Cytomorphology and Histology Correlation of Rosai-Dorfman Disease: A 15-Year Study from a Tertiary Referral Centre in South India

2016 ◽  
Vol 61 (1) ◽  
pp. 55-61 ◽  
Author(s):  
Abid Hussain ◽  
Ashwani Tandon ◽  
Aruna K. Prayaga ◽  
Tara Roshni Paul ◽  
Anukonda M.V.R. Narendra
Keyword(s):  
Hodgkin Lymphoma ◽  
Plasma Cells ◽  
Cervical Lymphadenopathy ◽  
Cost Effective ◽  
Needle Aspiration ◽  
Unknown Etiology ◽  
Tertiary Referral Centre ◽  
Reliable Technique ◽  
Male Predominance ◽  
Rosai Dorfman Disease

Objectives: Rosai-Dorfman disease (RDD) is an uncommon, benign histiocytic disorder of unknown etiology, typically presenting in young adulthood. We highlight the cytomorphology of RDD and correlate it with the histopathology. Study Design: All cases diagnosed as RDD on fine-needle aspiration cytology between January 2001 and June 2015 were included. Clinical details were obtained from medical records. The cytology smears were reviewed along with the histopathology and immunohistochemistry, wherever available. Results: The study included 10 cases ranging in age from 11 to 68 years (median 29). There was a male predominance with a male:female ratio of 1.5:1. The patients commonly presented with bilateral cervical lymphadenopathy. Extranodal involvement was seen in 2 cases in the nose and mandible, respectively. Of these 10 cases, 8 were later biopsied. The cytological features included numerous crescentic histiocytes, emperipolesis, reactive lymphocytes and plasma cells. A histological diagnosis of RDD was made in 7 out of 8 cases, and 1 was diagnosed as Hodgkin lymphoma. Conclusion: FNA represents an efficient, minimally invasive, cost-effective and reliable technique for the diagnosis of RDD and may obviate the need for further biopsy. However, the disease has close differential diagnoses, including Langerhans cell histiocytosis, granulomatous lesions, and Hodgkin lymphoma. Hence, it must be remembered that there can be pitfalls when the diagnosis is made by cytology alone.

Extra-nodal Rosai-Dorfman Disease Confined to Breast

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S41-S42
Author(s):  
E Ozluk ◽  
R Shackelford
Keyword(s):  
Plasma Cells ◽  
African American Woman ◽  
American Woman ◽  
Lymphocytic Infiltration ◽  
Needle Aspiration ◽  
Left Breast ◽  
Unknown Etiology ◽  
Diagnostic Challenge ◽  
Ductal Cells ◽  
Rosai Dorfman Disease

Abstract Introduction/Objective First described by Rosai and Dorfman in 1969, Rosai-Dorfman Disease (RDD) is an uncommon, idiopathic, reactive lymph node process with an unknown etiology. It may involve extra-nodal organs including the skin, bone, soft tissue, and eyes. However, breast involvement is rare and RDD confined to breast without nodal involvement is extremely uncommon. Methods Here we present a case of RDD confined to breast. The patient was a 51-year-old African American woman who was found to have an irregular, solid left breast mass on routine mammogram, which had speculated irregular margins and measured of 37.0 x 32.0 x 32.0 mm. She did not have any symptoms, nor any palpable lymph nodes. The clinician stated that the lesion was highly suspicious for a breast malignancy and requested a fine needle aspiration (FNA) of the mass, followed by total excision. Results An FNA was interpreted as atypical histiocytic cells in a mixed lymphoid background. Histopathologic examination revealed an ill-defined mass with sheets of histiocytes, plasma cells, and a mixed lymphocytic population, with occasional germinal centers. Some of the histiocytes were spindle shaped and associated with storiform collagen deposition. The histiocytes had single and multiple nuclei and exhibited occasional emperipolesis. Immunohistochemical staining with S100 diffusely highlighted the histiocytes, whereas CD1a was negative. CD3 and CD20 immunostains were positive for mixed-type lymphocytic infiltration. Cytokeratin staining was performed and reassuringly stained only the benign ductal cells. A diagnosis of RDD of the breast was made, based on these histopathologic findings. Conclusion We report an example of an extra-nodal RDD involving the mammary gland that was initially suspected to be breast carcinoma. RDD may still be a diagnostic challenge, especially in a patient with suspected carcinoma. It is the pathologist’s role to lead the clinician to the proper diagnosis and render a correct histopathologic diagnosis.

