Rosai–Dorfman Disease: A Rare Clinicopathological Presentation and Review of the Literature

Azari‐Yaam, Aileen; Abdolsalehi, Mohammad Reza; Vasei, Mohammad; Safavi, Mohammadreza; Mehdizadeh, Mehrzad

doi:10.1007/s12105-020-01183-7

Cited by 15 publications

(6 citation statements)

References 32 publications

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“…2 The presence of tender lymphadenopathy distinguishes KFD from nontuberculous mycobacterial lymphadenitis and Rosai-Dorfman-Destombes disease (RDD), both of which present with painless nodes. 3,4 Additionally, most cases of nontuberculous mycobacterial lymphadenitis occur in children younger than 5 years old, making this diagnosis more unlikely. 4 Although many patients with KFD have a normal complete blood count, leukopenia has been reported in up to 43% of cases.…”

Section: Discussionmentioning

confidence: 99%

Tender Cervical Lymphadenopathy in a Pediatric Patient

Brown,

Scott

2024

JAMA Otolaryngol Head Neck Surg

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Section: Discussionmentioning

confidence: 99%

Tender Cervical Lymphadenopathy in a Pediatric Patient

Brown,

Scott

2024

JAMA Otolaryngol Head Neck Surg

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“…The diagnosis of RDD may be delayed because of its low prevalence and late recognition. RDD is a rare disease that is often undiagnosed at early stages ( 14 ). There are many differential diagnoses, including infections with, for example, acid-fast bacilli and fungi; and malignant diseases, including malignant lymphoma; Erdheim-Chester disease; and IgG4-related disease.…”

Section: Discussionmentioning

confidence: 99%

Case report: Rosai-Dorfman disease with rare extranodal lesions in the pelvis, heart, liver and skin

Yoshida

Zoshima²,

Hara³

et al. 2023

Front. Oncol.

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Rosai-Dorfman disease (RDD), a rare form of non-Langerhans cell histiocytosis, can involve systemic extranodal lesions. Skin lesions are the most common, whereas intrapelvic, cardiac, and hepatic lesions are infrequent. The present study describes a 74-year-old woman with multiple extranodal lesions in the pelvis, heart, liver, and skin that were successfully treated with glucocorticoid therapy. She had experienced fever and persistent inflammation without cervical lymphadenopathy for several months and 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) showed abnormal FDG uptake in the left cheek; cervical, axillary, inguinal lymph nodes; periatrium; and pelvis. She was diagnosed with RDD based on skin and pelvic biopsies. Although this was an atypical case without bilateral cervical lymphadenopathy, the FDG-PET detection of inflammatory lesions led to selection of suitable biopsy sites, and pathological examination led to a correct diagnosis. Findings in this patient indicate that RDD can present with an atypical distribution of infrequent extranodal lesions, with attention required to prevent a delayed diagnosis.

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“…The classical nodal disease is described as enlarged, painless bilateral cervical lymph nodes [ 6 ], night sweats, fever or weight loss, and leukocytosis. The mediastinal, axillary, inguinal, and para-aortic regions are other frequent sites for lymphadenopathies.…”

Section: Discussionmentioning

confidence: 99%

“…The underlying causes of RDD remain unclear. Cervical lymphadenopathy is the most common presentation of RDD [ 6 ], with extranodal compromise reported in less than 50% of the cases. Therefore, histopathologic evaluation is crucial for diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Synchronous Mediastinal and Central Nervous System Involvement in Rosai-Dorfman Disease: A Case Report

Soto-Davila,

Klapper,

Afude

et al. 2023

Cureus

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Rosai-Dorfman disease (RDD) is a rare disorder characterized by the proliferation and accumulation of histiocytes, primarily within lymph node sinuses. Uncommonly, other extranodal sites, such as the central nervous system, can also be affected. Here, we document the case of a 61-year-old woman presenting with dizziness, confusion, and headaches. Magnetic resonance imaging (MRI) showed an extra-axial avidly enhancing mass in the left parietal region presumed to be a meningioma based solely on its imaging appearance. The patient underwent surgical resection, and histopathological examination showed enlarged histiocytes positive for S100, CD68, and CD163 and negative for CD1a, consistent with RDD. She was followed up with a positron emission tomography/computed tomography (PET/CT) to evaluate other disease activity sites. A single mediastinal node was identified adjacent to the atriocaval junction intensely fluorodeoxyglucose avid. The patient underwent robotic node excision, with pathology analysis compatible with RDD. We emphasize the need to increase recognition of RDD on differential brain lesions, especially meningiomas, and suggest PET/CT as a valid tool to search for other disease activity lesions.

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Rosai–Dorfman Disease: A Rare Clinicopathological Presentation and Review of the Literature

Cited by 15 publications

References 32 publications

Tender Cervical Lymphadenopathy in a Pediatric Patient

Tender Cervical Lymphadenopathy in a Pediatric Patient

Case report: Rosai-Dorfman disease with rare extranodal lesions in the pelvis, heart, liver and skin

Synchronous Mediastinal and Central Nervous System Involvement in Rosai-Dorfman Disease: A Case Report

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