Isolated spinal Rosai–Dorfman disease misdiagnosed as lymphoplasmacyte-rich meningioma by intraoperative histological examination

Fu, Xinchu; Jiang, Ju hong; Tian, Xiao Ying; Li, Zhi

doi:10.1007/s10014-014-0181-5

Cited by 11 publications

(4 citation statements)

References 7 publications

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“…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%

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

Wei

Liu

et al. 2021

Syst Rev

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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.

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Section: Discussionmentioning

confidence: 77%

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

Wei

Liu

et al. 2021

Syst Rev

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“…RDD typically presents as a solitary extra-axial or dura-based, homogeneously enhancing, well-circumscribed mass with iso-intense to central neural structures in T1-weighted imaging (WI) and hypo-or iso-intense signals in T2-WI. Reports of intracranial RDD often highlight heterogenicity with low-intensity T2-WI 22) , supporting the notion of macrophage-produced free radicals during their lifecycle 7) . The MRI findings in our cases adhered to typical features, such as enhancing patterns and dura-based growth.…”

Section: Discussionmentioning

confidence: 84%

Diagnostic Challenges of Rosai-Dorfman Disease Presenting as Pachymeningitis and Meningiomatosis: A Report of Two Unusual Cases Involving the Spinal Cord

Park,

Lee,

Kim

et al. 2023

Nerve

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Rosai-Dorfman disease (RDD) is an exceptionally rare medical condition, with spinal cord involvement even more infrequent. It is frequently misidentified as another malignancy or infectious disease. In this report, we present two cases of RDD with spinal cord involvement. Each patient presented with spinal intradural extramedullary masses within the thoracic and cervical levels, respectively. The differential diagnoses remained uncertain, with the potential for pachymeningitis and meningiomatosis due to the shared nature of nonspecific imaging findings and clinical symptoms in both conditions. Given the presence of neurologic symptoms and spinal cord compression, the individual lesions were surgically excised. Subsequent pathological analysis definitively confirmed the diagnosis of RDD. As an adjunctive therapeutic measure, both patients were orally administered prednisolone. Both patients exhibited favorable neurologic improvement. These cases highlight the diagnostic challenges of RDD when solely relying on imaging studies. Surgical intervention, in conjunction with a meticulous pathologic examination, is imperative for a precise diagnosis and guides appropriate treatment strategies. The multifaceted spatial manifestation of this disease further underscores the potential for multifocal occurrences within the central nervous system, providing an avenue for improved comprehension of the underlying mechanisms contributing to its dissemination.

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“… 4 As per the World Health Organization classification of tumors, these lesions are categorized as a reactive condition of unknown etiology. 5 Extranodal RDD accompanied by lymphadenopathy is seen in almost half of the cases; however, extranodal manifestation of RDD in the absence of lymphadenopathy is extremely rare. 6 The skin, respiratory tract, orbital cavity, and the central nervous system are the main extranodal sites involved, followed by the skeleton.…”

Section: Discussionmentioning

confidence: 99%

“… 10 Simply put, skeletal involvement of RDD is seldom fatal, and the long-term prognosis is good for isolated extranodal lesion. 5 , 6 …”

Section: Discussionmentioning

confidence: 99%

Extranodal Rosai–Dorfman Disease as Isolated Lesion of the Tibia Diagnosed by Fine-Needle Aspiration Cytology

Liu

Wang

et al. 2015

Medicine

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Few studies have used fine-needle aspiration cytology for the purpose of isolated skeletal Rosai–Dorfman diseases (RDDs) diagnosis.Herein, we described an extremely rare case of a 56-year-old woman who presented to our hospital with an insidious onset of pain in the right proximal tibia.The provisional cytologic diagnosis of RDDs was confirmed by a computer tomography-guided core needle biopsy of the lesion. Subsequently, curettage and autogenous iliac crest bone graft were performed successfully. At the 4th year of follow-up her clinical symptoms disappeared, and there was no clinical evidence of lesion recurrence.Our case highlighted the role of fine-needle aspiration cytology with immunohistochemical studies in the diagnosis of RDDs and the characteristic features of isolated skeletal RDDs in an unusual location, the knowledge of which would help avoid missed or delayed diagnosis in the future.

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Isolated spinal Rosai–Dorfman disease misdiagnosed as lymphoplasmacyte-rich meningioma by intraoperative histological examination

Cited by 11 publications

References 7 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

Diagnostic Challenges of Rosai-Dorfman Disease Presenting as Pachymeningitis and Meningiomatosis: A Report of Two Unusual Cases Involving the Spinal Cord

Extranodal Rosai–Dorfman Disease as Isolated Lesion of the Tibia Diagnosed by Fine-Needle Aspiration Cytology

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