Rosai-Dorfman disease of the cauda equina: illustrative case

William Mangham Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee
Semmes Murphey Clinic, Memphis, Tennessee

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Emal Lesha Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee
Semmes Murphey Clinic, Memphis, Tennessee

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Elsa Nico University of Illinois College of Medicine at Chicago, Chicago, Illinois

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Kaan Yagmurlu Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee
Semmes Murphey Clinic, Memphis, Tennessee

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Christopher P Golembeski Department of Pathology, Baptist Memorial Hospital, Memphis, Tennessee
Baptist Health Sciences University College of Osteopathic Medicine, Memphis, Tennessee; and

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David C Portnoy West Cancer Center, Memphis, Tennessee

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Jason Weaver Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee
Semmes Murphey Clinic, Memphis, Tennessee

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BACKGROUND

Rosai-Dorfman disease (RDD) is a rare, nonmalignant histiocytosis. It typically occurs in lymph nodes, skin, and soft tissues, but numerous reports of central nervous system involvement exist in the literature. The peripheral nervous system has rarely been involved. In this study, the authors present a case of RDD isolated to the cauda equina. The presentation, management, surgical technique, and adjunctive treatment strategy are described.

OBSERVATIONS

A 31-year-old female presented with 6 months of progressive left lower-extremity numbness involving the lateral aspect of the foot and weakness of the left toes. Magnetic resonance imaging of the lumbar spine demonstrated a homogeneously enhancing intradural lesion involving the cauda equina at the L2–3 levels. Histopathology after resection revealed a histiocytic infiltrate, positive for CD68 and S100, and emperipolesis consistent with RDD. No adjuvant therapy was administered, and the patient had full remission at the 1-year follow-up. Only five other cases of intradural RDD lesions of the cauda equina have been reported in the literature.

LESSONS

RDD of the cauda equina is an especially rare and challenging diagnosis that can mimic other dura-based lesions, such as meningiomas. A definitive diagnosis of RDD relies on pathognomonic histopathological and immunohistochemical findings.

ABBREVIATIONS

CNS = central nervous system; CT = computed tomography; LCH = Langerhans cell histiocytosis; MEP = motor evoked potential; MRI = magnetic resonance imaging; RDD = Rosai-Dorfman disease; SSEP = somatosensory evoked potential

BACKGROUND

Rosai-Dorfman disease (RDD) is a rare, nonmalignant histiocytosis. It typically occurs in lymph nodes, skin, and soft tissues, but numerous reports of central nervous system involvement exist in the literature. The peripheral nervous system has rarely been involved. In this study, the authors present a case of RDD isolated to the cauda equina. The presentation, management, surgical technique, and adjunctive treatment strategy are described.

OBSERVATIONS

A 31-year-old female presented with 6 months of progressive left lower-extremity numbness involving the lateral aspect of the foot and weakness of the left toes. Magnetic resonance imaging of the lumbar spine demonstrated a homogeneously enhancing intradural lesion involving the cauda equina at the L2–3 levels. Histopathology after resection revealed a histiocytic infiltrate, positive for CD68 and S100, and emperipolesis consistent with RDD. No adjuvant therapy was administered, and the patient had full remission at the 1-year follow-up. Only five other cases of intradural RDD lesions of the cauda equina have been reported in the literature.

LESSONS

RDD of the cauda equina is an especially rare and challenging diagnosis that can mimic other dura-based lesions, such as meningiomas. A definitive diagnosis of RDD relies on pathognomonic histopathological and immunohistochemical findings.

