An Intractable Intra-Axial central nervous system Rosai-Dorfman Disease: A Case Report

An Intractable Intra-Axial central nervous system Rosai-Dorfman Disease: A Case Report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article An Intractable Intra-Axial central nervous system Rosai-Dorfman Disease: A Case Report Akihiro Fujinaga, Hirokazu Sadahiro, Natsumi Fujii, Kazutaka Sugimoto, and 4 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6620676/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that often presents with fever, painless cervical lymphadenopathy and leukemia. Most cases of RDD have a favorable outcome. RDD involving only the central nervous system (CNS) is very rare, and intra-axial CNS RDD is little. We report a case of intra-axial intractable CNS RDD with an atypical presentation. The case reported here is that of a woman in her 60s. Magnetic resonance imaging (MRI) showed a solid tumor 30 mm in diameter in the right frontal lobe. Because the tumor was in the primary motor area, a biopsy was performed for diagnosis. The tumor was ultimately diagnosed as RDD. Although the steroids and cladribine temporarily reduced the size of the tumor, it was ultimately unable to control the growth of the tumor. The patient finally died about two and a half years after the onset. In previous reports, two cases of death directly caused by intra-axial CNS RDD had been reported in the past, because in both cases surgical treatment was not possible. In our case, the tumor was in primary motor area and hard to resect. Total surgical resection is likely to be required to cure intra-axial CNS RDD. Rosai-Dorfman disease intra-axial tumor Cladribine Figures Figure 1 Figure 2 Introduction Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that often presents with fever, painless cervical lymphadenopathy and leukemia [ 1 ]. Most cases of RDD have a favorable outcome: approximately 20% of cases of RDD are asymptomatic with spontaneous remission without treatment; approximately 70% of cases of RDD have mild and recurrent symptoms; approximately 10% of cases of RDD have severe symptoms [ 2 ]. The remaining 10% are potentially serious and should be considered for treatment [ 3 ]. RDD has no established treatment strategy, but steroids, surgical excision, chemotherapy, and radiation therapy may be considered depending on the clinical presentation [ 4 ]. RDD involving only the central nervous system (CNS) is very rare, and the prognosis and treatment are different from those of ordinary RDD [ 5 ]. Although it varies from case to case, CNS RDD often requires medical intervention [ 6 ]. In CNS RDD, and intra-axial CNS RDD is little [ 7 ]. We report a case of intra-axial intractable CNS RDD with an atypical presentation. Clinical Summary The case reported here is that of a woman in her 60s. She presented to her general practitioner for gradual progression of left hemiplegia. She was referred to our department after a detailed examination which revealed a brain tumor. She did not have any major past illnesses, and her family history was unremarkable. On admission, physical examination revealed clear consciousness and left hemiplegia with MMT of 4/5. There were no other significant neurological findings. Blood tests showed no abnormalities in the general examination, tumor markers were negative and various markers such as antinuclear antibodies and autoimmune diseases were also negative. Magnetic resonance imaging (MRI) showed a solid tumor 30 mm in diameter in the right frontal lobe with low intensity on T1-weighted image and iso intensity on T2-weighted image. The tumor was homogeneously contrasted with a gadolinium agent. A wide area around the tumor was high intensity on fluid attenuation inversion recovery (Fig. 1 a-c). Digital Subtraction Angiography did not show any blood vessels feeding the tumor. Systemic computed tomography (CT) did not show any other lesion. Because the tumor was in the primary motor area, a biopsy was performed for diagnosis. The intraoperative findings showed a light grayish, invasive, poorly vascularized and quite solid tumor. The tumor was ultimately diagnosed as RDD by the pathological findings. Figure 2 shows her treatment process. Treatment with steroids was administrated for six months after the biopsy, the tumor had grown with worsening paralysis and spasticity. Cladribine was administrated, the tumor shrank and the edema around the tumor gradually improved. However, 8 months after 4 courses of Cladribine, a rapid worsening of the symptoms was observed; MRI showed an increase in tumor size and a new parietal lesion. The patient was subsequently treated with 40 Gy of radiation for whole brain according to primary central nervus system lymphoma, but the lesion remained