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Radiological and clinical findings of isolated meningeal Rosai–Dorfman disease of the central nervous system

Wen, Jia-Hua, MDa; Wang, Chao, MDa; Jin, Yun-Yun, MDa; Xu, Duo, MDa; Jiang, Biao, MDa; He, Xiao-Juan, MDb; Min, Jie, MDa,*

Section Editor(s): Kumar., Manoj

doi: 10.1097/MD.0000000000015365
Research Article: Observational Study
Open

Rosai–Dorfman disease (RDD) with isolated central nervous system (CNS) involvement is an extremely rare disease. Most RDD of the CNS present as dural-based mass mimicking meningioma and other common lesions, which makes preoperative accurate diagnosis of great difficulty. We searched the pathology database in our hospital and 3 cases of RDD with isolated CNS involvement were finally included in our study. Radiological and clinical findings of these three cases were retrospectively analyzed. The lesions of 2 cases were dura-based against the cerebral convexity, presenting as a sheet-shaped thickened dura mater, another case was located just across the cerebral falx, the dural display in the center was intact. The 3 cases showed low signal intensity on T2-weighted image, obviously enhanced, significantly surrounding edema and finger-like protuberance but no invasion of the brain parenchyma or no sign of hyperplasia or sclerosis of the surrounding cranial bones. In conclusion, when we come across a disease that mimicking meningioma, especially when it manifests as the above radiological features, we should considered it might be a kind of proliferative disease of the meninges, such as RDD.

aDepartment of Radiology

bDepartment of Pathology, the Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.

Correspondence: Jie Min, Department of Radiology, the Second Affiliated Hospital, Zhejiang University School of Medicine, No. 88 Jiefang Road, Hangzhou 310009, China (e-mail: 396517680@qq.com).

Abbreviations: CNS = central nervous system, FOV = field of view, RDD = Rosai–Dorfman disease, T1-WI = T1 weighted image, T2-WI = T2-weighted image, TE = echo time, TR = repetition time.

The authors have no conflicts of interest to disclose.

This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc/4.0

Received October 25, 2018

Received in revised form March 1, 2019

Accepted March 30, 2019

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1 Introduction

Rosai–Dorfman disease (RDD) was first reported by pathologists Juan Rosai and Ronald Dorfman in 1969,[1] RDD is a rare lymphoproliferative disease typically characterized with massive painless cervical lymphadenopathy, fever, weight loss, and leukocytosis. RDD can involve any nodal or extranodal site, but most commonly presents as massive cervical lymphadenopathy. The incidence of extranodal manifestations of RDD is 43% as reported and usually involved the skin, upper respiratory system, orbits, bones, and endocrine glands. Central nervous system (CNS) involvement has been reported, but it is relatively uncommon.[2–4] RDD of CNS usually manifests as dura-based masses which is difficult for radiologists to make an accurate diagnosis. In this report, we described 3 cases of isolated meningeal RDD with clinical and radiological presentations mimicking meningiomas, and review literature to raise the awareness of this disease to avoid misdiagnosis of meningiomas.

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2 Material and methods

We searched the pathology database at The Second Affiliated Hospital of Zhejiang University School of Medicine from January 2011 to August 2018, and a total of 3 patients with RDD diagnosed were included. This study was approved by the institute review board of Zhenjiang University and informed consent was obtained from the subject. All patients underwent conventional brain MR enhancement scan, with plain scan of T1 and T2 sequences in axial position, coronal, sagittal and enhancement. Images were acquired using a 3.0-T scanner (Discovery MR750, GE Medical Systems) with an 8-channel head coil. Images were acquired using T1-weighted images (T1-WI) (repetition time [TR] = 500 ms, echo time [TE] =120 ms) and T2-weighted image (T2-WI) (TR = 500 ms, TE =15 ms, at the same time with a field of view =260 × 260 mm2, matrix size = 256 × 256, slice thickness = 5 mm). All the 3 patients underwent surgery with total or subtotal resection of the masses. All the histologic sections underwent routine pathological and immunohistochemical examination, the pathological results of case 2 and case 3 were sent to University of California at Los Angeles for consultation. Follow-up after the surgery were performed ranging from 2 months to 14 months, recurrences were observed in 1 case during the follow-up in 14 months.

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3 Results

3.1 Clinical findings

Clinical data of the 3 patients were summarized in Table 1. One woman and 2 men ranging from 40 to 54 years (mean 49.3 years) were involved in this study. Case 1 came to our hospital with a symptom of headache, case 2 was dizziness accompanied by aphasia, and case 3 bore repeated left limb convulsions for more than a year but aggravating in the last month. None of the 3 cases suffered cervical lymphadenopathy or the other extranodal involvement. All the 3 cases demonstrated no unusual results of routine laboratory assays.

Table 1

Table 1

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3.2 Neuroimaging and pathological findings

Of the first 2 cases, Brain MRI showed that the lesions were located extraparenchymal and dura-based against the cerebral convexity (Figs. 1 and 2), and case 3 was located just across the cerebral falx (Fig. 3). The thickened meningeal of the first 2 cases on T1-WI and T2-WI was clear, presenting as hypo to isointense on T1-WI, but T2-WI signal of the thickened meninges decreased significantly. On contrast-enhanced T1-WI, the sheet-shaped irregularly thickened meningeal had a region of a finger-like protuberance (arrow in D in all the 3 cases) extending to the brain parenchyma. The lesion of the case 3 located just across the cerebral falx can be easily identified by T2-WI and T1-WI as a heterogeneous signal, was homogeneously enhanced (Fig. 3). The lesion was symmetrically distributed on the left and the right of the cerebral falx, the dural in the center was intact, but the finger-like protuberance on the left was blurring displayed.

