Rosai-Dorfman disease with spinal and multiple intracranial involvement: a case report and literature review

Qin, Guowen; Ye, Jin; Lan, Shengyong; Liang, Yinghua; Xu, Peng; Tang, Xihe; Guo, Wenwen

doi:10.1080/02688697.2019.1567681

Cited by 8 publications

(7 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, for symptomatic patients, for whom cord decompression and restoration of spinal stability are indicated, or malignancy cannot be ruled out, surgery is the treatment of choice whenever feasible. Theoretically, total resection is a better choice, considering the tendency of the lesions to recur (Table 4) [3,14,15,29,30,32,43,44,49,51]. This study found that the recurrence rate of total resection was marginally lower than that of non-total resection (9.1% versus 26.1%).…”

Section: Discussionmentioning

confidence: 77%

“…Chemotherapy regimens were usually prescribed based on personal experience rather than high-level evidence, and its efficacy was not verified in the most patients [73]. The efficacy of steroids was not supported in the case series of this study, either, especially for aged ones or patients with multiple organs involvement [11,14,31,44,45,62]. Thus, surgical resection, with or without adjuvant therapies, remains as a primary modality for spinal RDD when therapeutic interventions are indicated.…”

Section: Discussionmentioning

confidence: 85%

See 1 more Smart Citation

Diagnosis and treatment of Rosai-Dorfman disease of the spine: a systematic literature review

Wei

Liu

et al. 2021

Syst Rev

View full text Add to dashboard Cite

Purpose To review and summarize the clinical features, diagnosis, treatment strategies, and prognosis of spinal Rosai-Dorfman disease (RDD). Methods RDD is also termed as sinus histiocytosis with massive lymphadenopathy. We searched the databases of PubMed, Elsevier ScienceDirect, SpringerLink, and OVID. The keywords were Rosai-Dorfman disease and spine/central nervous system. Research articles and case reports with accessibility to full texts regarding spinal RDD were eligible for the inclusion. A total of 62 articles were included, and they contained 69 cases. We extracted the information of interest and analyzed them using SPSS statistics package. Results The average age was 33.1 ± 18.3 years. The ratio of males to females was 1.9/1. Overall, 63 cases presented with spine-related symptoms. A total of 27 cases (39.1%) had multi-organ lesions, and 12 cases had records of massive lymphadenopathy. Among 47 cases who first manifested spine-related symptoms, 93.6% were preoperatively misdiagnosed. The disease had a predilection for cervical spine (38.8%) and thoracic spine (40.3%). 62.9% of lesions were dura-based. Surgery remained the mainstream treatment option (78.8%), with or without adjuvant therapies. Total lesion resection was achieved in 34.8% of cases. The rate of lesion recurrence/progression was 19.5%, which was marginally lower for total resection than for non-total resection. Conclusion Spinal RDD has no pathognomonic clinical and imaging features. Most cases first present with spine-relevant symptoms. Massive lymphadenopathy is not common, but a tendency for multi-organ involvement should be considered. Spinal RDD has a high recurrence rate; thus, total resection is the treatment of choice. Adjuvant therapies are indicated for multi-organ lesions and residual lesions. A wait and watch strategy is recommended for asymptomatic patients. Herein, a workflow of diagnosis and treatment of the spinal RDD is established.

show abstract

Section: Discussionmentioning

confidence: 77%

Section: Discussionmentioning

confidence: 85%

Diagnosis and treatment of Rosai-Dorfman disease of the spine: a systematic literature review

Wei

Liu

et al. 2021

Syst Rev

View full text Add to dashboard Cite

show abstract

“…Rosai-Dorfman disease is an uncommon disorder that rarely involves CNS, moreover, multiple intracranial lesions have also been seldom reported. As indicated in previous studies, this specific type of RDD has a propensity to occur in males (male : female 3.3 : 1) and the age of patients at diagnosis ranges from 2 to 72 years [1,4,7,8,10,13,16,20,[25][26][27][31][32][33]35,[38][39][40][41]44,48,51,53,55] (Table II). Presenting symptoms associated with neurological deficits and mass effects depend on the location, size, and growth pattern of lesions.…”

Section: Discussionmentioning

confidence: 63%

Clinicopathological characteristics of multiple intracranial Rosai-Dorfman disease with increased IgG4-positive plasma cells: a report of two cases

Zhou¹,

Ren²,

Liu³

et al. 2021

View full text Add to dashboard Cite

Rosai-Dorfman disease (RDD) is an uncommon condition characterized by the proliferation of histiocytes and multiple intracranial involvements and it is extremely rare. Here, we present two cases of multiple intracranial RDD mimicking meningioma. These patients underwent surgery for tumour resection and pathological findings revealed an increased number of IgG4-positive plasma cells in RDD. The radiographic appearance and histology may contribute to a diagnostic dilemma, and immunohistochemical and serological examinations are a necessary complement for definitive diagnosis. Treatment protocols pertaining to such types of RDD cases are reviewed. Currently, surgical resection is the most effective therapy, and steroid therapy, radiotherapy, or chemotherapy may be provided as adjuvant treatments in some selected patients.

show abstract

“…There are rare presentations of RDD as intraventricular masses, similar to the case presented here, or dural, venous sinuses, and bone lesions ( 3 , 10 ). Nearly 90% of CNS RDD cases are intracranial lesions, however, it is possible to observe dural-based masses in the vertebral canal usually causing compression of the spinal cord and subsequent paraplegia ( 9 , 11 – 13 ). The available literature has described some cases of intraventricular presentation ( 12 , 14 ) in adult patients, and our case demonstrates multiple intraventricular masses in a child.…”

Section: Discussionmentioning

confidence: 99%

Intracranial Rosai Dorfman Disease Presented With Multiple Huge Intraventricular Masses: A Case Report

et al. 2022

View full text Add to dashboard Cite

IntroductionRosai-Dorfman disease (RDD) usually presents with painless cervical lymphadenopathy during the two first decades of life, with or without extranodal involvement. Exclusive extranodal manifestation, and especially central nervous system (CNS) involvement, is uncommon. The etiology remains unknown and definitive diagnosis is based on characteristic histopathological and immunohistochemical features showing S100(+) CD1a(−) RDD type histiocytes with emperipolesis. Most CNS cases are dural-based masses.Case PresentationHerein, we present a case of RDD in an 8-year-old boy, who presented with multiple huge intraventricular masses.ConclusionRegarding rare CNS involvement and lack of established evidence-based therapeutic approaches, reports of any individual case can supply further beneficial data concerning treatment approaches and long-term effectiveness of therapeutic strategies.

show abstract

Rosai-Dorfman disease with spinal and multiple intracranial involvement: a case report and literature review

Cited by 8 publications

References 26 publications

Diagnosis and treatment of Rosai-Dorfman disease of the spine: a systematic literature review

Diagnosis and treatment of Rosai-Dorfman disease of the spine: a systematic literature review

Clinicopathological characteristics of multiple intracranial Rosai-Dorfman disease with increased IgG4-positive plasma cells: a report of two cases

Intracranial Rosai Dorfman Disease Presented With Multiple Huge Intraventricular Masses: A Case Report

Contact Info

Product

Resources

About