unchanged. After radiation, another 4 courses of cladribine were given, but the tumor continued to grow. The patient finally died about two and a half years after the onset. Pathological Findings The pathological findings of the tumor showed a predominantly lymphocytic inflammatory cell with phagocytosis of lymphocytes within the histiocytes, called emperipolesis in Hematoxylin-Eosin (HE) (Fig. 1 d). In immunohistochemistry, leukocyte common antigen (LCA) was positive, suggesting a tumor of lymphocytic origin (Fig. 1 e). Both CD1a (Fig. 1 f) and BRAF-V600 were negative. S-100 protein was strongly positive and cyclin-D1 was also positive (Fig. 1 g, h). Discussion This case was an atypical RDD in terms of only in intra-axial CNS and intractable. The typical radiological findings of CNS RDD show dural-based, extra-axial, well-circumscribed masses [ 6 ]. Because intracranial lesions often present with meningioma-like imaging findings, extra-axial RDD should be considered in cases of neoplastic lesions adjacent to the meninges [ 8 ]. The suprasellar region, cerebral convexity, parasagittal region, cavernous sinus, and petroclival region are the structures that are most affected by extra-axial CNS RDD [ 8 ]. CNS RDD are widely confused due to the similarity of their radiological images, so histopathologic and immunohistochemical studies are essential for a definitive diagnosis of RDD [ 6 ]. According to the Histiocyte Society [ 9 ], histiocyte syndromes are classified according to whether the proliferating histiocytes are Langerhans cells and whether they are benign or malignant [ 10 ]. The presence of lymphocyte emperipolesis is mostly characteristic in RDD, and the viable lymphocytes are located in well-defined cytoplasmic vacuoles of intact histiocytes [ 1 , 11 , 12 ]. RDD is a usually benign histiocytosis of the non-Langerhans cell because it is CD1a negative, but S-100 protein positive and cyclin D1 positive [ 13 , 14 ]. In previous reports, there were 29 cases of only intracranial and intra-axial lesions (Table 1 ). More than half of the cases (62%) were female, and the age range was relatively young, from children to those in their 60s (the median age was 40 years old). Lesion sites varied and were located in the cerebrum, cerebellum, brainstem, and ventricles, and there were 9 cases of multiple lesions. The initial presentations were often headache and seizure in addition to neurological findings consistent with the site of the lesion. Unlike typical RDD, all patients were treated. More than half of the patients (59%) had a good outcome with only surgery, and there were no reports of recurrence after total resection (Table 2 ). Some cases (24%) were cured radically by radiation therapy or chemotherapy after subtotal resection or biopsy. Most of the patients who underwent biopsy and adjuvant therapy had lesions that were multiple or located in the basal ganglia and brainstem. When radical surgical resection is difficult, radiation therapy and chemotherapy can be effective [ 6 ]. Some report showed that cladribine was one of the most effective agent for RDD [ 15 , 16 ]. Steroids are effective in reducing the size and symptoms, but their effects are not permanent [ 17 ]. Two cases of death directly caused by intra-axial CNS RDD had been reported in the past, because in both cases the lesions were located in the brainstem and radical surgical treatment was not possible. Total surgical resection is likely to be required to cure intra-axial CNS RDD, as stated in some reports [ 6 ]. Table 1 29 Reported cases of isolated intra-axial Rosai Dorfman disease Case Authors Age/Sex Location Presentation Treatment Outcomes 1 Beros et al. (2011) [ 18 ] 41/M R cerebellum ataxia Total resection 3 years Complete remission 2 Fukushima et al. (2011) [ 19 ] 33/F R frontal headache Total resection 5 months Complete remission 3 Gaetani et al. (2000) [ 20 ] 67/F R cerebellum ataxia Total resection 1 month Complete remission 4 Gui et al. (2014) [ 21 ] 60/M Multiple lesions, R cerebellum, corpus callosum headache, vomiting Total resection 3 months Complete remission 5 Gui et al. (2014) [ 21 ] 54/M L fronto-parietal seizure Total resection 3 months Complete remission 6 Hong et al. (2016) [ 22 ] 59/F R cerebellum dizziness, blurred vision, falls, seizure Total resection 2 months Complete remission 7 Johnston et al. (2009) [ 23 ] 14/M R cerebellum headache, emesis, ataxia, seizure Total resection 12 months Complete remission 8 Joshi et al. (2019) [ 24 ] 42/F L occipito-parietal right sided weakness, memory disturbances, urgency of micturition Subtotal resection Marked reduction in the size of the lesion 9 Juric et al. (2003) [ 25 ] 39/M R temporal visual disturbances, vertigo, syncope Total resection 10 months Complete