Rosai dorfman disease is a benign, selflimited nonlangerhans cell histiocytosis of unknown etiology. For over 60 years, residents and practicing pathologists have turned to rosai and ackermans surgical pathology for definitive guidance on every aspect of the field, delivered in a readable, easytodigest, and engaging manner. Rosai and ackermans surgical pathology 2 volume set. Rosai dorfman disease commonly involves cervical lymph nodes. However, more than 87% of patients present with enlargement of the lymph nodes in the neck. Intracranial location is rare in children often mimicking meningiomas. It usually occurs in the first decade of life and manifest as massive enlargement of cervical lymph nodes. Consensus recommendations for the diagnosis and clinical. There was a marked female predominance 90% in our series.
Top 10 facts about rosai dorfman disease pathology. Initial mr imaging showed subtle mild change in the left frontal region. It is a rare and idiopathic histoproliferation disorder characterized by painless lymphadenopathy. Rosaidorfman disease, otherwise known as sinus histiocytosis with. Rosaidorfman disease is an idiopathic benign lymphoproliferative disorder that can, on rare occasions, cause intracranial or intraspinal lesions with nonspecific features on conventional imaging. We describe two distinctly rare cases of isolated intradural rdd of the spine without cervical lymphadenopathy. This is usually on both sides and is painless but often get very. Rosaidorfman disease sinus histiocyosis with massive lymphadenopathy is an idiopathic benign disorder characterized by an abnormal proliferation of histiocytes.
May 23, 2019 this case report discusses the presentation of rosaidorfman disease in a 4 year old female patient, who presented with bilateral level v lymphadenopathy of the cervical neck. Mediastinal rosaidorfman disease with widespread lesions. Immunophenotypic characterization of the cell infiltrate in five cases of sinus histiocytosis with massive lymphadenopathy rosai dorfman disease. Cutaneous rosaidorfman disease crdd is a rare proliferative disorder of histiocytes with unknown etiology, broadly different from systemic rosaidorfman disease. Rosai and ronald dorfman, with a prevalence of around 1. Rosaidorfman disease with primary cutaneous manifestations a. Rosaidorfman disease of the gastrointestinal tract. Small foci of rddlike histopathology ie, histiocytes with emperipolesis are. Rosai dorfman disease is an idiopathic benign proliferative disorder of histiocytes. Dorfman disease in the breast, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. View the article pdf and any associated supplements and figures for a period of. Rosaidorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a non. Histopathological workup of the tumor showed widespread granulomatous tissue with lymphocyte follicles. Rosaidorfman disease is an idiopathic benign proliferative disorder of histiocytes.
Rosai dorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a benign multiorgan nonlangerhans cell histiocytic proliferation that was first described in 1969 by rosai and dorfman. Fnac showed mixed cellularity with histiocytic and marked lymphophagocytosis as seen in rosai dorfman disease. 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. Rosaidorfman disease rdd is a rare, acquired disease of unknown etiology that affects primarily children and young adults. Purely cutaneous rosaidorfman disease with immunohistochemistry. Clinical picture of girl showing left nasal mass that was diagnosed with rosaidorfman disease comparison of clinical and histopathological findings showed that of the 84 patients with clinically nonneoplastic. The diagnosis of rosai dorfman rd is made following a biopsy of the affected tissue. The characteristic clinical features of this rare but wellestablished disorder were first described by rosai and dorfman in 1969 as lymphadenopathy, fever and leucocytosis.
Cutaneous rosai dorfman disease typically occurs in older females and presents in various forms, ranging from single papules to multiple nodules and plaques. Extranodal rosaidorfman disease presenting as a cardiac mass in an adult. This rare condition commonly causes massive cervical lymphadenopathy. Rosai dorfman disease, abbreviated rdd, is a rare lymph node pathology. Sinus histiocytosis rosaidorfman disease with massive lymphadenopathy is a rare nonneoplastic and nonlangerhans cell proliferation disorder of the histiocytes. Rosai dorfman shows infiltration of the dermis or subcutaneous tissue with associated sclerosis figures 1, 2. It is a rare, benign, selflimiting disease of phagocytic histiocytes affecting a young age group presenting with massive painless cervical lymphadenopathy. Rosai dorfman disease is an idiopathic benign lymphoproliferative disorder that can, on rare occasions, cause intracranial or intraspinal lesions with nonspecific features on conventional imaging. 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.
