This disease is defined by histological and cytological characteristics, including emperipolesis and positive s100 and cd68 markers. Lesion biopsy and characteristic histopathological. Rosaidorfman disease the american journal of the medical. Via a data mining engine, we evaluated cases of extranodal rdd in 10 patients treated at our institution from 2000 to 2014. Cutaneous rosaidorfman disease typically occurs in older females and presents in various forms, ranging from single papules to multiple nodules and plaques histology of rosaidorfman disease. Rosai dorfman disease rdd is an uncommon benign histiocytic disorder. Histological examination of the specimens from pituitary, spinal mass, and nasal sinuses showed rosai.
Rosaidorfman disease is a rare, idiopathic disorder of histiocyte proliferation. Rosai dorfman disease is a rare disorder characterized by overproduction proliferation and accumulation of a specific type of white blood cell histiocyte in the lymph nodes of the body lymphadenopathy, most often those of the neck cervical lymphadenopathy. This suggests a possible overlap or even a common cause between rosaidorfman disease and igg4related disease. What are the signs and symptoms of rosaidorfman disease of breast. Shml nomenclature reflects typical disease presentation as a massive painless bilateral cervical lymphadenopathy with fever. Rosaidorfman disease rdd is also known as sinus histiocytosis with massive lymphadenopathy shml. Other lymph node groups may also be involved and, in some cases, abnormal accumulation of histiocytes may occur in other areas of. Pathology outlines sinus histiocytosis with massive. Rosai dorfman disease is an idiopathic benign proliferative disorder of histiocytes. Rosaidorfman disease rdd is a histiocytic proliferative disorder and considered to be extremely rare in the cns. Rosaidorfman disease was only recently associated with increased igg4 cell populations 9, 10. First described by rosai and dorfman in 1969, it is also known as sinus histiocytosis with massive lymphadenopathy. Sinus histiocytosis with massive lymphadenopathy, or rosaidorfman disease, is a benign idiopathic histiocytic proliferative disorder that commonly involves the lymph nodes but secondarily may involve the skin.
Rosaidorfman disease rdd, which is also referred to as sinus histiocytosis with massive lymphadenopathy, is a rare nonlangerhans cell reactive histiocytic disorder that was initially described in 1969. Apr 11, 2017 rosai dorfman disease also known as sinus histiocytosis with massive lymphadenopathy is a rare benign proliferative disorder of histiocytes that typically involves the lymph nodes and can also involve extranodal sites. Diagnosis and management of rosaidorfman disease involving the central nervous system. Jul 22, 20 rosai dorfman disease was first described by rosai and dorfman in 1969.
In this paper, we reported a patient confirmedas isolated intracranial rdd in. Rosaidorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign histioproliferative disorder of unknown etiology. Rosaidorfman disease rdd is a rare nonneoplastic proliferative disorder of histiocytes of unknown etiology. Rosai dorfman disease rdd is a rare, nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features. Rosaidorfman disease presenting as multiple breast masses in an otherwise asymptomatic male patient.
Nevertheless, primary cutaneous manifestation of rdd crdd without systemic involvement is rare. The disease is characterized with massive, painless bilateral cervical lymphadenopathy and. Rosaidorfman disease sinus histiocytosis with massive lymphadenopathy rarely affects the intracranial region without involvement of other sites. Dorfman disease, or sinus histiocytosis with massive lymphadenopathy shml, is a rare benign disease of unknown etiology that involves the cervical lymph nodes. Cutaneous rosaidorfman disease md anderson cancer center. Rosai dorfman disease also known as sinus histiocytosis with massive lymphadenopathy is a rare benign proliferative disorder of histiocytes that typically involves the lymph nodes and can also involve extranodal sites. Consensus recommendations for the diagnosis and clinical.