remission 10 Li et al. (2012) [ 26 ] 40/M R occipital headache, psychological disorders Total resection 2 years Complete remission 11 Mahzoni et al. (2012) [ 27 ] 33/M L temporal headache, ataxia, unconsciousness Total resection 14 months Alive 12 Morandi et al. (2000) [ 28 ] 22/F Floor of fourth ventricle diplopia Total resection 3 years Complete remission 13 Natarajan et al. (2000) [ 29 ] 45/F R frontal seizure Total resection 5 months Complete remission 14 Patwardhan et al. (2018) [ 30 ] 40/F intraventricular headache Total resection Asymptomatic 15 Sundaram et al. (2005) [ 31 ] 35/M Multiple lesions, bilateral parietal, occipital headache Total resection of R parietal lesion 21.5 years Asymptomatic 16 Varan et al. (2015) [ 32 ] 5/M dorsal pons diplopia, L ptosis, ataxia Subtotal resection 3 months Asymptomatic, resolution of the lesion 17 Zhu et al. (2013) [ 33 ] 4/M Multiple lesions, bilateral cerebral and cerebellar seizure Surgery 8 months Stable condition 18 Camp et al. (2012) [ 34 ] 31/F Multiple lesions, L frontal, R frontal, R parietal seizure Total resection of L frontal lesion + steroid therapy 1 year Complete remission 19 Ludemann et al. (2015) [ 35 ] 2/F Multiple lesions, both frontal lobes , intraventricular leg weakness, fever Steroid therapy + followed subtotal resection 16 months Asymptomatic 20 Symss et al. (2010) [ 36 ] 17/F Multiple lesions, subfrontal lobe extending into suprasellar region, L temporal headache, emesis, amenorrhea, weight gain, memory lapses, decreased visual acuity Subtotal resection of subfrontal lesion + radiation therapy 2 years Asymptomatic, resolution of the lesion 21 Bing et al. (2009) [ 37 ] 32/F L parieto-occipital seizure Biopsy + steroid therapy 5 years Complete remission 22 Chivukula et al. (2015) [ 38 ] 66/F hypothalamus headache, diplopia, confusion, bilateral partial abducens Biopsy + radiation therapy + steroid therapy 19 months Complete remission 23 El Majodoub et al. (2009) [ 39 ] 10/F L white matter and basal ganglia headache, nausea, vomiting, weight loss, dizziness Biopsy + radiation therapy + steroid therapy 49 months Complete remission 24 Miletic et al. (2008) [ 40 ] 8/F L insula and thalamus symptoms of raised intracranial pressure Biopsy + steroid therapy + radiation therapy 3 years Complete remission 25 Imada et al. (2015) [ 41 ] 68/F brainstem visual disturbances, respiratory failure Steroid therapy 3 years Death 26 Richardson et al. (2018) [ 42 ] 64/F Multiple lesions, brainstem, R cerebellar, multiple cerebral double vision Biopsy + cladribine 2 years Death 27 Present case 6x/F Multiple lesions, R frontal L hemiplegia Biopsy + steroid therapy + radiation therapy + cladribine 30 months Death 28 Purav et al. (2005) [ 43 ] 50/M Multiple lesions, location unspecified spastic hemiparesis, mild dysphasia Biopsy R parietal lesion 10 days Death from cardiac disease 29 Deshayes et al. (2013) [ 44 ] 51/F Floor of third ventricle memory disturbance Biopsy Not reported Table 2 Outcomes and treatment of isolated intra-axial Rosai Dorfman disease Outcomes Treatment Case Number (%) controlled Resection alone 1–17 17 (59%) Subtotal resection + additional therapy 18–20 3 (10%) Biopsy + additional therapy 21–24 4 (14%) intractable Death of unresectable lesions + additional therapy 25–27 3 (10%) others Death of another cause 28 1 (3%) Not reported 29 1 (3%) In our case, the tumor was in primary motor area and hard to resect. The first 4 courses of cladribine were temporally effective, however the residual regrowing lesions were treated with steroids, radiotherapy and additional cladribine, but to no avail. It may have been necessary to consider total resection when the tumor began to grow again even though it was in the primary motor area. Since intra-axial CNS RDD itself has rarely been reported, further study is needed for its treatment. Declarations Acknowledgements None. Ethics declarations All procedures performed in studies were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Conflict of interest The authors declare no conflicts of interest. Ethical approval The patient family gave informed consent for publication. References Rosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 87:63-70 La Barge DV 3rd, Salzman KL, Harnsberger HR et al (2008) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): imaging manifestations in the head and neck. AJR Am J Roentgenol 191:W299-306 Bruce-Brand C, Schneider JW, Schubert P (2020) Rosai-Dorfman disease: an overview. J Clin Pathol 73:697-705 Sathyanarayanan V, Issa A, Pinto R et al (2019) Rosai-Dorfman Disease: The MD Anderson Cancer Center Experience. 