ABBREVIATIONS

CNS = central nervous system; CT = computed tomography; LCH = Langerhans cell histiocytosis; MEP = motor evoked potential; MRI = magnetic resonance imaging; RDD = Rosai-Dorfman disease; SSEP = somatosensory evoked potential

Rosai-Dorfman disease (RDD) is a benign histiocytic disorder first reported by Destombes1 in 1965 and later distinguished as a unique disease separate from Langerhans’s disease by Rosai and Dorfman2 in 1969. Commonly characterized by profound lymphadenopathy, RDD has an overall prevalence of 1 case in 200,000 persons.3 Although the exact etiology of RDD is mostly unknown, viral infections, autoimmune components, or genetic alterations have been proposed as potential causes.4–10 RDD is subcategorized into nodal and extranodal types, the latter of which is less common.11 Extranodal RDD most commonly affects the skin, soft tissue, nasal and paranasal sinuses, and rarely the central nervous system (CNS) or peripheral nervous system.12,13 Isolated RDD of the CNS is observed in only ≤5% of cases.14 Even rarer is isolated spinal involvement, occurring in 20% to 25% of RDD cases with nervous system disease.15 Spinal RDD primarily affects the cervical and thoracic regions and presents with symptoms related to mass effect, such as myelopathy or radiculopathy, with sensory and/or motor impairment in the limbs.15,16 Radiologically, spinal RDD is homogeneously enhancing and has an affinity for the meninges, appearing and presenting like a meningioma.17–19 Herein, we present a rare variant of RDD of the cauda equina.

Illustrative Case

History and Examination

A 31-year-old female presented with 6 months of progressive left lower-extremity numbness involving the lateral aspect of the foot. She also noted weakness in her left toes, which caused difficulty with walking, but she did not have any loss of bowel and bladder control. She denied any fevers, chills, night sweats, palpable lymphadenopathy, or unexplained weight loss, and laboratory findings were unremarkable. Magnetic resonance imaging (MRI) of the lumbar spine with contrast revealed an intradural lesion involving the cauda equina, centered at the L2 and L3 levels, as demonstrated in Fig. 1. Although there was concern that this lesion was a glioma drop metastasis, complete MRI of the neuraxis and computed tomography (CT) of the chest, abdomen, and pelvis did not reveal other lesions. Her past medical history was remarkable for a subcutaneous left flank cribriform carcinoma, which had been resected 10 years earlier. Because of her progressive symptoms and unclear diagnosis, the decision was made to perform a resection to relieve the patient’s radicular symptoms and obtain a tissue diagnosis.

FIG. 1
FIG. 1

Sagittal gadolinium T1-weighted (A) and T2-weighted (C) MRI sequences demonstrating an avid and homogeneously enhancing intradural lesion (arrows) at the L2–3 level of the spinal cord. Axial gadolinium T1-weighted (B) and T2-weighted (D) MRI sequences demonstrating a contrast-enhancing lesion (arrows) involving multiple nerve rootlets.

Surgical Management and Postoperative Course

The patient underwent resection of the lesion with motor evoked potential (MEP) and somatosensory evoked potential (SSEP) neuromonitoring through a L2–3 laminectomy. The intradural lesion was visible through the dura, and ultrasound was used for further confirmation of the lesion location prior to opening the dura. Upon dural opening, a tan mass was noted to be adherent to a nerve rootlet and had several finger-like projections onto adjacent rootlets in an en plaque manner, as illustrated in Fig. 2A. A nerve stimulator confirmed that the largest mass was not adherent to a motor root, which was then removed by circumferential microdissection followed by coagulating and sharply cutting the involved root proximally and distally. The mass was then rotated, and its en plaque attachment to adjacent roots was sharply divided. Similarly, a second lesion was removed after stimulation did not provoke a motor response. There were several other nerve rootlets with an en plaque appearance that were not directly connected to the main lesion. The stimulator was then used adjacent to the other remaining lesions, which were found to produce a motor response. Therefore, the remaining small lesions were left in place with the plan to defer further management decisions while awaiting pathology. MEP and SSEP recordings remained stable throughout the case, and the patient’s neurological function was stable postoperatively.

FIG. 2
FIG. 2

Intraoperative photograph of the operative field after dural opening, which demonstrates a tan lesion with an en plaque attachment to multiple nerve rootlets of the cauda equina (A). Histopathology on hematoxylin and eosin (H&E) staining (B) shows extranodal accumulation of histiocytes with characteristic pale eosinophilic cytoplasm and round to ovoid nuclei containing open vesicular chromatin and small nucleoli. Nuclear grooves are notably absent. Lesional histiocytes are immunoreactive for S100 (C) and CD68 (D) but negative for CD1a (E). Original magnification ×40 (B) and ×60 (C–E).