Rosai dorfman disease, abbreviated rdd, is a rare lymph node pathology. Rosaidorfman disease also known as sinus histiocytosis with massive lymphadenopathy is a rare benign proliferative disorder of histiocytes that typically involves the lymph nodes and can also involve extranodal sites. The signs and symptoms of rosaidorfman disease of breast can vary from one individual to another. Feb 28, 2014 rosai dorfman disease rdd is also known as sinus histiocytosis with massive lymphadenopathy shml. Rosaidorfmandestombes disease is a rare nonmalignant histiocytic condition. Isolated extranodal involvement is relatively uncommon. Rosai dorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign histioproliferative disorder of unknown etiology. We report the case of a 68yearold woman with isolated rosai dorfman disease of the frontal dura. Rosaidorfman disease, also called sinus histiocytosis with massive lymphadenopathy, is a nonlangerhans histiocytic disease. To describe the radiologic and clinicopathologic features of extranodal rosai dorfman disease rdd in our patient population. Rosaidorfman disease is a rare subtype of nonlangerhans cell histiocytosis. Rosaidorfman disease symptoms histiocytosis association. Intracranial involvement is relatively rare and isolated intracranial rdd is very scarce. One could even speculate that rosaidorfman disease might be a reactive pattern to igg4related disease.
Rosaidorfman disease was first described by rosai and dorfman in 1969. Rosai dorfman disease, or sinus histiocytosis with massive lymphadenopathy shml, is a rare histiocytosis of unknown etiology that most commonly involves the cervical lymph nodes. It was first described in 1969 by rosai and dorfman. Cureus rosaidorfman disease and unusual local invasive. Extranodal intracranial rosaidorfmandestombes disease in. Nsinus histiocytosis with massive lymphadenopathy shml, also called rosaidorfman disease, is a rare entity. Only small percent of patients present with intracranial extranodal form of disease. Lymphadenopathy among 358 cases of rosaidorfman disease that rosai collected in a disease registry for which the location of lymphadenopathy was specified, 87. Rosaidorfman disease an overview sciencedirect topics. Although the disease typically presents clinically with massive bilateral lymphadenopathy due. Rosai dorfman disease confined to the breast is extremely rare, but important to recognize as it can mimic malignancy. However, more than 87% of patients present with enlargement of the lymph nodes in the neck. A rare case of rosaidorfman disease without lymphadenopathy.
Some are known to have mild symptoms, while others may have severe symptoms. Rosai dorfman disease rdd is a rare, benign condition that causes proliferation of histiocytes, a type of white blood cells, within the lymph nodes and other organs of the body. Rosaidorman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, was originally described by destombes in 1965. Confirmed efficacy of lenalidomide and dexamethasone in. Rosaidorfman disease confined to the breast is extremely rare, but important to recognize as it can mimic malignancy. Rosai dorfman disease is most common among patients mar 12, 2020 rosai dorfman destombes rdd disease also known as sinus histiocytosis with massive lymphadenopathy is a rare disease characterized by disorder of histiocytes within lymph nodes or other affected tissues. We describe a case of a 59yearold woman who presented with heart failure symptoms from a large pericardial mass causing constrictive pericarditis. Rdd, which now belongs to the r group of the 2016 revised histiocytosis classification, is a widely heterogeneous entity with a range of clinical phenotypes occurring in isolation or. We report the case of a 68yearold woman with isolated rosaidorfman disease of the frontal dura. Rosai dorfman disease rdd is a rare idiopathic his to proliferative disorder that usually presents with systemic symptoms and cervical lymphadenopathy. Extranodal involvement occurs in 43% and most commonly involves the head and neck, skin, and bones. Rosaidorfman disease, originally described as sinus histiocytosis with massive lymphadenopathy, is an idiopathic histiocytic proliferative disorder that may present either as lymphadenopathy or as a mass lesion.
Histologic findings include emperiopolesis of lymphocytes and s. We present the case of a 63yearold woman who presented. Rosai dorfman disease rdd is a rare selflimited histiocytic proliferative disorder of unknown etiology. It is also known as sinus histiocytosis with massive lymphadenopathy, abbreviated shml. Rosai dorfman disease sinus histiocytosis with massive lymphadenopathy is a rare benign idiopathic proliferative disease of phagocytic histiocytes. This is usually on both sides and is painless but often get very. Although the disease typically presents clinically with massive bilateral 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. Rosaidorfman 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. Extranodal sites are involved in up to 43% of the cases, with 23% of. Rosaidorfmandestombes 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 or sinus histiocytosis is a rare, idiopathic, non neoplastic histiocytic proliferation of unknown etiology typically presenting with massive cervical lymphadenopathy with or without systemic involvement. The diagnosis of rosaidorfman rd is made following a biopsy of the affected tissue. It commonly occurs during the first or second decade of life and is usually characterized by massive painless lymphadenopathy and other systemic manifestations, including fever, night sweats, and weight loss.