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Brain Pathol 18:598-601 Imada H, Sakatani T, Sawada M et al (2015) A lethal intracranial Rosai-Dorfman disease of the brainstem diagnosed at autopsy. Pathol Int 65:549-53 Richardson TE, Wachsmann M, Oliver D et al (2018) BRAF mutation leading to central nervous system rosai-dorfman disease. Ann Neurol 84:147-52 Purav P, Ganapathy K, Mallikarjuna VS et al (2005) Rosai-Dorfman disease of the central nervous system. J Clin Neurosci 12:656-9 Deshayes E, Le Berre JP, Jouanneau E et al (2013) 18F-FDG PET/CT findings in a patient with isolated intracranial Rosai-Dorfman disease. Clin Nucl Med 38:e50-2 Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-6620676","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":465973487,"identity":"d46aeb79-482f-4e23-8878-65f6240ee392","order_by":0,"name":"Akihiro Fujinaga","email":"data:image/png;base64,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","orcid":"https://orcid.org/0009-0000-6600-7991","institution":"Department of Neurosurgery, Yamaguchi University School of Medicine","correspondingAuthor":true,"prefix":"","firstName":"Akihiro","middleName":"","lastName":"Fujinaga","suffix":""},{"id":465973488,"identity":"6409d8ec-d47b-49a7-ba80-b56ecb4e797f","order_by":1,"name":"Hirokazu Sadahiro","email":"","orcid":"","institution":"Department of Neurosurgery, Yamaguchi University School of Medicine","correspondingAuthor":false,"prefix":"","firstName":"Hirokazu","middleName":"","lastName":"Sadahiro","suffix":""},{"id":465973489,"identity":"7678ec20-b7e1-431d-92a1-744c466f3df0","order_by":2,"name":"Natsumi Fujii","email":"","orcid":"","institution":"Department of Neurosurgery, Yamaguchi University School of Medicine","correspondingAuthor":false,"prefix":"","firstName":"Natsumi","middleName":"","lastName":"Fujii","suffix":""},{"id":465973490,"identity":"fb1b13c9-e229-470a-980a-6cc3f2f83a2b","order_by":3,"name":"Kazutaka Sugimoto","email":"","orcid":"","institution":"Department of Neurosurgery, Yamaguchi University School of medicine","correspondingAuthor":false,"prefix":"","firstName":"Kazutaka","middleName":"","lastName":"Sugimoto","suffix":""},{"id":465973491,"identity":"5a88bd6d-51e5-4ffa-970b-138e6feb976d","order_by":4,"name":"Sadahiro Nomura","email":"","orcid":"","institution":"Department of Neurosurgery, Yamaguchi University School of Medicine","correspondingAuthor":false,"prefix":"","firstName":"Sadahiro","middleName":"","lastName":"Nomura","suffix":""},{"id":465973492,"identity":"4e44215d-0aec-41f8-9aac-95f79674681b","order_by":5,"name":"Yoshinobu Hoshii","email":"","orcid":"","institution":"Department of Pathology, Yamaguchi University School of Medicine","correspondingAuthor":false,"prefix":"","firstName":"Yoshinobu","middleName":"","lastName":"Hoshii","suffix":""},{"id":465973493,"identity":"1711ee05-a863-4e97-8803-904bbf548960","order_by":6,"name":"Takanori Hirose","email":"","orcid":"","institution":"Department of Pathology, Harima-Himeji General Medical Center","correspondingAuthor":false,"prefix":"","firstName":"Takanori","middleName":"","lastName":"Hirose","suffix":""},{"id":465973494,"identity":"de82bb96-0001-49ad-99f7-62db4976ee7b","order_by":7,"name":"Hideyuki Ishihara","email":"","orcid":"","institution":"Department of Neurosurgery, Yamaguchi University School of medicine","correspondingAuthor":false,"prefix":"","firstName":"Hideyuki","middleName":"","lastName":"Ishihara","suffix":""}],"badges":[],"createdAt":"2025-05-08 12:29:41","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6620676/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6620676/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":84214146,"identity":"56123fe7-c51a-4a43-bd97-e9036a033d81","added_by":"auto","created_at":"2025-06-09 10:25:39","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":10338831,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003ea\u003c/strong\u003e A mass lesion approximately 3 cm in size was observed in the right frontal lobe with low intensity on T1-weighted image. \u003cstrong\u003eb\u003c/strong\u003e On enhanced T1-weighted images, the tumor was homogeneously high intensity. \u003cstrong\u003ec\u003c/strong\u003e A wide area around the tumor was high intensity on fluid attenuation inversion recovery (FLAIR). \u003cstrong\u003ed\u003c/strong\u003eHematoxylin-Eosin (HE) staining showed a predominant infiltrate of inflammatory cells, mainly lymphocytes, with no clear tumor cells. There was also phagocytosis of lymphocytes within the histiocytes, called emperipolesis (arrow). \u003cstrong\u003ee\u003c/strong\u003e Leukocyte common antigen (LCA) was positive. \u003cstrong\u003ef\u003c/strong\u003e CD1a was negative and Langerhans histiocytosis was negative. \u003cstrong\u003eg \u003c/strong\u003eS-100 protein was strongly positive, \u003cstrong\u003eh\u003c/strong\u003e and cyclin-D1 was