The patient had an uneventful postoperative course. At her 2-month follow up, she remained intact neurologically, free of the preoperative radicular symptoms, and free of systemic symptoms, such as palpable lymphadenopathy, skin lesions, or unexplained weight loss. Therefore, no further adjuvant therapy was prescribed. At the 6-month follow-up, the patient remained stable neurologically with no evidence of disease progression. Postoperative MRI of the lumbar spine at the 1-year follow-up showed near-total resection of the lesion with some mild, contrast-enhanced speckling along the nerve roots consistent with RDD, stable to immediate postoperative imaging (Fig. 3). The patient will undergo continued surveillance with imaging.

FIG. 3
FIG. 3

Sagittal and axial gadolinium T1-weighted MRI sequences demonstrating resection of the intradural lesion with minimal residual enhancement at 6 months (A and C) and 1 year (B and D) postoperatively.

Pathology

Histopathological evaluation of the lesion revealed emperipolesis, a diffuse histiocytic infiltrate positive for CD68 and S100, as well as small lymphocytes positive for LCA, CD3 T cells, and CD20 B cells. Furthermore, CD138 and MUM-1 highlighted rare plasma cells, GAFP and EMA were negative, and Ki-67 was not increased (Fig. 2B–E). Taken together, these findings were consistent with a final diagnosis of RDD.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Histocytoses are a heterogeneous group of rare disorders marked by abnormal growth of histiocytes that can infiltrate any tissue but have a predilection for skin, bone, lung, lymph nodes, and the CNS.20 Symptoms from histiocytoses are usually caused by mass effect of the histiocyte tumor mass toward adjacent tissue, as well as a chronic inflammatory response. Historically, histiocytoses have been classified as Langerhans cell histiocytosis (LCH) or non-LCH. However, the development of recent molecular techniques have paved the way for a more recent classification of histiocytoses, which includes five groups (L, R, C, M, and H) based on the clinical, histological, and molecular properties of each condition.20 RDD, also known as “sinus histiocytosis with massive lymphadenopathy,” is self-limited and benign.2 Commonly affecting children and young adults at a mean age of 39 years old and a male to female ratio of 1.8:1.0, RDD is typically marked by painless, bilateral cervical lymphadenopathy or myelopathy related to the mass effect; however, other systemic symptoms can include fever, weight loss, anemia, neutrophilia, and an increased erythrocyte sedimentation rate.21–23 Numerous reports in the literature have discussed CNS involvement of RDD. Intracranially, the lesions can present like space-occupying meningiomas with intradural, extraaxial involvement.19 In the spine, presentation within the lumbar region only occurs in 6% of patients according to a recent review, with the majority of cases presenting in the cervical and thoracic regions.24 Interestingly, although most cases of spinal RDD are intradural, the disease can also be intramedullary in 10% of patients. In our case, the lesion had encased several rootlets of the cauda equina. Although the mechanism of RDD, especially its dissemination into the CNS and spine, remains largely unclear, its presentation makes it challenging to diagnose the lesion based solely on imaging findings. Thus, resection is necessary to make a diagnosis, relieve mass effect, and prevent neurological deterioration.

Observations

In the present case, the patient presented with radicular symptoms and was found to have intradural RDD lesions situated on the nerve roots of the cauda equina without any other systemic manifestations. A literature review showed that this is the sixth reported intradural RDD lesion involving the cauda equina (Table 1).25–29 Eighty-three percent of the cases were female and showed symptoms of myelopathy/radiculopathy, and 67% of cases involved the lumbar levels and 33% the sacral levels. Fifty percent of patients presented with lower back pain or anal pain, and 33% had bowel or bladder incontinence. Only one patient had lymph node involvement or other hallmark RDD features.