The presence of preserved inflammatory cells surrounded by a clear halo may be noticed inside the cytoplasm of these histiocytes, this phenomenon being referred to as emperipolesis. Rosai dorfman disease rdd is a rare, nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features. 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 disease rdd is a rare, nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features. 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. A young bangladeshi male complained of bilateral complete blindness with left sided deafness for about three years. Langerhans cell histiocytosis shares some clinical and immunohistochemical features such as s100 expression with sinus histiocytosis with massive lymphadenopathy rosai dorfman disease. 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. Histopathological examination demonstrated lymphophagocytosis. Extranodal sites are involved in up to 43% of the cases, with 23% of cases having only extranodal involvement. It is a rare, benign, selflimiting disease of phagocytic histiocytes affecting a young age group presenting with massive painless cervical. Jun 30, 2017 rosaidorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare nodal andor extranodal histiocytic disorder characterized by the accumulation of abnormal.
Rosaidorfman disease is a benign, selflimited nonlangerhans cell histiocytosis of unknown etiology. Similarly, nras and kras mutations are present in 12. Pleomorphic cutaneous rosaidorfman disease martinez. Rosaidorfman disease rdd is also known as sinus histiocytosis with massive lymphadenopathy shml. Rosai dorfman disease, also called sinus histiocytosis with massive lymphadenopathy, is a nonlangerhans histiocytic disease. This case is an unusual and rare clinical presentation of cervical lymphadenopathy and nasal mass diagnosed as rdd with cytohistopathological correlation. Paulli m, rosso r, kindl s, boveri e, marocolo d, chioda c, agostini c, magrini u, facchetti f. Rosaidorfman disease rdd was diagnosed and no evidence of a. In our report, a 18ffluorodeoxyglucose positron emission tomography scan of a mediastinal mass infiltrating the lungs and of widespread lesions suggested the presence. First described by rosai and dorfman in 1969, it is also known as sinus histiocytosis with massive lymphadenopathy. Via a data mining engine, we evaluated cases of extranodal rdd in 10 patients treated at our institution from 2000 to 2014.
Crdd is oftentimes misdiagnosed because it has variable clinical manifestations, particularly in the absence of lymphadenopathy. Rosaidorfmandestombes disease rdd is a rare nonlangerhans cell. A small piece of the tissue is obtained so that it can be viewed under a microscope by a pathologist. Extranodal location with or without lymphadenopathy occurs in about 40 % of the cases. Rosai dorfman disease rdd, originally known as sinus histiocytosis with massive lymphadenopathy, is a nonlangerhans cell histiocytosis that was first described in 1965 by destombes and subsequently recognized as a distinct entity by rosai and dorfman in 1969. This case report discusses the presentation of rosai dorfman disease in a 4 year old female patient, who presented with bilateral level v lymphadenopathy of the cervical neck. Rosaidorfman disease introduction 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. Cutaneous rosai dorfman disease crdd is a rare proliferative disorder of histiocytes with unknown etiology, broadly different from systemic rosai dorfman disease.
Masters degree in tropical pathology awarded by the federal university of. Primary rosaidorfman disease sinus histiocytosis with. A case report cutaneous rosaidorfman disease crdd is an uncommon benign histiocytosis of unknown etiology. Histopathology always gives a confirmatory diagnosis. This case report briefly outlines the findings for rosaidorfman disease using the advanced imaging techniques of dti, swi and perfusion mri, all of which can substantiate the findings of routine mri sequences.
Rosai dorfman disease sinus histiocytosis with massive lymphadenopathy was first described in 1969 as a benign proliferative lesion with systemic symptoms and lymphadenopathy. Rosai dorman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, was originally described by destombes in 1965. It is defined by a characteristic histopathology with sinus histiocytosis and haemophagocytosis known as emperipolesis. Tumor biology, clinical features, pathology, and treatment. Rosaidorfman disease presenting in the gastrointestinal tract. Rosaidorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a benign proliferation of histiocytes. We present the largest series of crdd, describing the clinical manifestation, histopathology, immunohistochemistry, and followup course of. In the twovolume 11th edition, a dynamic new author team ensures that this classic text retains its signature anecdotal style, while revising the content to bring you. Cutaneous rosaidorfman disease a case report 689 an bras dermatol. Rosai dorfman disease sinus histiocytosis with massive lymphadenopathy is an uncommon clinicopathological entity characterized by proliferation of histiocytes with distinctive cytological features and emperipolesis.
