Manifestation Of Rosai-Dorfman Disease Research Articles

Rosai-Dorfman disease: a rare presentation of extranodal involvement of isolated bone. Case report

Introduction: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis, is a rare disorder characterized by histiocyte proliferation. Case presentation: A 33-year-old man consulted the emergency department of a tertiary care institution in Cartagena de Indias, Colombia, due to a 6-month history of progressive deformity in the frontal right side of the face, associated with pain of slow progression, without any other symptoms or dermatological involvement. There were no other major findings on physical examination and laboratory tests performed were negative. Imaging scans obtained showed extensive inflammatory involvement of the frontal bone, which led to suspect osteomyelitis as the first diagnostic possibility. A biopsy of the lesion was performed with negative cultures for bacteria, which allowed establishing a diagnosis of extranodal Rosai-Dorfman disease with isolated bone involvement. Treatment with systemic corticosteroids was indicated with poor response, so methotrexate was added, achieving an evident improvement after 2 months. Conclusions: Little is known about the manifestations of Rosai-Dorfman disease and its treatment in the adult population. The present case report contributes to expanding the literature on this topic, which can present with rare symptoms that may pose challenges for its diagnosis.

Thyroid Rosai-Dorfman disease with infiltration of IgG4-bearing plasma cells associated with multiple small pulmonary cysts

BackgroundRosai-Dorfman disease (RDD) is a rare histiocytosis which involves principally lymph nodes. Thyroid involvement in RDD is a very rare situation, and lung involvement is even rarer.Case presentationWe report the case of a 46-year-old woman presenting a painless mass in the right side of the neck and subacute dyspnoea. Computerised tomography (CT) scans of the neck and thorax showed a large thyroid mass causing tracheal stenosis and multiple cystic lesions in both lungs. Subtotal thyroidectomy with a tracheal segment resection and histological analysis confirmed the diagnosis of nodal and extranodal (thyroid, tracheal and probably lung) Rosai-Dorfman disease (RDD) with the presence of increased numbers of IgG4-bearing plasma cells. Clinical, functional and radiological follow up 4 years after surgery without medical treatment did not show any disease progression.ConclusionsThis case report indicates a benign course of nodal RDD with thyroid and tracheal infiltration following surgical resection, association of typical histological signs of RDD (emperipolesis) with IgG4-related disease features, and that lung cysts might be a manifestation 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.

Recurrent Bilateral Orbital Infiltration as Primary Manifestation of Rosai Dorfman Disease

Rosai Dorfman disease, also known as histiocytosis with massive lymphadenopathy, is a very rare idiopathic disease. It is characterized by over production and accumulation of non Langerhans sinus histiocyte most often in lymph nodes, but may occur in other areas leading to organ damage. Based on the research, it can be caused by an infectious agent, immunodeficiency or autoimmunity and genetic causes. Extranodal manifestation is uncommon, however extranodal sites include liver, kidney, respiratory organs, orbit and eyeball. We present a case report of a 44-year-old female with recurrent bilateral orbital infiltration as first location of Rosai Dorfman disease.

Recurrent Bilateral Orbital Infiltration as Primary Manifestation of Rosai Dorfman Disease

Rosai Dorfman disease also known as histiocytosis with massive lymphadenopathy is a very rare idiopathic disease. It is characterized by over production and accumulation of non-Langerhans sinus histiocyte most often in lymph nodes, but may occur in other areas leading to organ damage. Based on the research it can be caused by an infectious agent, immunodeficiency or autoimmunity and genetic causes. Extranodal manifestation is uncommon; however extranodal sites include liver, kidney, respiratory organs, orbit and eyeball. We present a case report of a 44-year-old female with recurrent bilateral orbital infiltration as first location of Rosai Dorfman disease.

Sonographic Manifestation of Thyroid's Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare and benign histiocytic proliferative disorder of unknown etiology. It can affect all age groups, particularly young adults. The typical clinical manifestation is painless, bilateral, massive cervical lymphadenopathy with or without extra nodal involvement, along with fever, weight loss, and night sweats. However, thyroid involvement is very uncommon. The ultrasonographic manifestations of RDD are rarely reported. A case of RDD involving the thyroid that was recorded in our hospital PACS system during 2002–2010 is reported here, which was initially misdiagnosed as non-Hodgkin’s lymphoma (NHL). In our case, ultrasonography revealed diffuse enlargement of whole thyroid glands with heterogeneous hypoechogenicity. Areas of linear hyperechogenicity were noted on the sonograms with multiple enlarged nodes over the cervical, supraclavicular, and submandibular areas. Though not specific, in order to avoid unnecessary surgery or a total thyroidectomy, ultrasonography and ultrasonography-guided core needle biopsy should be performed to establish an accurate diagnosis.

Extranodal Rosai-Dorfman Disease Presenting as an Epibulbar Tumor

To describe a case of an epibulbar tumor as a manifestation of Rosai-Dorfman Disease (RDD) and review the pertinent literature. This was an interventional case report and literature review. A 19-year-old African-Brazilian man was referred for evaluation of a 12 x 11-mm subconjunctival mass at the temporal limbus OD. Intraocular pressure was normal, and funduscopy was unremarkable OU. Ultrasound biomicroscopy showed an ill-defined scleral homogeneous lesion at the temporal quadrant, without intraocular invasion. A superficial sclerokeratectomy was performed. Histopathologic evaluation revealed inflammatory aggregates composed of lymphocytes, plasma cells, and histiocytes. The histiocytes appeared pale, and some of them had intact lymphocytes and plasma cells within their cytoplasm. Russell bodies were also found. The histiocytes were positive for CD68 and S-100 and negative for lysozyme and CD1a. Special stains for microorganisms were all negative. Those findings were consistent with the diagnosis of extranodal RDD. Systemic workup failed to reveal lymphadenopathy or extranodal disease elsewhere. At the 14-month follow-up, there were no signs of recurrence. Although extranodal manifestations are common in RDD and the lack of lymph node involvement is rare, our study supports that whenever there is an epibulbar tumor as a manifestation of RDD, the absence of lymphadenopathy is characteristic. Only 2 of the 9 reported cases presented with lymphadenopathy. The presence of emperipolesis and S-100-positive histiocytes during histopathologic evaluation confirms the diagnosis even in the absence of lymphadenopathy.

Extranodal manifestation of Rosai Dorfman Disease of the nasopharynx.

The aim of presenting this case is to highlight the fact that extranodal manifestation of Rosai Dorfman Disease (RDD) without lymphadenopathy was seen in a teenaged girl with recurrent episodes of epistaxis, due to a lobulated mass in the nasopharynx, which mimicked malignancy. This case is a rare presentation because the extranodal manifestation of RDD in multisystem organs are reported in literature, but the nasopharyngeal manifestation without lymphadenopathy is unique. The clinical presentation and surgical management of the case by endoscopic sinus surgery, the aetiopathology, differential diagnosis and review of literature are described here.