Bone pathologic fracture revealing an unusual association: coexistence of Langerhans cell histiocytosis with Rosai-Dorfman disease

Efared, Boubacar; Mazti, Asmae; Chaibou, Badarou; Atsame-Ebang, Gabrielle; Sidibé, Ibrahim S.; Tahiri, Layla; Erregad, Fatimazahra; Hammas, Nawal; Mrini, Abdelmajid El; Fatemi, Hinde El; Chbani, Laïla

doi:10.1186/s12907-017-0044-1

Cited by 9 publications

(9 citation statements)

References 15 publications

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“…14 Alternatively, few studies have shown some 'bank' cells demonstrating features of both RDD and LCH. 9 These bank cells may also explain one condition evolving into another. Our study may represent the same evolution.…”

Section: Discussionmentioning

confidence: 99%

“…10 Orbital RDD and LCH are both rare manifestations of histiocytic disorders. The co-existence of RDD and LCH in a patient has been reported in few studies only, mostly affecting separate organs 8,9,[11][12][13][14] (Table 2). We hereby report a case of co-occurrence of orbital RDD and LCH in a patient with lymphadenopathy and orbital mass.…”

Section: Introductionmentioning

confidence: 99%

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Coexistent Rosai Dorfman disease and Langerhans cell histiocytosis in an Orbital mass: A Case Report

Rastogi

Jaisingh

Rajurkar

et al. 2022

European Journal of Ophthalmology

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Introduction Rosai Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder of lymph node sinuses. Langerhans cell histiocytosis (LCH) is a solitary or multisystem clonal proliferation of abnormal dendritic cells (Langerhans cells) with varied presentations. The co-occurrence of these two entities is quite rare. Case description A six-year-old boy presented with multiple mass lesions in the neck since two years and a nodular lesion in right upper eyelid for the past 4 months. He was diagnosed with tubercular lymphadenitis 2 years back, and was given a course of anti-tubercular therapy (ATT) elsewhere. No improvement was seen. Fine needle aspiration cytology (FNAC) of the cervical lymph nodes revealed reactive lymphadenitis while lymph node biopsy showed features of RDD. Excision biopsy of the orbital mass showed features of both RDD and LCH. The patient was started on tablet prednisolone. Six months later, complete resolution of lymph node enlargement and remaining orbital mass was noted. Post operative contrast enhanced Magnetic Resonance Imaging of head and neck was normal. Conclusion The coexistence of RDD and LCH may be a result of divergent differentiation from a common lineage or a de novo phenotypic evolution.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Coexistent Rosai Dorfman disease and Langerhans cell histiocytosis in an Orbital mass: A Case Report

Rastogi

Jaisingh

Rajurkar

et al. 2022

European Journal of Ophthalmology

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“…Langerhans cell histiocytosis (LCH) and RDD coexisting as 2 distinct lesions has been reported, as have LCH and JXG, and RDD and JXG . LCH and RDD coexistence as a single lesion has also been recently reported . O'Malley et al have recently evaluated clonal abnormalities and genetic gains and/or losses in LCH and RDD coexistence, and highlight that these diseases may belong to a spectrum linked by clonal abnormalities .…”

Section: Discussionmentioning

confidence: 99%

Emperipolesis and S100 expression may be seen in cutaneous xanthogranulomas: A multi‐institutional observation

et al. 2018

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Cutaneous Rosai-Dorfman disease (RDD) can be difficult to distinguish from other non-Langerhans cell histiocytoses, particularly xanthogranuloma (XG). Pathologists use S100 immunoreactivity, abundant plasma cells, and the presence of emperipolesis to distinguish RDD from XG. However, S100 expression has been reported in XG and, in practice, we have occasionally observed emperipolesis in cases that were otherwise clinically and pathologically consistent with XG. We present 10 cases of XG with emperipolesis and variable S100 immunoreactivity. Histologically, 7 cases were most in keeping with XG, and a histologic differential of XG versus RDD was raised in the remaining 3 cases. All 10 cases were clinically consistent with XG. Notably, none of these cases showed abundant plasma cells. Nine cases showed variable S100 immunostaining, ranging from focal/weak expression, to focal/strong, diffuse/moderate, and diffuse/strong expression. Histiocytes in all cases were CD68 positive and CD1a negative. We conclude that emperipolesis and S100 expression in a skin biopsy cannot reliably distinguish XG from cutaneous manifestations of RDD. Clinical correlations are essential, as are histologic clues to a diagnosis of classic XG that include an abundance of foamy mononuclear cells, Touton giant cells, and an absence of pale-stained histiocytes, abundant plasma cells, fibrosis, or vascular proliferation.

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“…54,55 Rosai-Dorfman disease and LCH are distinct clinicopathologic entities, but rare cases with concurrence of both conditions in a single site have been described, including cases involving bone and soft tissue, suggesting a possible relationship between the two. [56][57][58] Furthermore, BRAF-V600E mutations have been detected in such combined cases, suggesting that Langerhans cells and RDD histiocytes may derive from a common precursor. 59 One case of nodal RDD with confirmed BRAF-V600E mutation has been reported, representing a promising therapeutic target, especially for patients with refractory or extensive disease.…”

Section: Pathogenesismentioning

confidence: 99%

Rosai-Dorfman Disease of Bone and Soft Tissue

Garcı́a

DiCarlo

2021

Archives of Pathology &Amp; Laboratory Medicine

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Context.— Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown pathogenesis that may be diagnostically difficult in extranodal sites. It is commonly an unsuspected diagnosis when arising in bone and soft tissue, especially when it presents without associated lymphadenopathy. Its variable clinical presentation and nonspecific imaging findings make the diagnosis quite challenging, particularly in small biopsies. The problem is compounded by its less-characteristic histomorphologic features in comparison with nodal disease. Awareness of the potential diagnostic pitfalls in Rosai-Dorfman disease of bone and soft tissue should raise the degree of diagnostic accuracy. Objective.— To review the clinical manifestations, imaging characteristics, and histomorphologic features of Rosai-Dorfman disease of bone and soft tissue along with a brief discussion of its differential diagnosis, pathogenesis, and current management. Data Sources.— Thorough review of the literature with focus on clinical manifestations, imaging findings, key histomorphologic features, pathogenesis, and treatment. Conclusions.— The diagnosis of Rosai-Dorfman disease of bone and soft tissue may be quite challenging because of its variable clinical presentation and nonspecific imaging findings. It may be asymptomatic without systemic manifestations or associated lymphadenopathy. The definitive diagnosis relies on histopathologic identification of the characteristic S-100–positive histiocytes demonstrating emperipolesis. Bone and soft tissue lesions tend to have lower numbers of characteristic histiocytes and less conspicuous emperipolesis and often demonstrate areas of fibrosis or storiform spindle cell areas resembling fibrohistiocytic lesions. Awareness of these unusual features is necessary in order to consider Rosai-Dorfman disease in the differential diagnosis when confronting these rare and often misleading lesions.

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Bone pathologic fracture revealing an unusual association: coexistence of Langerhans cell histiocytosis with Rosai-Dorfman disease

Cited by 9 publications

References 15 publications

Coexistent Rosai Dorfman disease and Langerhans cell histiocytosis in an Orbital mass: A Case Report

Coexistent Rosai Dorfman disease and Langerhans cell histiocytosis in an Orbital mass: A Case Report

Emperipolesis and S100 expression may be seen in cutaneous xanthogranulomas: A multi‐institutional observation

Rosai-Dorfman Disease of Bone and Soft Tissue

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