TABLE 1

Literature review of reported cases of intradural RDD of the cauda equina

Authors & YearAge (yrs), SexClinical PresentationLymph Node InvolvementOther RDD FeaturesMRI FindingsSpinal LevelsPathologyTreatmentDisease/Clinical Status at Last FU
Ma et al., 20082544, MLBP, progressive numbness/weakness of LEs for 6 mos, bowel & bladder incontinence for 3 mosNoNoIntradural & extramedullary; T1 & T2 hypointense w/ homogeneous enhancementT12–L4Large lymphoplasmacytic areas infiltrated w/ large histiocytic cells, CD68/S100+, CD1−, w/ emperipolesisResectionImproved numbness & motor strength, resolved LBP; no recurrence
de Oliveira et al., 20162650, FProgressive spastic lower-limb paraparesis for 20 daysNoNoIntradural; homogeneous enhancement; adherent to conus medullaris & cauda equinaL1RDDResectionImproved: ambulatory w/ a cane w/o sphincter dysfunction; disease progression
Tripathi et al., 2017277, FBilat LE pain, 0/5 strength in proximal bilat LEsYesNoContrast enhancement of cauda equina nerve roots & pial enhancement surrounding conusL4S100+, CD1a−, emperipolesisSteroids & IVIGComplete symptom resolution; no recurrence
Chhabria et al., 20182819, FSevere LBP, proximal lt LE weakness, asymmetrical LE sensory loss, bladder bowel incontinence, saddle anesthesiaNoAnemia, elevated ESRT2 isointense to hyperintense; involving the cauda equina nerve roots extending up to sacral level w/in spinal canalSacralLymphoplasmacytic infiltrate rich in histiocytes, CD68/S100+, CD1−, w/ emperipolesisResectionComplete remission at FU
Bahauddin et al., 20222952, FAnal & sacral painNoNoIntradural & extramedullary; T1 isointense; STIR hyperintense; contrast enhancing; extrinsic bony erosions in pst vertebral bodiesS1–2RDDResection & steroidsComplete remission at 4-mo FU
Present case31, FLt LE numbness, lt EHL weaknessNoNoIntradural, homogeneously enhancing, involving cauda equinaL2–3Diffuse histiocytic infiltrate, CD68/S100+ w/ emperipolesis; LCA/CD3 T-cell/CD20 B-cell+; GAFP/EMA−ResectionComplete remission at 1-yr FU

EHL = extensor hallucis longus; ESR = erythrocyte sedimentation rate; FU = follow-up; IVIG = intravenous immunoglobulin; LBP = low-back pain; LE = lower extremity; pst = posterior; STIR = short tau inversion recovery.

Radiological Findings

Spinal RDD is challenging to differentiate from other dura-based lesions, such as meningiomas, lymphomas, metastases, and plasma cell granulomas, based on radiological imaging alone (Table 2).17–19,30,31 However, a dural tail and calcification favor meningioma.32 Of the RDD cauda equina cases from our literature review that commented on MRI contrast of the lesion, all showed contrast enhancement (Table 1).25–27,29 However, Ma et al.25 demonstrated an RDD lesion with T1 hypointensity, whereas Chhabria et al.28 and Bahauddin et al.29 demonstrated RDD lesions with iso- and hyperintensity on T2-weighted imaging, respectively. Nevertheless, it is important to note that RDD tends to invade multiple organs, with a higher recurrence rate of 22.2% and 6.7% in multiorgan and isolated spinal lesions, respectively. Therefore, positron emission tomography or CT scans are strongly recommended to rule out systemic lesions.2,33

TABLE 2

MRI findings of spinal RDD versus other spinal dura-based lesions

Dura-Based LesionT1T2Enhancement
RDDIso- or hyperintenseHypointenseYes–homogeneous
MeningiomaIso- or hyperintenseYes–homogeneous
LymphomaIsointenseIsointense
MetastasesHypointenseHyperintense
Plasma cell granulomaYes–heterogeneous

Histopathological Findings

In a recent systematic review of 47 RDD cases involving the spine,24 93.6% were misdiagnosed preoperatively. Classic histopathological findings of RDD include infiltrates of lymphocytes, plasma cells, and large, pale histiocytes.34,35 Emperipolesis, or lymphophagocytosis, is frequently seen and pathognomonic for RDD.4 Furthermore, RDD histiocytes are immunoreactive for CD68 and S100 and negative for EMA and CD1a.36 Immunoreactivity for S100 differentiates RDD from granulomatous diseases, whereas negative expression of CD1a removes LCH from the differential diagnosis.37

