Rosai dorfman disease sinus histiocytosis with massive lymphadenopathy was first described in 1969 as a benign proliferative lesion with systemic symptoms and lymphadenopathy. Pulmonary involvement during rdd has been poorly described. Mutually exclusive recurrent kras and map2k1 mutations in. Destombes rosai dorfman disease drd is an inflammatory nonlangerhansian histiocytosis with adenomegaly. It is a rare histiocytic disorder worldwide, less than reported cases. Rosai dorfman disease is a rare disorder of histiocytes, which present as massive cervical lymphadenopathy. It is a rare benign disease with typical clinical manifestation of painless enlargement of bilateral cervical ly the cause of. Rosaidorfman sinusal histiocytosis is a benign histiocytic disorder presenting as large. Rosai dorfman disease was first described by rosai and dorfman in 1969. Rosaidorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign histioproliferative disorder of unknown etiology. She was treated with corticosteroids and improved clinically. Thyroid involvement in rdd is a very rare situation, and lung involvement is even rarer.
Rosai dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare, idiopathic, nonneoplastic histiocytic proliferative disorder 14. Isolated intracranial rosaidorfman disease mimicking a. It occurs more often in children and young adults and shows a mild male sex predilection. However, chronic disease can occur with patterns of stability, slow progression, exacerbation, or spontaneous remission. Rdd is a benign disorder that presents with massive lymphadenopathy, but can have extranodal involvement. The destombesrosaidorfman disease or sinus histiocytosis. Thyroid rosaidorfman disease with infiltration of igg4. Pdf rosai dorfman disease of the orbit santosh honavar. Destombes rosai dorfman disease rdd is a rare multisystemic histiocytosis. Rosai dorfman disease more details is a nonlangerhans cell histiocytosis first described in 1965 by destombes and recognized as a distinct clinicopathological entity by rosai and dorfman in 1969. Pathologic findings including immunohistochemistry suggested the diagnosis. Lung involvement in destombesrosaidorfman disease chest. Rosai dorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy shml, is a disease of histiocytic proliferation with no known pathogenesis.
Rosai dorman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, was originally described by destombes in 1965. Sinus histiocytosis with massive lymphadenopathy rosai dorfman disease rdd is a benign entity first described by rosai and dorfman in 1969 1 and characterized by marked, painless lymph node enlargement. Cutaneous rosai dorfman disease crdd is a rare form of rosai dorfman disease rddalso known as sinus histiocytosis with massive lymphadenopathy that has a varied clinical presentation, an unknown etiology, and multiple treatment options that lack efficacy. Other lymph node groups may also be involved and, in some cases, abnormal. This is usually on both sides and is painless but often get very. A new presentation of isolated cutaneous rosaidorfman. The full text of this article is available in pdf format. This paper presents a case of a 15yearold male patient who presented with nasal obstruction and was surgically treated for a mass filling in the left nasal meatus that was. Rosai dorfman disease generally has a longterm clinical course characterized by exacerbations and remissions and appears to undergo complete remission in most patients.
Rosaidorfman disease bn mahanta1, t goswami mahanta2 abstract a young male presented with recurrent neck swellings with initial leucocyte count of 16800mm 3, with noncaseating glands on ultrasonography neck. Le traitement avait consiste en une chimiotherapie asso ciant en j1. However, more than 87% of patients present with enlargement of the lymph nodes in the neck. We describe a case of a 59yearold woman who presented with heart failure symptoms from a large pericardial mass causing constrictive pericarditis. Rosaidorfman disease originating from nasal septal mucosa. Rosai dorfman destombes disease rdd is a rare nonlangerhans cell histiocytosis lch first described in 1965 by a french pathologist, pierre paul louis lucien destombes, who reported 4 children and young adults with lymphadenopathy and sinus histiocytosis upon histologic analysis. Rosai dorfman disease rdd, sinus histocytosis with massive lymphadenopathy is an idiopathic histocytic proliferation affecting the lymph nodes in its classic form. Intracranial involvement is relatively rare and isolated intracranial rdd is very scarce. Rosai dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare nodal andor extranodal histiocytic disorder characterized by the accumulation of abnormal. Here we report a case of rdd that presented a diagnostic dilemma due to its atypical presentation and the overlap with igg4 disease. Rosai dorfman disease rdd is a rare histiocytosis which involves principally lymph nodes.
Rosai dorfman disease is a rarely seen disease with unknown etiology. Rosai dorfman disease commonly involves cervical lymph nodes. Rosai dorfman disease is a rare, idiopathic disorder of histiocyte proliferation. The goal of this study was to examine the clinical presentations, radiological features, and outcomes of 15 patients with rdd and lung involvement. Rosaidorfman disease sinus histiocytosis with massive lymphadenopathy shml rosaidorfman disease rd, also known as sinus histiocytosis with massive lymphadenopathy shml, is a rare histiocytic disorder which involves the overproduction of a type of white blood cell called non langerhans sinus histiocyte.