scholarly journals A case report of Rosai Dorfman disease

2018 ◽  
Vol 4 (3) ◽  
pp. 868
Author(s):  
Jyotika Waghray ◽  
Pradyut Waghray
Keyword(s):  
Lymph Node ◽  
Plasma Cells ◽  
Final Diagnosis ◽  
Needle Aspiration ◽  
Lymph Node Involvement ◽  
Unknown Etiology ◽  
Low Grade ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Node Involvement

<p>Rosai-Dorfman’s disease also known as sinus histiocytosis with massive lymphadenopathy (SHML) is characterized by distorted lymph node architecture with marked dilation of lymphatic sinuses occupied by numerous lymphocytes, as well as histiocytes with vesicular nucleus and abundant clear cytoplasm with phagocytized lymphocytes or plasma cells, also known as ‘emperipolesis’. This disease of unknown etiology progresses with a benign prognosis strictly and only when an early diagnosis and treatment is made. A late diagnosis and a generalized lymph node involvement contribute to a poor prognosis. We reported a case of a 29-year-old Indian female with a 4-month history of painful unilateral cervical mass and low-grade fever with the final diagnosis of Rosai-Dorfman disease. The final diagnosis was made by fine needle aspiration (FNA) biopsy of the cervical lymph node. In conclusion, FNA biopsy can be enough to make the diagnosis in most cases due to the distinct cytological features of SHML, thereby avoiding more invasive approaches that potentially are unnecessary.</p>

Rosai-Dorfman Disease of the Parotid Gland

2001 ◽  
Vol 125 (10) ◽  
pp. 1348-1350
Author(s):  
Ridas Juskevicius ◽  
James L. Finley
Keyword(s):  
Salivary Gland ◽  
Lupus Erythematosus ◽  
Plasma Cells ◽  
Correct Diagnosis ◽  
Unknown Origin ◽  
Needle Aspiration ◽  
Major Salivary Gland ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Mixed Inflammatory Infiltrate

Abstract Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown origin and a distinct clinicopathologic entity also known as sinus histiocytosis with massive lymphadenopathy. The disease can involve extranodal tissues and rarely can present as salivary gland enlargement without significant lymphadenopathy. Involvement of the extranodal head and neck sites appears to be more common in patients with immunologic abnormalities. The disease was first described in 4 patients in 1969, and with later descriptions of more patients, the disease was established as a well-defined clinicopathologic entity. The characteristic pathologic feature of this disease is proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrate, consisting of moderately abundant plasma cells and lymphocytes. Fine-needle aspiration biopsy can be helpful in establishing the correct diagnosis, since surgical treatment is not necessary other than obtaining tissue for definitive diagnosis. We describe cytologic, histopathologic, and immunohistochemical features of a case of Rosai-Dorfman disease that involved a major salivary gland without significant lymphadenopathy in a 48-year-old patient with systemic lupus erythematosus. We also briefly discuss possible causes and pathogenesis and review the literature.

scholarly journals Disseminated Histoplasmosis: A Fatal Opportunistic Infection in Disguise

2016 ◽  
Vol 8 (02) ◽  
pp. 129-131 ◽  
Author(s):  
Ruchita Tyagi ◽  
Arshdeep Kaur ◽  
Pavneet Kaur Selhi ◽  
Harpreet Kaur Puri ◽  
Neena Sood
Keyword(s):  
Unusual Case ◽  
Histoplasma Capsulatum ◽  
Clinical Presentation ◽  
Cervical Lymphadenopathy ◽  
Cost Effective ◽  
Needle Aspiration ◽  
Hiv Positive ◽  
African Origin ◽  
Disseminated Histoplasmosis ◽  
Variable Nature

ABSTRACTHistoplasma capsulatum is no longer confined to certain geographic areas and should always be considered in the differential diagnosis of lymphadenopathy and organomegaly in HIV-positive patients. We present an unusual case of a 20-year-old immunocompromised male of African origin presenting with fever, jaundice, hepatosplenomegaly, and retroperitoneal and cervical lymphadenopathy. Fine needle aspiration (FNA) smears from the cervical lymph node revealed numerous yeast forms of histoplasma in macrophages. The patient succumbed to the fulminant infection. Postmortem liver biopsy also revealed infiltration by histoplasma, confirming the diagnosis of disseminated histoplasmosis. This case highlights the variable nature of the clinical presentation of disseminated histoplasmosis which can mimic tuberculosis, leishmaniasis, or lymphoma. FNA cytology is a rapid, cost-effective, and reliable diagnostic tool for early detection and prompt management of histoplasmosis.

scholarly journals A unique combination of Rosai-Dorfman disease and mycosis fungoides: a case report