Clinical Management

There is no standard approach to treating RDD; thus, treatment decisions should consider clinical features such as the distribution of lesions and symptoms.4,37 Frequently, if the patient is asymptomatic and there is little risk of end-organ damage, observation is appropriate, as many cases will resolve spontaneously. In one series, 40 of 80 patients with RDD did not require treatment, and 33 of those patients had spontaneous complete remission of the disease.38 Resection is the preferred option for interventional treatment and is appropriate when there is a unifocal extranodal disease for the debulking of cranial, spinal, sinus, or airway disease or other lesions that can compromise organ function.39,40 Total resection is preferred over subtotal resection because of a lower likelihood of recurrence.24 In our literature review of cases with RDD involving the cauda equina, for instance, resection was performed in 83% of patients. Disease progression was noted in only one patient postsurgically.

Adjuvant therapies, including radiation, corticosteroids, and chemotherapy, should be considered for unresectable, recurrent, or systemic RDD.3 Radiation therapy is particularly useful in progressive or recurrent disease.24 Chemotherapy is typically reserved for multifocal, refractory, or life-threatening systemic disease; thus, it was not a suitable option for the present case of unifocal disease with no systemic manifestations. Moreover, since the present case did not show signs of systemic or recurrent disease, a watchful waiting strategy was appropriate over adjuvant therapy. If the lesion enlarges on surveillance MRI or if the patient develops recurrent symptoms, radiation therapy can be considered next. There is no standard recommendation for radiation therapy, although typically, doses between 20 and 30 Gy are used. However, some authors recommend doses higher than 50 Gy; at least one report showed complete relief of symptoms with as little as 10 Gy.12

Systemic therapy options include corticosteroids, cytotoxic chemotherapy, immunomodulatory agents, and MEK inhibitors. Given the rarity of RDD, experience with systemic therapy comes from case reports and retrospective series. Steroids have been shown to induce responses when used both as a single agent and in combination with chemotherapy, although responses are variable and may not be durable.41 In our review, one patient underwent treatment with intravenous immunoglobulin, and one patient was placed on steroids postoperatively.

Response rates to cytotoxic chemotherapy are overall low; however, durable responses to combination regimens containing vinca alkaloids, such as prednisone, 6-mercaptopurine, methotrexate, and vinblastine, have been observed.41,42 Purine analogs are efficacious in other histiocytic disorders and effective in RDD. In a case series from MD Anderson, five patients with RDD were treated with cladribine, with two complete and two partial responses. The fifth patient had stable disease after two cycles of cladribine and then had a prolonged partial response to clofarabine. An additional patient, who had progression after an initial response to cladribine, also had a prolonged response to clofarabine.43 Purine analogs can cause significant myelosuppression, suggesting that they be reserved for severe or refractory cases.4 The immunomodulatory drugs lenalidomide and thalidomide have also been reported to induce responses in RDD. Lenalidomide, with or without steroids, has been shown to induce durable responses with a better side-effect profile than thalidomide.44 Seven patients were treated with rituximab, and 64% remained progression free after 24 months.

Unlike in Erdheim-Chester disease and LCH, BRAF V600E mutations are not frequently found in RDD.45 However, mutations in the MAPK pathway, mainly in the KRAS and MEK genes, were found in 35% to 40% of patients with RDD. In a retrospective review of patients treated at the Mayo Clinic and the University of Alabama, 16 patients with extranodal RDD were treated with the MEK inhibitor cobimetinib.44 Among these 16 patients, the response rate was 63%, with 5 complete responses and 5 partial responses. Responses were durable, with a progression-free survival rate of 65%. Mutations in the KRAS or MEK genes were associated with a higher response rate (88% vs 38%; p = 0.03) and 1-year progression-free survival (100% vs 29%; p < 0.001).

Last, there are no studies evaluating the role of adjuvant therapy following resection. Given that some lesions will not progress, some may eventually spontaneously regress. In our case, the patient’s symptoms improved with surgery; therefore, we believed that observation after surgery was appropriate. If the lesion had progressed, the first step would be restaging studies. If the progression had localized to the spine only, radiation would be our treatment option. In the case of systemic progression, our first option would be systemic therapy with an MEK inhibitor.