Figure 1

Figure 1

Figure 2

Figure 2

Figure 3

Figure 3

Histolopathological examination showed chronic inflammatory cells emperipolesis but without the destructive phagocytosis of plasma cells or lymphocytes. Immunohistochemical results with an expression of CD68 and S-100 protein but CD1a negative confirmed that it was RDD.

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4 Discussion

Extranodal RDD occurs in 43% of all the cases.[2] The rate of RDD isolated to the CNS is less than 5% as reported.[4–7] Most of the RDD of CNS are dural-based mimicking meningioma, which makes it a challenge for accurate diagnosis.[2] The hallmark of RDD is the characteristic emperipolesis of lymphatic plasma cells with the background of many plasma cells and lymphocytes, the other characteristic is with an expression of CD68 and S-100 protein but CD1a negative. Immunohistochemical stains are generally sufficient to differentiate RDD.[8,9]

As far as we know, there are few articles about imaging to differentiate from meningioma. The lesions of the 3 cases were hypo to isointense on T1-WI and low on T2-WI, possibly reflect their process of inflammatory process. After enhancement, the thickened meningeal is sheet-like in shape with a finger-like protuberance (Figs. 1–3, arrow in D) extending to the brain parenchyma. The lesion of the case 1 and case 2 did not cause cranial hyperplastic sclerosis or bone invasion. We have carefully contrasted the images provided by Huang et al,[10] Zhu et al,[11] the finger-like protuberance also appeared in their cases, this performance may be directly related to the significant edema around the lesion or possible lead to postoperative recurrence. The dura mater in case 3 shared the same imaging features as the case Forest et al[12] reported, which indicates that RDD is a kind of neoplastic state, rarely involving the parenchyma.

RDD and meningioma could show the similar signal intensity on T1 and T2, but when the lesions were inhomogeneous, such as necrotic, cystic, or calcified, most of this chance, it is likely to be meningioma. Most meningiomas are slow-growing benign tumors always board-based to the dura mater with an enhancing “dural tail." RDD often resembles an en plaque meningioma, which is a rare type of meningioma characterized by diffuse and extensive dural involvement, and at times invading the bone. In addition, hyperosteogeny and sclerosis at the site are common and meningioma is well known to invade the bone.[13] Note that bone invasion is more often seen in en plaque meningioma. Apart from meningioma, the differential diagnosis of RDD includes lymphoma, metastasis, and some other dura-based diseases.

In conclusion, when we come across a disease manifesting as dura-based mass, sheet-like in shape, showed a finger-like protuberance, with a significant edema but without invasion of the brain parenchyma or bone invasion, we should consider it might be a kind of proliferative disease of the meninges, such as RDD.

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Author contributions

Data curation: Xiao-juan He.

Methodology: Yun-yun Jin, Duo Xu, Biao Jiang.

Supervision: Jie Min.

Writing – original draft: Jia-hua Wen.

Writing – review & editing: Chao Wang.

Jie Min orcid: 0000-0001-9932-6163.

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References

[1]. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63–70.
[2]. Andriko JA, Morrison A, Colegial CH, et al. Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol 2001;14:172–8.
[3]. Juric G, Jakic-Razumovic J, Rotim K, et al. Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma. Acta Neurochir 2003;145:145–9. discussion 149.
[4]. Luo Z, Zhang Y, Zhao P, et al. Characteristics of Rosai-Dorfman disease primarily involved in the central nervous system: 3 case reports and review of literature. World Neurosurg 2017;97:58–63.
[5]. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 1990;7:19–73.
[6]. Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, et al. Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. Medicine 2014;93:165–75.
[7]. Hong CS, Starke RM, Hays MA, et al. Redefining the prevalence of dural involvement in Rosai-Dorfman disease of the central nervous system. World Neurosurg 2016;90:702.e13–20.
[8]. Prayson RA, Rowe JJ. Dural-based Rosai-Dorfman disease: differential diagnostic considerations. J Clin Neurosci 2014;21:1872–3.
[9]. Dalia S, Sagatys E, Sokol L, et al. Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment. Cancer Control 2014;21:322–7.
[10]. Huang BY, Zong M, Zong WJ, et al. Intracranial Rosai-Dorfman disease. J Clin Neurosci 2016;32:133–6.
[11]. Zhu H, Qiu LH, Dou YF, et al. Imaging characteristics of Rosai-Dorfman disease in the central nervous system. Eur J Radiol 2012;81:1265–72.
[12]. Forest F, N’Guyen AT, Fesselet J, et al. Meningeal Rosai-Dorfman disease mimicking meningioma. Ann Hematol 2014;93:937–40.
[13]. Juarez A, Dominguez-Fernandez I, Santiago-Sanchez-Mateos D, et al. Cutaneous meningioma: extracranial extension from an intracranial tumour. Clin Exp Dermatol 2009;34:e1001–3.
Keywords:

central nervous system; magnetic resonance imaging; meningiomas; Rosai–Dorfman disease

Copyright © 2019 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.