positive\u003c/p\u003e","description":"","filename":"Fig1..png","url":"https://assets-eu.researchsquare.com/files/rs-6620676/v1/6dfdcaed65e11818f31153e0.png"},{"id":84214142,"identity":"e43e3005-444a-47dd-ac58-cc2acb596131","added_by":"auto","created_at":"2025-06-09 10:25:39","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":2208828,"visible":true,"origin":"","legend":"\u003cp\u003eTable summarizing the treatment progress and tumor size. The tumor size is compared using enhanced T1-weighted images. The steroid used was betamethasone, and the dosage was increased or decreased as necessary. Cladribine had a temporary effect, but the tumor gradually worsened\u003c/p\u003e","description":"","filename":"Fig2..png","url":"https://assets-eu.researchsquare.com/files/rs-6620676/v1/4c65bc7bddc86b2b5f52ab60.png"},{"id":85152840,"identity":"b1aacdcc-739d-41c9-9d67-b289de89002c","added_by":"auto","created_at":"2025-06-22 16:49:46","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":23019386,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6620676/v1/0f14df36-4989-41f1-af33-63ae7fc3616f.pdf"}],"financialInterests":"","formattedTitle":"An Intractable Intra-Axial central nervous system Rosai-Dorfman Disease: A Case Report","fulltext":[{"header":"Introduction","content":"\u003cp\u003eRosai-Dorfman disease (RDD) is a rare histiocytic disorder that often presents with fever, painless cervical lymphadenopathy and leukemia [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Most cases of RDD have a favorable outcome: approximately 20% of cases of RDD are asymptomatic with spontaneous remission without treatment; approximately 70% of cases of RDD have mild and recurrent symptoms; approximately 10% of cases of RDD have severe symptoms [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. The remaining 10% are potentially serious and should be considered for treatment [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. RDD has no established treatment strategy, but steroids, surgical excision, chemotherapy, and radiation therapy may be considered depending on the clinical presentation [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. RDD involving only the central nervous system (CNS) is very rare, and the prognosis and treatment are different from those of ordinary RDD [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Although it varies from case to case, CNS RDD often requires medical intervention [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. In CNS RDD, and intra-axial CNS RDD is little [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. We report a case of intra-axial intractable CNS RDD with an atypical presentation.\u003c/p\u003e"},{"header":"Clinical Summary","content":"\u003cp\u003eThe case reported here is that of a woman in her 60s. She presented to her general practitioner for gradual progression of left hemiplegia. She was referred to our department after a detailed examination which revealed a brain tumor. She did not have any major past illnesses, and her family history was unremarkable. On admission, physical examination revealed clear consciousness and left hemiplegia with MMT of 4/5. There were no other significant neurological findings. Blood tests showed no abnormalities in the general examination, tumor markers were negative and various markers such as antinuclear antibodies and autoimmune diseases were also negative.\u003c/p\u003e \u003cp\u003eMagnetic resonance imaging (MRI) showed a solid tumor 30 mm in diameter in the right frontal lobe with low intensity on T1-weighted image and iso intensity on T2-weighted image. The tumor was homogeneously contrasted with a gadolinium agent. A wide area around the tumor was high intensity on fluid attenuation inversion recovery (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003ea-c). Digital Subtraction Angiography did not show any blood vessels feeding the tumor. Systemic computed tomography (CT) did not show any other lesion.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eBecause the tumor was in the primary motor area, a biopsy was performed for diagnosis. The intraoperative findings showed a light grayish, invasive, poorly vascularized and quite solid tumor. The tumor was ultimately diagnosed as RDD by the pathological findings.\u003c/p\u003e \u003cp\u003eFigure \u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e shows her treatment process. Treatment with steroids was administrated for six months after the biopsy, the tumor had grown with worsening paralysis and spasticity. Cladribine was administrated, the tumor shrank and the edema around the tumor gradually improved. However, 8 months after 4 courses of Cladribine, a rapid worsening of the symptoms was observed; MRI showed an increase in tumor size and a new parietal lesion. The patient was subsequently treated with 40 Gy of radiation for whole brain according to primary central nervus system lymphoma, but the lesion remained unchanged. After radiation, another 4 courses of cladribine were given, but the tumor continued to grow. The patient finally died about two and a half years after the onset.