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.

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.

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>

scholarly journals Cutaneous Rosai-Dorfman Disease: A Case Report

2018 ◽  
Vol 2 (4) ◽  
pp. 243-247
Author(s):  
Nahla Shihab ◽  
Whitney Talbott ◽  
Nathalie O Kahn ◽  
Erisa Alia ◽  
Jeffrey M Weinberg
Keyword(s):  
African American ◽  
Case Report ◽  
African American Woman ◽  
Clinical Manifestations ◽  
American Woman ◽  
Unknown Etiology ◽  
The Past ◽  
Rosai Dorfman Disease

Cutaneous Rosai-Dorfman Disease (CRDD) is an uncommon benign histiocytosis of unknown etiology. CRDD is oftentimes misdiagnosed because it has variable clinical manifestations, particularly in the absence of lymphadenopathy. We report a case of a 23-year-old African American woman presenting with clustered nodular plaques on her right thigh, buttocks, back, and chest for the past two years. History, clinical, histopathological, and immunochemistry findings corresponded with CRDD, and as such, she was treated with halobetasol propionate 0.05% cream twice daily.

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 Measurement of cerebrospinal fluid ACE level in aseptic meningitis: diagnostic?

2019 ◽  
Vol 12 (8) ◽  
pp. e230532
Author(s):  
Biplab Kumar Saha ◽  
Aditi Saha ◽  
Scott Beegle
Keyword(s):  
Cerebrospinal Fluid ◽  
Complete Resolution ◽  
Clinical Presentation ◽  
African American Woman ◽  
American Woman ◽  
High Specificity ◽  
Systemic Steroid ◽  
Diagnostic Challenge ◽  
Systemic Steroid Therapy ◽  
Structural Brain

Neurosarcoidosis (NS) is a rare disease, affecting only 3%–10% of patients with sarcoidosis. The clinical presentation can be protean and often represents a diagnostic challenge. Cerebrospinal fluid (CSF) ACE level has poor sensitivity, but high specificity for establishing a diagnosis of NS. We present a case of NS in a middle-aged African American woman who presented with dysphagia and dysphonia. An extensive radiological workup was negative for structural brain disease. CSF studies demonstrated lymphocyte predominant pleocytosis with an elevated ACE level. A diagnosis of possible neurosarcoidosis was made. She responded to systemic steroid therapy with complete resolution of her symptoms over the next five months. In the appropriate clinical setting, an elevated CSF ACE level could be of paramount importance for making a diagnosis of NS.

scholarly journals Giant lipoma of breast-a diagnostic dilemma

2021 ◽  
Vol 8 (2) ◽  
pp. 752
Author(s):  
D. J. Balsarkar ◽  
Sachin A. Suryawanshi ◽  
Muna Shaikh ◽  
Sudhir Dhobale
Keyword(s):  
Phyllodes Tumor ◽  
Surgical Excision ◽  
Needle Aspiration ◽  
Left Breast ◽  
Normal Breast ◽  
Diagnostic Challenge ◽  
Diagnostic Dilemma ◽  
Common Benign Tumor ◽  
Complete Surgical Excision ◽  
Giant Lipoma

Lipoma is the most common benign tumor of adipose tissue. Giant lipoma of breast is very rare.Majority of them are small in size, slow growing and asymptomatic until they reach large size. Most patients seek medical advice due to asymmetry in the breast and due to fear of malignancy. Breast lipomas pose a diagnostic challenge due to similarity of their texture to normal breast parenchyma and make it difficult to distinguish from other common breast lesions. The clinical and radiographic identification of breast lipoma remains challenging. Complete surgical excision with the capsule is essential to prevent recurrence. Breast reconstruction may require, to prevent asymmetry following surgical excision of giant breast lipoma. High degree of clinical suspicion and histolopathological correlation will help in preopertaive diagnosis of this clinical condition. Here, we report a case 50- year -old woman with left breast lump which clinically, radiologically and on fine needle aspiration cytology was diagnosed as phyllodes tumor, but after surgical excision it was reported as breast lipoma on final histopathology.

A 62-Year-Old African American Woman With Stable Lymphadenopathy Over 5 Years

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S110-S110
Author(s):  
Hanadi El Achi ◽  
William Glass ◽  
Miriam Velazquez ◽  
Zhihong Hu
Keyword(s):  
African American ◽  
Plasma Cells ◽  
African American Woman ◽  
American Woman ◽  
Granulomatous Inflammation ◽  
Chronic Fatigue ◽  
Giant Cells ◽  
Castleman Disease ◽  
African American Female ◽  
Mantle Zone