Lessons

RDD of the cauda equina is a rare and challenging diagnosis that can mimic other dura-based lesions, such as meningiomas. Although both RDD and meningiomas can appear iso- or hypointense on T1-weighted imaging and demonstrate homogeneous contrast enhancement, only meningiomas have a characteristic dural tail or signs of calcification. A definitive diagnosis of RDD depends on histopathological and immunohistochemical confirmation. Resection is the preferred interventional approach to unifocal RDD and leads to symptom improvement and disease control in cases involving the cauda equina. Radiation therapy is appropriate for unresectable or recurrent lesions, and chemotherapy for multisystemic variants.

Author Contributions

Conception and design: Lesha, Mangham, Weaver. Acquisition of data: Lesha, Mangham, Nico, Portnoy, Weaver. Analysis and interpretation of data: Lesha, Nico, Portnoy. Drafting the article: Lesha, Mangham, Nico, Golembeski, Portnoy. Critically revising the article: Lesha, Mangham, Portnoy, Weaver. Reviewed submitted version of manuscript: Lesha, Mangham, Nico, Yagmurlu, Weaver. Approved the final version of the manuscript on behalf of all authors: Lesha. Statistical analysis: Lesha. Administrative/technical/material support: Mangham, Yagmurlu. Study supervision: Lesha, Mangham, Yagmurlu, Weaver. Photomicrographs: Golembeski.

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    Goyal G, Young JR, Koster MJ, et al. The Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease. Mayo Clin Proc. 2019;94(10):20542071.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Mirra SS, Tindall SC, Check IJ, Brynes RK, Moore WW Inflammatory meningeal masses of unexplained origin. An ultrastructural and immunological study. J Neuropathol Exp Neurol. 1983;42(4):453468.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 35

    Cohen Aubart F, Idbaih A, Emile JF, et al. Histiocytosis and the nervous system: from diagnosis to targeted therapies. Neuro Oncol. 2021;23(9):14331446.

  • 36

    Huang BY, Zhang H, Zong WJ, Sun YH Rosai-Dorfman disease of rare isolated spinal involvement: report of 4 cases and literature review. World Neurosurg. 2016;85:367.e11-6.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 37

    Zhu Q, Liang Y, Fan Z, et al. Management of central nervous system Rosai-Dorfman disease: A single center treatment experience. J Clin Neurosci. 2022;99:275281.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 38

    Pulsoni A, Anghel G, Falcucci P, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol. 2002;69(1):6771.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 39

    Kayali H, Onguru O, Erdogan E, Sirin S, Timurkaynak E Isolated intracranial Rosai-Dorfman disease mimicking meningioma. Clin Neuropathol. 2004;23(5):204208.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 40

    Griffiths SJ, Tang W, Parameswaran R, Kelsey A, West CG Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. Br J Neurosurg. 2004;18(3):293297.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    McPherson CM, Brown J, Kim AW, DeMonte F Regression of intracranial rosai-dorfman disease following corticosteroid therapy. Case report. J Neurosurg. 2006;104(5):840844.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 42

    Jabali Y, Smrcka V, Pradna J Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report. Int J Surg Pathol. 2005;13(3):285289.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Sathyanarayanan V, Issa A, Pinto R, et al. Rosai-Dorfman disease: The MD Anderson Cancer Center experience. Clin Lymphoma Myeloma Leuk. 2019;19(11):709714.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 44

    Liu T, Cao X Successful treatment of multisystemic Rosai-Dorfman disease with lenalidomide and dexamethasone: a case report. Ann Hematol. 2022;101(6):13511353.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 45

    Abeykoon JP, Rech KL, Young JR, et al. Outcomes after treatment with cobimetinib in patients with Rosai-Dorfman disease based on KRAS and MEK alteration status. JAMA Oncol. 2022;8(12):18161820.