2018 ◽  
Vol 6 ◽  
pp. 2050313X1877219 ◽  
Author(s):  
Amanda J Shelley ◽  
Nordau Kanigsberg
Keyword(s):  
Case Report ◽  
T Cell ◽  
Mycosis Fungoides ◽  
Cell Lymphoma ◽  
Cervical Lymphadenopathy ◽  
B Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Unknown Etiology ◽  
Unique Combination ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare histiocytic condition of unknown etiology. Patients with RDD classically present with massive painless cervical lymphadenopathy. However, extra-nodal disease occurs in approximately 40% of cases, with the skin being among the most commonly involved sites. Patients with isolated extra-nodal involvement may present without adenopathy. Reports of RDD occurring in patients with Hodgkin’s lymphoma, and B-cell lymphoma have been published, but there has only been one previous report of RDD in a patient with a T-cell lymphoma. This case report documents a unique combination of RDD and mycosis fungoides (MF), a cutaneous T cell lymphoma. This report also highlights diagnostic challenges in RDD due to the rarity of the condition and its variable presentation.

scholarly journals Diagnosis of Rosai-Dorfman Disease in Elderly Female on Fine Needle Aspiration Cytology: A Case Report

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Meher Aziz ◽  
Prasenjit Sen Ray ◽  
Nazima Haider ◽  
Sumit Prakash Rathore
Keyword(s):  
Fine Needle Aspiration ◽  
Fine Needle Aspiration Cytology ◽  
Cervical Lymphadenopathy ◽  
Needle Aspiration ◽  
Aspiration Cytology ◽  
Fine Needle ◽  
Elderly Female ◽  
Rosai Dorfman Disease ◽  
Needle Aspiration Cytology ◽  
Generalized Lymphadenopathy

Rosai-Dorfman disease (RDD) is a rare benign disorder of histiocytic proliferation that usually presents with bilateral cervical lymphadenopathy in children. We describe the case of a 50-year-old lady suffering from this disease who presented with generalized lymphadenopathy and a left sided chest wall lump. Fine needle aspiration cytology (FNAC) from all the lesions showed abundant benign histiocytes with lymphophagocytosis which was compatible with the diagnosis of RDD. This case is being reported for its rarity in presentation in an elderly female with both generalized nodal as well as extranodal manifestations.

scholarly journals Rosai-Dorfman disease: an overview

2020 ◽  
Vol 73 (11) ◽  
pp. 697-705
Author(s):  
Cassandra Bruce-Brand ◽  
Johann W Schneider ◽  
Pawel Schubert
Keyword(s):  
Plasma Cells ◽  
English Literature ◽  
Significant Proportion ◽  
Cervical Lymphadenopathy ◽  
Molecular Profile ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Rosai Dorfman Disease ◽  
Neoplastic Process

ContextRosai-Dorfman disease is an uncommon histiocytic disorder most frequently presenting as bilateral cervical lymphadenopathy in children and young adults. Extranodal disease occurs in a significant proportion of patients. It has been recently classified as part of the ‘R group’ of histiocytoses by the Histiocyte Society in 2016. Cutaneous Rosai-Dorfman disease is regarded as a separate disease entity that falls into the ‘C group’ of histiocytoses according to this classification system. The pathogenesis was previously poorly understood; however, recent evidence demonstrating clonality in a subset of cases raises the possibility of a neoplastic process. A possible association with IgG4-related disease remains controversial.ObjectivesTo provide a comprehensive review of Rosai-Dorfman disease, including nodal, extranodal and cutaneous forms, with a particular emphasis on new insights into the possible clonal nature of the disease; to discuss the recently revised classification of the histiocytoses by the Histiocyte Society; and to summarise the findings from the literature regarding the controversial association with IgG4-related disease.Data sourcesThis review is based on published peer-reviewed English literature.ConclusionsClassic Rosai-Dorfman disease, which may be sporadic or familial, is considered a separate entity from cutaneous disease, which is reflected in the revised classification of histiocytoses. An increase in IgG4-positive plasma cells may be seen in Rosai-Dorfman disease. This finding in isolation is of limited significance and should be interpreted with caution. Studies investigating the molecular profile of the disease show that in at least a subset of cases the disease is a clonal process. The classification of Rosai-Dorfman disease is therefore likely to change as our understanding of the aetiopathogenesis evolves.

scholarly journals Rosai-Dorfman Disease: A Rare Cause of Cervical Lymphadenopathy