Rosaidorfman disease and neurological manifestations. Rosai dorfman disease rdd is an uncommon benign histiocytic disorder. Rosai dorfman disease rdd is a rare idiopathic his to proliferative disorder that usually presents with systemic symptoms and cervical lymphadenopathy. It is a benign disease which is characterized by overproduction and accumulation of a specific type of white blood cell in the lymph nodes of the body, most often those of the neck cervical. Most patients with rdd present with multiple lymphadenopathy, although rdd also involves a.
Rosaidorfman disease symptoms histiocytosis association. Extranodal rosaidorfman disease american journal of. Consensus recommendations for the diagnosis and clinical. Rosai dorfman disease rdd, juvenile xanthogranuloma jxg, and erdheimchester disease ecd are nonlangerhans cell nonlch disorders arising from either a dendritic or a macrophage cell. Extranodal involvement is most commonly seen in the head and neck region.
Rosai dorfman disease rdd is a rare nonmalignant proliferation of histiocytes of unknown aetiology that mainly affects lymph nodes. It is of unknown etiology and is characterized in its classical form by multiple adenomegalia, especially localized at cervical area, but also at mediastinal, axillary and inguinal areas. Cutaneous rosai dorfman disease crdd is a rare form of rosai dorfman disease rddalso known as sinus histiocytosis with mas sive lymphadenopathy that has a varied clinical presentation, an unknown etiology, and multiple treatment options that lack efficacy. Rosai dorfman disease should be added in the list of differential diagnosis for a dural mass mimicking meningioma or cerebral mass mimicking glioma, therefore, immunohistochemical staining for ema, s100 and cd1a should be performed to rule out the differential diagnosis.
Rosai dorfman may affect lymph nodes only, it can affect lymph nodes plus other body systems, or it can occur in body systems without lymph node involvement. Cutaneous rosai dorfman disease typically occurs in older females and presents in various forms, ranging from single papules to multiple nodules and plaques. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for rosaidorfman disease. It is most commonly seen in male teenagers and young adults. Rosaidorfman disease genetic and rare diseases information. Extranodal involvement occurs in 43% and most commonly involves the head and neck, skin, and bones.
In rare instances, rosai dorfman disease can take on a more aggressive, potentially fatal. Emperipolesis, intracranial rosaidorfman disease introduction. This disease is defined by histological and cytological characteristics, including emperipolesis and positive s100 and cd68 markers. We present a case of a 31yearold woman who presented with. Histopathologically, it is characterized by histiocytic cell proliferation. Rosai dorfman disease is a rare benign disease that was first described by rosai and dorfman as sinus histiocytosis with massive lymphadenopathy in the 1960s. Fnac showed mixed cellularity with histiocytic and marked lymphophagocytosis as seen in rosai dorfman disease. Rosaidorfman disease presenting in the gastrointestinal tract. Rosaidorfman disease rdd, a sinus histiocytosis with massive lymphadenopathy, represents an idiopathic proliferation of peculiar, histiocytelike cells of unknown etiology.
Rosai dorfman disease aka sinus histiocytosis with massive lymphadenopathy is a benign histiocytic proliferation that was first described in lymph nodes. Here we describe a case of extranodal rosai dorfman disease involving the. Case report extranodal rosaidorfman disease presenting as. Rosai dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy or sometimes as destombes rosai dorfman disease, is a rare disorder of unknown cause that is characterized by abundant histiocytes in the lymph nodes or other locations throughout the body.
Age of presentation the disease usually occurs in the first two decades of life, in children as well as in young adults mean age 20,6, with a slight predominance in men. Although the disease typically presents clinically with massive bilateral lymphadenopathy. The case history is presented of a woman who was thought to have atypical carcinoma of the lung but in whom a diagnosis of rosai dorfman disease was eventually made following a range of investigations including ct scans, positron emission tomography pet and. Rosai dorfman disease a clinicopathological presentation. Rosai dorfman disease is a rare disorder which seems to involve not only the lymph nodes but most other organs of the body. Arch pathol lab medvol 127, february 2003 rosai dorfman disease in the gi tractanders et al e75 a, crosssection of large bowel wall revealing a submucosal mass that in. Rosai dorfman disease, also called sinus histiocytosis with massive lymphadenopathy, is a nonlangerhans histiocytic disease. Computerised tomography ct scans of the neck and thorax showed a large thyroid mass. Rosai dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy shml, was first described systematically by rosai and dorfman in 1969.
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