    • PubMed
    • Search Google Scholar
    • Export Citation
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  • FIG. 1

    Sagittal gadolinium T1-weighted (A) and T2-weighted (C) MRI sequences demonstrating an avid and homogeneously enhancing intradural lesion (arrows) at the L2–3 level of the spinal cord. Axial gadolinium T1-weighted (B) and T2-weighted (D) MRI sequences demonstrating a contrast-enhancing lesion (arrows) involving multiple nerve rootlets.

  • FIG. 2

    Intraoperative photograph of the operative field after dural opening, which demonstrates a tan lesion with an en plaque attachment to multiple nerve rootlets of the cauda equina (A). Histopathology on hematoxylin and eosin (H&E) staining (B) shows extranodal accumulation of histiocytes with characteristic pale eosinophilic cytoplasm and round to ovoid nuclei containing open vesicular chromatin and small nucleoli. Nuclear grooves are notably absent. Lesional histiocytes are immunoreactive for S100 (C) and CD68 (D) but negative for CD1a (E). Original magnification ×40 (B) and ×60 (C–E).

  • FIG. 3

    Sagittal and axial gadolinium T1-weighted MRI sequences demonstrating resection of the intradural lesion with minimal residual enhancement at 6 months (A and C) and 1 year (B and D) postoperatively.

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    Johnston JM, Limbrick DD, Ray WZ, Brown S, Shimony J, Park TS Isolated cerebellar Rosai-Dorfman granuloma mimicking Lhermitte-Duclos disease. Case report. J Neurosurg Pediatr. 2009;4(2):118120.

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  • 32

    Joshi SS, Joshi S, Muzumdar G, et al. Cranio-spinal Rosai Dorfman disease: case series and literature review. Br J Neurosurg. 2019;33(2):176183.

  • 33

    Goyal G, Young JR, Koster MJ, et al. The Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease. Mayo Clin Proc. 2019;94(10):20542071.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Mirra SS, Tindall SC, Check IJ, Brynes RK, Moore WW Inflammatory meningeal masses of unexplained origin. An ultrastructural and immunological study. J Neuropathol Exp Neurol. 1983;42(4):453468.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 35

    Cohen Aubart F, Idbaih A, Emile JF, et al. Histiocytosis and the nervous system: from diagnosis to targeted therapies. Neuro Oncol. 2021;23(9):14331446.

  • 36

    Huang BY, Zhang H, Zong WJ, Sun YH Rosai-Dorfman disease of rare isolated spinal involvement: report of 4 cases and literature review. World Neurosurg. 2016;85:367.e11-6.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 37

    Zhu Q, Liang Y, Fan Z, et al. Management of central nervous system Rosai-Dorfman disease: A single center treatment experience. J Clin Neurosci. 2022;99:275281.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 38

    Pulsoni A, Anghel G, Falcucci P, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol. 2002;69(1):6771.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 39

    Kayali H, Onguru O, Erdogan E, Sirin S, Timurkaynak E Isolated intracranial Rosai-Dorfman disease mimicking meningioma. Clin Neuropathol. 2004;23(5):204208.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 40

    Griffiths SJ, Tang W, Parameswaran R, Kelsey A, West CG Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. Br J Neurosurg. 2004;18(3):293297.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    McPherson CM, Brown J, Kim AW, DeMonte F Regression of intracranial rosai-dorfman disease following corticosteroid therapy. Case report. J Neurosurg. 2006;104(5):840844.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 42

    Jabali Y, Smrcka V, Pradna J Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report. Int J Surg Pathol. 2005;13(3):285289.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Sathyanarayanan V, Issa A, Pinto R, et al. Rosai-Dorfman disease: The MD Anderson Cancer Center experience. Clin Lymphoma Myeloma Leuk. 2019;19(11):709714.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 44

    Liu T, Cao X Successful treatment of multisystemic Rosai-Dorfman disease with lenalidomide and dexamethasone: a case report. Ann Hematol. 2022;101(6):13511353.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 45

    Abeykoon JP, Rech KL, Young JR, et al. Outcomes after treatment with cobimetinib in patients with Rosai-Dorfman disease based on KRAS and MEK alteration status. JAMA Oncol. 2022;8(12):18161820.

    • PubMed
    • Search Google Scholar
    • Export Citation

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