2014 ◽  
Vol 5 (3) ◽  
pp. 152-154
Author(s):  
Adharsh Anand ◽  
Kanchan Lanjewar ◽  
Vipin Ram Ekhar ◽  
Ritesh N Shelkar ◽  
Sachin Rane ◽  
...  
Keyword(s):  
Pathological Condition ◽  
Cervical Lymphadenopathy ◽  
Needle Aspiration ◽  
Benign Condition ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Life Threatening ◽  
Children And Young Adults ◽  
Needle Aspiration Cytology ◽  
Extranodal Manifestations

ABSTRACT Rosai-Dorfman disease is also known as sinus histiocytosis with massive lymphadenopathy (SHML) is a rare clinico-pathological condition. It is a benign condition which causes significant cervical lymphadenopathy in children and young adults. The clinical presentation varies from isolated nodal involvement to significant extranodal manifestations. The clinical features are usually mild, but rarely life-threatening complications can occur in some individuals depending on the site of involvement. Here, we present two cases of Rosai-Dorfman disease, both diagnosed on fine needle aspiration cytology (FNAC) and histopathology and responded well to steroids. One of the patients had extranodal site involvement in the form of bilateral nasal mass which is very rare. How to cite this article Ekhar VR, Shelkar RN, Rane S, Anand A, Lanjewar K, Jain SKT. Rosai-Dorfman Disease: A Rare Cause of Cervical Lymphadenopathy. Int J Head Neck Surg 2014;5(3): 152-154.

scholarly journals FNAC: A Simple & Cost-Effective Diagnostic Tool for Benign & Malignant Pathologies Associated with Cervical Lymphadenopathy

2021 ◽  
Vol 35 (4) ◽  
pp. 25-29
Author(s):  
Sohail Anwar
Keyword(s):  
Diagnostic Tool ◽  
Cervical Lymphadenopathy ◽  
Cost Effective ◽  
Needle Aspiration ◽  
P Value ◽  
Invasive Test ◽  
Outpatient Departments ◽  
Metastatic Neoplasm ◽  
Needle Aspiration Cytology ◽  
Age Range

Introduction: Cervical lymphadenopathy is very common as the presenting complaint in our outpatient departments. Its etiology may either be benign or malignant. Fine needle aspiration cytology (FNAC) is a comparatively cheap, easy to perform minimally invasive test. It has become the go to test to determine the cause of lymphadenopathy Aims & Objectives: To determine whether FNAC is a cost-effective diagnostic tool in evaluation of various benign and malignant Pathologies associated with cervical lymphadenopathy. Place and duration of study: It is a retrospective study conducted at Gulab Devi Hospital from April 2019 to April 2021. Material & Methods: The cyto-morphologic features seen in the aspirates were critically analyzed and correlated with their etiology. SPSS version 24.0 was analyzed for data analysis. A p-value of <0.05 was considered significant. Results: Among the 100 patients 56 were females and 44 were males with a female to male ratio of 1.3:1. The age range of the patients was from 3 to 75 years. 37% were reactive lymphadenitis, 32% were tuberculosis, 12% were suppurative, 11% were lymphoproliferative disorders, 8% cases were metastatic neoplasm. Conclusion: FNAC is a reliable and cost effective to diagnose the etiologies of cervical lymphadenopathy.

scholarly journals Isolated Intracranial Rosai-Dorfman Disease

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Md. Taufiq ◽  
Abul Khair ◽  
Ferdousy Begum ◽  
Shabnam Akhter ◽  
Md. Shamim Farooq ◽  
...  
Keyword(s):  
Case Report ◽  
Ct Scan ◽  
Clinical Improvement ◽  
Cervical Lymphadenopathy ◽  
Rare Condition ◽  
Unknown Etiology ◽  
Nodal Involvement ◽  
Rosai Dorfman Disease ◽  
Parasellar Region ◽  
Significant Clinical Improvement

Background. Rosai-Dorfman disease (RDD) is a benign histiocytic proliferative disorder of unknown etiology. This rare condition commonly causes massive cervical lymphadenopathy. Intracranial RDD without any nodal involvement is extremely rare.Case Report. A young Bangladeshi male complained of bilateral complete blindness with left sided deafness for about three years. There was no lymphadenopathy. MRI and CT scan of brain suggested an inflammatory/neoplastic (?meningioma) lesion located at left parasellar region which extended frontally to encircle both optic nerves and also to left prepontine area. Histopathologically the lesion was diagnosed as RDD. The patient was treated with steroid and significant clinical improvement observed.Conclusion. The prognosis of intracranial RDD is not poor. It can be treated with surgery with or without corticosteroids, chemotherapy, and so forth. But as the condition is extremely rare and often misdiagnosed, the clinician, radiologist, and histopathologist should have a suspicion in their mind about the possibility of RDD.

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