Rosai dorfman disease was first described by rosai and dorfman in 1969. Also, in some individuals, the symptoms are known to appear and disappear over time. Rosaidorfman disease most often manifests with bilaterally enlarged, painless cervical lymphadenopathy, fever, and leukocytosis. We present a 30yearold man with a large unresectable cutaneous facial mass refractory to corticosteroids and rituximab. Pdf rosaidorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disorder that typically manifests as. Rosaidorfman disease of the breast pubmed central pmc. Rosai dorman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, was originally described by destombes in 1965. Rosai dorfman disease rdd, sometimes known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder that most commonly presents as painless, massive cervical lymphadenopathy in young adults. The case history is presented of a woman who was thought to have atypical carcinoma of the lung but in whom a diagnosis of rosaidorfman disease was eventually made following a range of investigations including ct scans, positron emission tomography pet and. Rosaidorfman 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 is a rare benign idiopathic proliferative disease of phagocytic histiocytes.
Rosai dorfman disease, also called sinus histiocytosis with massive lymphadenopathy, is a nonlangerhans histiocytic disease. Accompanying laboratory findings include leukocytosis. Isolated intracranial rosaidorfman disease mimicking a. Rosaidorfman disease rdd is a rare idiopathic histiocytic proliferative disorder. Fine needle aspiration diagnosis of rosaidorfman disease involving thyroid. Rosaidorfman disease, mediastinum, thymus, bronchogenic cyst introduction rosaidorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy shml, is an uncommon macrophagerelated histiocytic disorder 1, 2. Rdd is a benign disorder that presents with massive lymphadenopathy, but can have extranodal involvement. Tumor biology, clinical features, pathology, and treatment. Rosaidorfman disease rdd is a rare medical condition with bilateral painless lymphadenopathy.
Excision and corticosteroid therapy provide a favorable. Pdf rosai dorfman disease of the orbit santosh honavar. Approximately 80% of patients present with painless massive cervical lymphadenopathy. Rosaidorfman disease rdd is a rare, benign histiocytic proliferative disorder of unknown etiology. If the cells in the tissue have certain specific characteristics, the diagnosis of rd can be made. Pdf sinus histiocytosis with massive lymphadenopathy, also known as rosaidorfman disease, is a rare histiocytic proliferative disorder of. The diagnosis of rosai dorfman rd is made following a biopsy of the affected tissue. Typical presentation is with massive cervical lymphadenopathy alongside features of a raised. Rosai dorfman disease of the orbit, although rare, should be considered in young individuals with chronic proptosis with rubbery masses. It is a benign disease which is characterized by overproduction and accumulation of a specific type of white blood cell histiocyte in the lymph nodes of the body, most often those of the neck cervical. Rosai dorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a benign proliferation of histiocytes.
A 40yearold africanamerican man presented with a firm, nontender, progressive chest and neck mass appeared three months ago. The histiocytosis of rosaidorfman disease can occur in lymph nodes, causing lymphadenopathy, or can occur outside lymph nodes in extranodal disease. We present the case of a young man diagnosed with a very unique presentation of rosaidorfman disease. Initial mr imaging showed subtle mild change in the left frontal region.
Rosaidorfman disease is an idiopathic benign proliferative disorder of histiocytes. Dorfman disease, a rare entity characterized by histiocytic proliferation, emperipolesis lymphophagocytosis and lymphadenopathy. Rosaidorfman disease is a rare histiocytic disorder of children and adults which presents with massive lymphadenopathy and infiltration of the nasopharynx, respiratory pathways, endocrine glands, bones, and skin. Rosaidorfman disease rdd is a rare, nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features.
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. Rosaidorfmandestombes disease rdd is a rare nonlangerhans cell histiocytosis characterized by accumulation of activated histiocytes within affected. Rosai dorfman disease rdd is a rare medical condition with bilateral painless lymphadenopathy. Rosaidorfman disease presenting with isolated bilateral. Rosaidorfman disease merck manuals professional edition. Rosaidorfman disease of the breast bjrcase reports. Rosaidorfman disease genetic and rare diseases information. Rosai dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy or rosai dorfman destombes disease, is a rare benign idiopathic proliferative disease that involves phagocytic histiocytes. She was treated with corticosteroids and improved clinically. With the last major report published in 1990, there is a paucity of contemporary data on this disease. Rosai dorfman disease is a rare disorder characterized by accumulation of histiocytes and massive lymphadenopathy, particularly in the neck and head.