Soft tissue rosai dorfman disease with unusual histopathologic features. Emperipolesis, purely cutaneous, rosaidorfman disease. Langerhans cell histiocytosis archives of pathology. A 64yrold female was seen with left ankle pain and swelling for 68 months. Sheets of large foamy histiocytes without nuclear grooves, mixed with lymphocytes and plasma cells. Sinus histiocytosis with massive lymphadenopathy shml, also designated as rosaidorfman disease rdd, is a rare benign reactive lymphoproliferative disorder. Isolated intracranial rosaidorfman disease mimicking a. Article information, pdf download for rosaidorfman disease. Rosaidorfman disease rdd is uncommon in daily practice, but needs to be ruled out in rheumatologic conditions to elucidate a wide differential diagnosis. We present the largest series of crdd, describing the clinical manifestation, histopathology, immunohistochemistry, and followup course of 25 cases in china. The histopathology demonstrated numerous histiocytes, with characteristic emperipolesis.
Cutaneous rosaidorfman disease crdd is a rare form of rosaidorfman 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. Media in category histopathology of rosaidorfman disease the following 14 files are in this category, out of 14 total. To the best of our knowledge, such findings have never been previously described in the literature. Media in category histopathology of rosai dorfman disease the following 14 files are in this category, out of 14 total. We report the case of a 68yearold woman with isolated rosai dorfman disease of the frontal dura.
This case report discusses the presentation of rosaidorfman disease in a 4 year old female patient, who presented with bilateral level v lymphadenopathy of the cervical neck. Rosaidorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a benign multiorgan nonlangerhans cell histiocytic proliferation that was first described in 1969 by rosai and dorfman. It is characterized by a proliferation of distinctive histiocytes in the lymph nodes andor extranodal sites. In the twovolume 11th edition, a dynamic new author team ensures that this classic text retains its signature. Jul 22, 20 rosai dorfman disease was first described by rosai and dorfman in 1969. Rosai dorfman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, is a benign proliferation of histiocytes. Extranodal and central nervous system cns involvement is unusual. Cytomorphology and histology correlation of rosaidorfman disease. Rosaidorman disease rdd, also known as sinus histiocytosis with massive lymphadenopathy, was originally described by destombes in 1965. Intracranial involvement is relatively rare and isolated intracranial rdd is very scarce. Histopathological examination demonstrated lymphophagocytosis emperipolesis consistent with a diagnosis of rosaidorfman disease. Rosaidorfman disease rdd is a rare, benign pseudolymphomatous condition, predominantly involving lymph nodes.
Extranodal sites are involved in up to 43% of the cases, with 23% of. Nord gratefully acknowledges juan rosai, md, pathology cancer center clinics, italian diagnostic center cdi, milan, italy for assistance in the preparation of this report. Rosaidorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare nodal andor extranodal histiocytic disorder characterized by the accumulation of abnormal. However, rosaidorfman disease does not show expression of cd1a or langerin, and large histiocytic cells are present. Case report of rosaidorfman disease diagnostic histopathology. Beside its typical presentation, soft tissue masses can be easily seen in our rheumatology clinics. Sonographic manifestation of thyroids rosaidorfman disease. Immunostaining showed the tumor to be positive for s100, cd68 and vimentin. Pdf sinus histiocytosis with massive lymphadenopathy, also known as rosaidorfman disease, is a rare histiocytic proliferative disorder of. Parvovirus b19 detected in rosaidorfman disease in nodal. Rosai dorfman disease rdd is a rare idiopathic his to proliferative disorder that usually presents with systemic symptoms and cervical lymphadenopathy. Rosaidorfman disease nord national organization for rare.