Abstract The patient is a 62-year-old African American female with medical history of Plummer Vinson syndrome and CT scan in 2014 showing lymphadenopathy of the porta hepatis. Needle core biopsy of enlarged lymph nodes revealed sheets of polytypic plasma cells with no aberrant B or T cells or granulomas or hyaline-like depositions identified. The findings were suspicious for unicentric Castleman disease. In 2019, the patient presented with chronic fatigue without other systemic symptoms. Contrast CT revealed persistent retroperitoneal lymphadenopathy and calcified granulomas in the liver, spleen, and lung. Excision of portal and celiac nodes showed preserved nodal architecture with mild mantle zone expansion and marked hyaline-like deposition in the follicles. Sheets of mature plasma cells and focally increased vasculatures were seen in the interfollicular areas. Focally frequent nonnecrotizing granulomas were noted. Occasional giant cells and rare asteroid bodies were identified in the granulomas. Immunohistochemistry stain workup revealed normal distribution of the B and T lymphocytes. CD138 stain highlighted marked increased plasma cells and HHV8 was negative. Immunofluorescence microscopy for kappa and lambda did not show monoclonality; IgA was positive in <10% of plasma cells; special stain for Congo red and serum amyloid associated protein (SSA) IHC stain were strongly positive in the follicles. The overall findings are consistent with reactive lymphadenopathy with interfollicular polytypic plasmacytosis, noncaseating granuloma formation, and reactive amyloidosis. The features of mantle zone hyperplasia and polytypic plasmacytosis are suggestive of HHV8-negative/idiopathic unicentric Castleman disease. This case possibly represents an example of concurrent amyloidosis, sarcoidosis, and idiopathic unicentric Castleman disease. Given that SSA was recently reported to have a pathogenic role in granulomatous inflammation of sarcoidosis through sustained inflammatory reaction, the possibility of increased SSA triggering sarcoidoisis in this patient can be considered. However, the exact pathogenesis of these concurrent findings needs further investigation and clarification.

scholarly journals Diagnosis of Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease) by Fine Needle Aspiration Cytology in a Paediatric patient

2021 ◽  
Vol 7 (4) ◽  
pp. 215-218
Author(s):  
Dr. Neema Ankur Rana ◽  
◽  
Dr. Durva Vijaykumar Patel ◽  
Dr. Hiral Samir Shah ◽  
Dr. Meena Rajiv Daveshwar ◽  
...  
Keyword(s):  
Paediatric Patient ◽  
Fine Needle Aspiration ◽  
Plasma Cells ◽  
Needle Aspiration ◽  
Sinus Histiocytosis ◽  
Aspiration Cytology ◽  
Fine Needle ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Needle Aspiration Cytology

Rosai-Dorfman Disease (RDD) is also known as Sinus Histiocytosis of Massive Lymphadenopathy(SHML). Clinically Rosai-Dorfman Disease is characterized by massive, painless, bilateral cervicallymph node enlargement, often mimics lymphoma. Microscopically, it shows dilatation of lymphaticsinuses occupied by numerous lymphocytes and proliferation of histiocytes with abundant paleeosinophilic cytoplasm containing engulfed lymphocytes or plasma cells- emperipolesis.

scholarly journals Asymptomatic flow of Rosai-Dorfman disease

2014 ◽  
Vol 71 (8) ◽  
pp. 780-783 ◽  
Author(s):  
Dragan Petrovic ◽  
Dragan Mihailovic ◽  
Sladjana Petrovic ◽  
Nikola Zivkovic ◽  
Zaklina Mijovic ◽  
...  
Keyword(s):  
Plasma Cells ◽  
S100 Protein ◽  
Unknown Etiology ◽  
Neoplastic Disease ◽  
Diagnostic Dilemma ◽  
Regional Lymph Nodes ◽  
Parotid Region ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Abundant Cytoplasm

Introduction. Sinus histiocytosis with massive lymphadenopathy is a rare benign self-limiting disease of unknown etiology. The salivary gland involvement, indicating the extranodal character of the disease, often presents a diagnostic dilemma requiring immunohistochemical staining of surgically removed tumor to confirm the diagnosis. Case report. We report a 43-year-old man presented with an asymptomatic mass in the left mandibular angle. On physical examination, the lesion was described as a painless, mobile, firm-elastic consistency nodule, which measured 4 ? 3 cm in diameter, with normal overlying skin. A mass with the same characteristics, dimensions 2 ? 2 cm, was also noted in the right parotid region. No other changes in regional lymph nodes were detected. On macroscopic examination the lesion was firm, multilobulated, yellowish and rounded, while on microscopic examination the lesion was composed almost entirely of polygonal histiocytes with abundant cytoplasm, emperipolesis, plasma cells arranged in sheets, and lymphocytes scattered or within clusters. The observed histiocytes were found to be CD68 and S100 protein positive. Conclusion. Rosai-Dorfman disease is a beningn and frequently overlooked clinical and pathological entity that may be misinterpreted as a neoplastic disease.

scholarly journals Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Volkan Karakuş ◽  
Yelda Morgül Dere ◽  
Dilek Ersil Soysal
Keyword(s):  
Lymph Nodes ◽  
Low Dose ◽  
Plasma Cells ◽  
Oral Prednisolone ◽  
Excisional Biopsy ◽  
Steroid Treatment ◽  
Unknown Etiology ◽  
Rosai Dorfman Disease ◽  
S 100

Rosai–Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the enlarged sinusoids of the lymph nodes. The clinical course is unpredictable, but is often benign with spontaneous resolution of disease in most patients. We report a patient with bilateral massive enlargement of cervical, axillary, and inguinal lymph nodes, moderately enlarged spleen, and a weight loss of 15 kg. Excisional biopsy from the cervical lymph node showed that the dilated sinusoids were infiltrated by lymphocytes, plasma cells, and large histiocytes with CD 68 and S-100 protein positive. Due to the slow progression of the disease, oral prednisolone with a body weight of 1 mg/kg was started in March 2016. The steroid dosage has been adjusted many times during the clinical follow-up. After 33 months, steroid treatment resulted in partial shrinkage of lymph nodes, the spleen returned to its normal size, and the patient gained weight. After 38 months of follow-up, no systemic symptoms, sign, or extranodal involvement were detected, and the patient continued with low-dose steroid treatment.

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