A small piece of the tissue is obtained so that it can be viewed under a microscope by a pathologist. It is a rare, benign, selflimiting disease of phagocytic histiocytes affecting a young age group presenting with massive painless cervical lymphadenopathy. Pdf rosaidorfman disease of the orbit researchgate. Rosai dorfman disease sinus histiocytosis with massive lymphadenopathy rarely affects the intracranial region without involvement of other sites. However, purely cutaneous disease without lymphadenopathy or internal organ involvement rarely may occur. Rosaidorfmandestombes disease rdd is a rare nonlangerhans cell histiocytosis characterized by accumulation of activated. Rosaidorfman disease rdd, sometimes known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder that most commonly presents as painless, massive cervical lymphadenopathy in young adults. Cutaneous rosai dorfman disease typically occurs in older females and presents in various forms, ranging from single papules to multiple nodules and plaques.
Also called rosaidorfman disease clinical features associated with fever, leukocytosis, anemia, elevated sedimentation rate, polyclonal hypergammaglobulinemia. Rdd was initially described as sinus histiocytosis with massive lymphadenopathy in 1969. The disease usually manifests during the first two decades of life with extraordinary massive painless cervical lymphadenopathy as its most common clinical feature. Dec 05, 2015 rosaidorfman disease rdd, juvenile xanthogranuloma jxg, and erdheimchester disease ecd are nonlangerhans cell nonlch disorders arising from either a dendritic or a macrophage cell. 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. Rosai dorfman disease confined to the breast is extremely rare, but important. There was a marked female predominance 90% in our series. Rosaidorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a benign proliferation of histiocytes. Rosaidorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy shml, is a disease of histiocytic proliferation with no known pathogenesis. Rosaidorfman disease nord national organization for rare. To describe the radiologic and clinicopathologic features of extranodal rosaidorfman disease rdd in our patient population. Cutaneous rdd is extremely rare and the etiology is unknown, though a. Extranodal forms of disease are even more uncommon.
Rosai dorfman destombes disease rdd is a rare nonlangerhans cell histiocytosis characterized by accumulation of activated histiocytes within affected tissues. Cutaneous disease is common and typically treated with surgical resection, but the optimal medical therapy for inoperable disease has not been determined. Clinical and histopathologic spectrum of cutaneous rosaidorfman disease in taiwan. Case report isolated thymic rosaidorfman disease with. This study reports a clinical case of cutaneous rosaidorfman disease, its manifestation, and its evolution in 10 years, thus contributing to the limited. Griffiths sj, tang w, parameswaran r, kelsey a, west cg 2004 isolated intracranial rosaidorfman disease mimicking meningioma in a child. Rosaidorfman disease also known as sinus histiocytosis with massive lymphadenopathy is a nonlangerhans cell histiocytosis. Extranodal rosaidorfman disease associated with increased. Pathologic findings including immunohistochemistry suggested the diagnosis. Rosai dorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy shml, is a disease of histiocytic proliferation with no known pathogenesis. Overlapping immunoglobulin g4related disease and rosai. Rosaidorfman disease rdd or sinus histiocytosis is a rare histiocytic disorder characterized by massive lymphadenopathy and with extranodal involvement in 25% to 43% of cases. Extranodal disease can occur in up to 40% of patients but primary involvement of bone is rare.
Rosaidorfman disease, also known as sinus histiocytosis with massive lymphadenopathy or sometimes as destombesrosaidorfman disease, is a rare disorder of unknown cause that is characterized by abundant histiocytes in the lymph nodes or other locations throughout the body. Rosaidorfman disease rdd is a rare histiocytic disorder characterized by a benign proliferation of s100positive histiocytic cells within the sinus of the lymph nodes and the lymphatic vessels of internal organs. Rosaidorfman disease, or sinus histiocytosis with massive lymphadenopathy shml, is a rare histiocytosis of unknown etiology that most commonly involves the cervical lymph nodes. Extranodal involvement occurs in 3040% of cases, often involving skin.
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