Rosaidorfman disease symptoms histiocytosis association. Mutually exclusive recurrent kras and map2k1 mutations in. We present the largest series of crdd, describing the clinical manifestation, histopathology, immunohistochemistry, and followup cours. Pdf rosaidorfman disease or sinus histiocytosis with massive. He is the principal author and editor of a major textbook in that field, and he has characterized novel medical conditions such as rosai dorfman disease and the desmoplastic small round cell tumor. Rosaidorfman disease genetic and rare diseases information. 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. 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. It occurs more often in children and young adults and shows a mild male sex predilection. 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.
Rosai dorfman disease sinus histiocytosis with massive lymphadenopathy rarely affects the intracranial region without involvement of other sites. Case report sonographic manifestation of thyroids rosaidorfman disease wenwuling1,parajulyshyamsundar1,yanluo1,yongjiang2,dimingcai1 1department of sonography, and 2 department of pathology, west china medical school of sichuan university, chengdu 610041, sichuan, china received june, 2011. Nsinus histiocytosis with massive lymphadenopathy shml, also called rosaidorfman disease, is a rare entity. Rosai dorfman syndrome with extranodal manifestation. Rosaidorfman disease masquerading as chronic ankle. It was first described in 1969 by rosai and dorfman. To describe the radiologic and clinicopathologic features of extranodal rosaidorfman disease rdd in our patient population. Rosaidorfman disease rdd is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Rosaidorfman disease sinus histiocytosis with massive lymphadenopathy was first described in 1969 as a benign proliferative lesion with systemic symptoms and lymphadenopathy. Since this is a skin disease, not a lifethreatening condition, and. Isolated intradural rosaidorfman disease of the spine.
Rosaidorfman disease iranian journal of blood and cancer. Its most common clinical feature is extraordinary massive painless cervical lymphadenopathy. Mar 30, 2018 rosaidorfman disease rdd is uncommon in daily practice, but needs to be ruled out in rheumatologic conditions to elucidate a wide differential diagnosis. Intracranial rdd without any nodal involvement is extremely rare. Arch pathol lab medvol 127, february 2003 rosaidorfman disease in the gi tractanders et al e75 a, crosssection of large bowel wall revealing a submucosal mass that in.
Rosaidorfman disease rdd sinus histiocytes with massive lymphadenopathy rarely affects the intracranial region without involvement of other sites. Rosaidorfman disease, abbreviated rdd, is a rare lymph node pathology. It is also known as sinus histiocytosis with massive lymphadenopathy, abbreviated shml. Rosaidorfman disease commonly involves cervical lymph nodes. Rosaidorfman disease rdd is a benign histiocytic proliferative disorder of unknown etiology. Feb 28, 2014 rosai dorfman disease rdd is a rare, benign disease that presents with massive painless cervical lymphadenopathy. The use of various immunosuppressive therapies, including antitnf therapy with infliximab resulted in no response. Langerhans cell histiocytosis shares some clinical and immunohistochemical features such as s100 expression with sinus histiocytosis with massive lymphadenopathy rosaidorfman disease. Rosaidorfman disease rdd is a rare idiopathic his to proliferative disorder that usually presents with systemic symptoms and cervical lymphadenopathy.
Microorganisms were not identified on special stains, nor on culture. Pdf sinus histiocytosis with massive lymphadenopathy, also known as rosai dorfman disease, is a rare histiocytic proliferative disorder of. Rosaidorfman disease rdd is a rare benign disorder of the histiocytes, affecting lymph nodes in its classic form. Comparison of cytomorphology in conventional smears, liquidbased preparations and histopathology chihyi liu1, fengchuan tai2, shihhung huang3 1 division of pathology, sijhih cathay general hospital, new taipei city, taiwan. Extranodal rdd is considered the uncommon subtype and potentially impairs all tissues and intrathoracic organs. Cutaneous rosaidorfman disease a case report scielo. Aijcr original article histopathology of nasal masses. To describe the radiologic and clinicopathologic features of extranodal rosai dorfman disease rdd in our patient population. Although rare, unexpected clinically relevant findings may be. However, rosai dorfman disease does not show expression of cd1a or langerin, and large histiocytic cells are present. In histiocytes s100 is strongly expressed, whereas cd1a staining typically is negative.