Rosai-Dorfman Disease and Unusual Local Invasive Presentation

Mohammadi, Oranus; Lisigurski, Miriam Zylberglait; Mehra, Divy; Pishdad, Reza; Güleç, Seza A.

doi:10.7759/cureus.7328

Cited by 4 publications

(8 citation statements)

References 10 publications

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“…Additionally, in 29 out of 53 cases, there was evidence of extra osseous involvement, particularly in soft tissues, lymph nodes, and sinuses. Nasany RA et al 60 found that RDD involvement was most commonly observed in the dura, followed by the spine, brain parenchyma, orbits, and calvarium.…”

Section: Discussionmentioning

confidence: 99%

Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

Agarwal,

Abozeed

2023

Preprint

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BACKGROUND We hypothesize that lymphadenopathy and extraosseous manifestation affect the management protocol and our aim is to aid in development of standardized diagnosis and treatment protocols. METHODS PubMed and Google Scholar were searched from January 2012 through December 2022 using keywords Rosai-Dorfman, Rosai and Dorfman, and bone. Out of 98 researches, meta-analysis of 51 full-text studies involving 53 individuals with osseous RDD was conducted. RESULTS A significant association was found between lymphadenopathy with steroid therapy (OR: 20.125, 95%CI: 3.02, 134.09, P=0.0004). Extraosseous RDD showed significant association with steroid therapy (P=0.0145), T1-isointense MRI (OR: 0.173, 95%CI: 0.0304, 0.986, P=0.035), T1-hyperintense MRI (OR: 0.16, 95%CI: 0.0314, 0.815, P=0.019) and T2-hyperintense MRI (OR: 0.115, 95%CI= 0.0206, 0.6456, P=0.007). CONCLUSION Osseous RDD is rare and lacks standardized diagnosis and treatment protocols. Patients with lymphadenopathy were 9 times more likely to receive steroids, and those with extraosseous manifestations were less likely to undergo MRI. RDD is often not initially considered in differential diagnoses, leading to worsened conditions. We propose that further testing is required in affected individuals to develop standardized protocols.

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Section: Discussionmentioning

confidence: 99%

Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

Agarwal,

Abozeed

2023

Preprint

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“…In RDD, the histiocytes tend to stain positive for CD68, CD14, fascin, CD163, HLA-DR, and S100; however, they stain negatively for CD1a. These findings can help to establish the diagnosis of RDD [1][2][3] and rule out other conditions such as Langerhans cell histiocytosis, histiocytic sarcoma, classical Hodgkin lymphoma, melanoma, metastatic carcinomas, and infectious lymphadenopathies [1,4] . The clinical diagnosis of RDD is challenging as the clinical presentation is usually variable, especially the extranodal type in the absence of lymphadenopathy.…”

Section: Discussionmentioning

confidence: 99%

“…RDD has shown to have a good prognosis, particularly the cutaneous subtype; however, lower respiratory tract or hepatic involvement is considered a poor prognostic factor. The major causes of mortality include amyloidosis or vital organ involvement [ 4 ] .…”

Section: Discussionmentioning

confidence: 99%

“…In refractory cases, dapsone, imatinib, and thalidomide may be used, yet more research is needed to confirm their efficacy [ 1 , 2 ] . Surgical excision may be indicated in symptomatic lesions with vital organ involvement or extranodal disease that is potentially resectable [ 4 ] . Our patient was treated with topical steroids with no improvement; however, the intralesional injection of steroids resulted in considerable clinical improvement.…”

Section: Discussionmentioning

confidence: 99%

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A Rare Case of Benign Histiocytic Neoplasm of Cutaneous Rosai–Dorfman Disease: A Case Report and Literature Review

Mekheal

Roman

Kania

et al. 2022

European Journal of Case Reports in Internal Medicine

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Rosai–Dorfman disease (RDD) is a rare disorder characterized by the proliferation of histiocytes in lymph nodes. It can occur anywhere in the body but commonly involves the cervical area of the neck. Its clinical presentation varies and patients with skin manifestations may develop papules, nodules, plaques, or pustules. Histologically, it typically presents with emperipolesis, where intact lymphocytes are found within histiocytes. The definitive treatment of RDD is not well established given the rarity of the disease and indeed skin lesions can regress spontaneously. Therapeutic treatment options include cryotherapy, radiation, or topical agents such as steroids or retinoids. Here we describe the case of a 24-year-old Hispanic female who presented with skin manifestations which proved to be histologically positive for Rosai–Dorfman disease. The patient clinically improved following the administration of intralesional steroids.

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“…The treatment strategies for RDD include an expectative approach and monitoring, corticosteroids (usually prednisone or dexamethasone), surgery for unifocal and/or symptomatic extranodal disease, radiotherapy (with a dose of 30-50 Gy), chemotherapy (Vinca alkaloids, methotrexate, cladribine), or immunomodulatory therapy (thalidomide, lenalidomide, rituximab, imatinib mesylate) [12,14,15].…”

Section: Introductionmentioning

confidence: 99%

Rosai–Dorfman Disease: Breast Involvement—Case Report and Literature Review

et al. 2021

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Background and objectives: Rosai–Dorfman disease (RDD) is a type of histiocytosis that usually appears in young adults or children as bilateral cervical lymphadenopathy, but extranodal involvement in not uncommon. Although the pathogenesis is not entirely elucidated, recent studies showed a possible neoplastic process. Materials and methods: Our manuscript presents a rare case of Rosai–Dorfman disease of the breast, the management of this rare case, and a literature review. There are few cases reported of RDD of the breast (around 90 globally reported cases); the data is poor, and the management not yet standardized for these cases. The case reported here shows the importance of correct breast investigation, breast imaging, and ultrasound-guided biopsy that provided an accurate diagnosis and guided further management. Results: Although RDD of the breast was rarely presented as bilateral disease in other case reports, our case showed bilateral breast disease with the suspicion of breast cancer on imaging. Pathology and immunohistochemistry were of critical importance and showed a specific pattern for histiocytosis. A multidisciplinary approach was taken into consideration for these cases in order to establish the approach. Some patients underwent surgery, but watchful waiting and close follow-up were the preferred approach. Conclusions: RDD of the breast is a rare form of histiocytosis, with fewer than 100 globally published cases. Although the management of this disease is not established yet by guidelines, a follow-up approach should be enough for these patients, and surgery might be overtreatment. Mortality from RDD is very low due to comorbidities. A multidisciplinary team decision is important, and abstinence might significantly benefit these patients.

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Rosai-Dorfman Disease and Unusual Local Invasive Presentation

Cited by 4 publications

References 10 publications

Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

A Rare Case of Benign Histiocytic Neoplasm of Cutaneous Rosai–Dorfman Disease: A Case Report and Literature Review

Rosai–Dorfman Disease: Breast Involvement—Case Report and Literature Review

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