FDG in the same lesions. The findings of 68Ga-FAPI PET/CT in this patient highlighted the potential value of 68Ga-FAPI for staging in Rosai-Dorfman disease.

Radiological features of Rosai-Dorfman disease: case series and review of the literature.

AIM: To improve the accuracy of diagnosis of Rosai-Dorfman disease (RDD) by summarising the computed tomography (CT) and magnetic resonance imaging (MRI) characteristics. MATERIALS AND METHODS: The clinical manifestations, imaging findings, and pathological characteristics of 14 patients with histopathologically confirmed RDD were analysed retrospectively and a literature review was undertaken. RESULTS: Of the 14 patients, nine had multiple lesions and five had single lesions. Eight patients had extranodal lesions, while six had mixed-type lesions. In patients with head and neck lesions, plain CT/MRI revealed irregularly shaped, well-defined, homogeneous, and mainly progressive lesions, with marked homogeneous enhancement on multiphasic contrast-enhanced imaging. One patient had dural lesions, one of which iso-intense to grey matter, with patchy hypo-intensity on T2-weighted imaging, meningeal tail signs, and characteristic crabfoot-like enhancement. Three patients with skeletal system involvement exhibited osteolytic bone destruction without sclerosis at the edges, associated soft-tissue masses, or periosteal reactions. Two patients had well-defined subcutaneous lesions, inhomogeneous density, and progressive parenchymal enhancement on contrast-enhanced CT. One patient had multiple intestinal lesions with inhomogeneous nodular thickening of the blind ascending colon and ileum, with marked and progressive enhancement. CONCLUSION: RDD involvement is mainly multifocal, primarily in the head and neck regions. Plain CT/MRI revealed well-defined, irregularly shaped lesions with homogeneous density/signal, with marked and progressive enhancement on multiphasic contrast-enhanced imaging; however, histopathology is still required to confirm the diagnosis of RDD.

[Magnetic Resonance Imaging Manifestations of Rosai-Dorfman Disease in Central Nervous System].

Objective To investigate the clinical and magnetic resonance imaging(MRI) manifestations of Rosai-Dorfman disease(RDD) in central nervous system. Method The clinical and MRI data of 5 cases of RDD in central nervous system confirmed by pathology in the PLA General Hospital were analyzed retrospectively. Results The 5 cases included 4 males and 1 female,aged(39.8±21.7) years on average.Among them,4 cases were located in the intracranial area and 1 case in the thoracic spinal canal.The lesion showed isointense signal on T1 weighted image and iso,slight-hypo,and slight-hyperintense signals on T2 weighted image,and it presented intensively homogeneous enhancement in contrast-enhanced MRI.Two cases showed compressed brain area with edema around the left parietal and left frontotemporal dura,thickening and enhancement in the adjacent dura,and dural tail sign.Three cases presented bone destruction in adjacent diploe and thoracic vertebrae.One case showcased slight-hypo perfusion of the left parietal dura in arterial spin labeling. Conclusions RDD lesion usually appears as iso,slight hypo and slight hyper-intense signals on T2 weighted image and presents intensively homogeneous enhancement in contrast-enhanced MRI.The disease may involve the adjacent bone and the lesion shows slight hypo-perfusion on perfusion images.The MRI manifestations of RDD are characteristic,which are helpful for preoperative diagnosis and evaluation of RDD.

Management of central nervous system Rosai-Dorfman disease: A single center treatment experience.

Rosai-Dorfman disease (RDD) is an idiopathic histiocytic proliferation disease with various clinical manifestations. A retrospective study of patients with pathological diagnosed RDD primarily involved in the central nervous system was conducted from January 2011 to December 2020 at a tertiary center. The clinical profile, imaging, and treatment data were collected. There were 16 male and 5 female patients with RDD-CNS. The patients were aged from 6 to 68 years with a median of 37 years. Of these 21 patients, 15 presented with intracranial RDD and 6 with spinal RDD. The main symptoms of RDD-CNS included headache, epilepsy, and neurological deficits. 76.19% (16/21) of the patients showed dura-based, homogeneous enhancement lesion on magnetic resonance imaging (MRI). Twenty patients received surgery as first treatment, and one patient received biopsy after steroid therapy. Total lesion resection was achieved in 42.9% (9/21) of the patients, subtotal resection in 47.6% (10/21), and biopsy in 0.9% (2/21). The symptoms were alleviated or stayed stable. Some RDDs (80%, 4/5) in the skull base had some complications. The patients were followed up for 11-108 months with a median duration of 47 months. Lesion progression or recurrence was found in two patients. The various clinical manifestations, as well as the dura-based and homogenous enhancement imaging profiles of RDD-CNS patients pose a great diagnostic challenge for clinicians. Surgery is effective for RDD-CNS requiring treatment. Medical therapy and radiotherapy would be feasible as noninvasive treatments, varying degrees of efficacy. The overall prognosis of RDD-CNS is acceptable. Periodic long-term follow-up is necessary.