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. Thyroid involvement in rdd is a very rare situation, and lung involvement is even rarer. Rosai dorfman disease is a rare disorder which seems to involve not only the lymph nodes but most other organs of the body. 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. 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. Rosai dorfman disease rdd is a rare nonmalignant proliferation of histiocytes of unknown aetiology that mainly affects lymph nodes. 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. Rosaidorfman disease rdd, a sinus histiocytosis with massive lymphadenopathy, represents an idiopathic proliferation of peculiar, histiocytelike cells of unknown etiology. However, more than 87% of patients present with enlargement of the lymph nodes in the neck. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for rosaidorfman disease.
Rosai dorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy shml, is a disease of histiocytic proliferation with no known pathogenesis. Lung involvement in destombesrosaidorfman disease chest. Emperipolesis, intracranial rosaidorfman disease introduction. Extranodal rosaidorfman disease american journal of. It is a rare benign disease with typical clinical manifestation of painless enlargement of bilateral cervical ly the cause of. Rosaidorfman disease and neurological manifestations. We present a case of a 31yearold woman who presented with. She was treated with corticosteroids and improved clinically. 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. Here we report a case of rdd that presented a diagnostic dilemma due to its atypical presentation and the overlap with igg4 disease. Extranodal involvement is most commonly seen in the head and neck region. Although the disease typically presents clinically with massive bilateral lymphadenopathy.
Rosaidorfman disease symptoms histiocytosis association. Cutaneous rosai dorfman disease typically occurs in older females and presents in various forms, ranging from single papules to multiple nodules and plaques. Destombes rosai dorfman disease rdd is a rare multisystemic histiocytosis. This is usually on both sides and is painless but often get very. In rare instances, rosai dorfman disease can take on a more aggressive, potentially fatal. Rosaidorfman disease presenting in the gastrointestinal tract. We report the case of a 46yearold woman presenting a painless mass in the right side of the neck and subacute dyspnoea. Rosai dorfman disease rdd is a rare histiocytosis which involves principally lymph nodes. Rosai dorfman disease is a rare, idiopathic disorder of histiocyte proliferation. Case report extranodal rosaidorfman disease presenting as. Rosai dorfman disease commonly involves cervical lymph nodes.
Rosai dorfman disease is a rarely seen disease with unknown etiology. Rosaidorfman disease genetic and rare diseases information. Rosai dorfman disease was first described by rosai and dorfman in 1969. Rdd is a benign disorder that presents with massive lymphadenopathy, but can have extranodal involvement.
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. Most patients with rdd present with multiple lymphadenopathy, although rdd also involves a. Pathologic findings including immunohistochemistry suggested the diagnosis. 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. Extranodal involvement occurs in 43% and most commonly involves the head and neck, skin, and bones. 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. Clinically, it is most frequently characterized by massive painless cervical lymphadenopathy with other systemic manifestations, including fever, night sweats, and weight loss. It is most commonly seen in male teenagers and young adults. 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. Consensus recommendations for the diagnosis and clinical. Rosaidorfman disease originating from nasal septal mucosa.
Intracranial involvement is relatively rare and isolated intracranial rdd is very scarce. Histopathologically, it is characterized by histiocytic cell proliferation. Rosai dorfman disease a clinicopathological presentation. Pulmonary involvement during rdd has been poorly described. Thyroid rosaidorfman disease with infiltration of igg4. Pdf rosai dorfman disease of the orbit santosh honavar. Fnac showed mixed cellularity with histiocytic and marked lymphophagocytosis as seen in rosai dorfman disease. 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. It occurs more often in children and young adults and shows a mild male sex predilection. 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. Le traitement avait consiste en une chimiotherapie asso ciant en j1.
Rosai dorman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, was originally described by destombes in 1965. Here we describe a case of extranodal rosai dorfman disease involving the. 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. Rosai dorfman disease rdd is an uncommon benign histiocytic disorder. Rosai dorfman disease aka sinus histiocytosis with massive lymphadenopathy is a benign histiocytic proliferation that was first described in lymph nodes. A new presentation of isolated cutaneous rosaidorfman. Computerised tomography ct scans of the neck and thorax showed a large thyroid mass. Other lymph node groups may also be involved and, in some cases, abnormal. This disease is defined by histological and cytological characteristics, including emperipolesis and positive s100 and cd68 markers.
Rosai dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare, idiopathic, nonneoplastic histiocytic proliferative disorder 14. It is a rare histiocytic disorder worldwide, less than reported cases. Rosaidorfman disease rdd is a rare histiocytic disorder initially described as a separate entity in 1969 by rosai and dorfman under the term sinus histiocytosis with massive lymphadenopathy shml in report of four cases that were originally diagnosed as malignant reticuloendotheliosis. Isolated intracranial rosaidorfman disease mimicking a. Rosaidorfman sinusal histiocytosis is a benign histiocytic disorder presenting as large. Rosai dorfman disease sinus histiocytosis with massive lymphadenopathy was first described in 1969 as a benign proliferative lesion with systemic symptoms and lymphadenopathy. Rosai dorfman disease, also called sinus histiocytosis with massive lymphadenopathy, is a nonlangerhans histiocytic disease. Destombes rosai dorfman disease drd is an inflammatory nonlangerhansian histiocytosis with adenomegaly. Mutually exclusive recurrent kras and map2k1 mutations in. Rosaidorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign histioproliferative disorder of unknown etiology. Rosai dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy shml, was first described systematically by rosai and dorfman in 1969. Rosai dorfman disease a clinicopathological presentation s mehrotra, s ather, p gupta, b mehrotra abstract rosai dorfman disease rdd or sinus histiocytosis with massive lymphadenopathy shml is a rare disorder that typically manifests as lymphadenopathy and systemic symptoms.
The destombesrosaidorfman disease or sinus histiocytosis. Rosai dorfman disease generally has a longterm clinical course characterized by exacerbations and remissions and appears to undergo complete remission in most patients. However, chronic disease can occur with patterns of stability, slow progression, exacerbation, or spontaneous remission. 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. The full text of this article is available in pdf format. Rosai dorfman disease is a rare disorder of histiocytes, which present as massive cervical lymphadenopathy. Rosai dorfman disease rdd, sinus histocytosis with massive lymphadenopathy is an idiopathic histocytic proliferation affecting the lymph nodes in its classic form. 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. 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 rdd is a rare idiopathic his to proliferative disorder that usually presents with systemic symptoms and cervical lymphadenopathy. 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. We describe a case of a 59yearold woman who presented with heart failure symptoms from a large pericardial mass causing constrictive pericarditis.
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