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003ePathological Findings\u003c/h2\u003e \u003cp\u003eThe pathological findings of the tumor showed a predominantly lymphocytic inflammatory cell with phagocytosis of lymphocytes within the histiocytes, called emperipolesis in Hematoxylin-Eosin (HE) (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003ed). In immunohistochemistry, leukocyte common antigen (LCA) was positive, suggesting a tumor of lymphocytic origin (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003ee). Both CD1a (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003ef) and BRAF-V600 were negative. S-100 protein was strongly positive and cyclin-D1 was also positive (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eg, h).\u003c/p\u003e \u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003eThis case was an atypical RDD in terms of only in intra-axial CNS and intractable. The typical radiological findings of CNS RDD show dural-based, extra-axial, well-circumscribed masses [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Because intracranial lesions often present with meningioma-like imaging findings, extra-axial RDD should be considered in cases of neoplastic lesions adjacent to the meninges [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. The suprasellar region, cerebral convexity, parasagittal region, cavernous sinus, and petroclival region are the structures that are most affected by extra-axial CNS RDD [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eCNS RDD are widely confused due to the similarity of their radiological images, so histopathologic and immunohistochemical studies are essential for a definitive diagnosis of RDD [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. According to the Histiocyte Society [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e], histiocyte syndromes are classified according to whether the proliferating histiocytes are Langerhans cells and whether they are benign or malignant [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. The presence of lymphocyte emperipolesis is mostly characteristic in RDD, and the viable lymphocytes are located in well-defined cytoplasmic vacuoles of intact histiocytes [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e, \u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. RDD is a usually benign histiocytosis of the non-Langerhans cell because it is CD1a negative, but S-100 protein positive and cyclin D1 positive [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e, \u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn previous reports, there were 29 cases of only intracranial and intra-axial lesions (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). More than half of the cases (62%) were female, and the age range was relatively young, from children to those in their 60s (the median age was 40 years old). Lesion sites varied and were located in the cerebrum, cerebellum, brainstem, and ventricles, and there were 9 cases of multiple lesions. The initial presentations were often headache and seizure in addition to neurological findings consistent with the site of the lesion. Unlike typical RDD, all patients were treated. More than half of the patients (59%) had a good outcome with only surgery, and there were no reports of recurrence after total resection (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). Some cases (24%) were cured radically by radiation therapy or chemotherapy after subtotal resection or biopsy. Most of the patients who underwent biopsy and adjuvant therapy had lesions that were multiple or located in the basal ganglia and brainstem. When radical surgical resection is difficult, radiation therapy and chemotherapy can be effective [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Some report showed that cladribine was one of the most effective agent for RDD [\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e, \u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. Steroids are effective in reducing the size and symptoms, but their effects are not permanent [\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. Two cases of death directly caused by intra-axial CNS RDD had been reported in the past, because in both cases the lesions were located in the brainstem and radical surgical treatment was not possible. Total surgical resection is likely to be required to cure intra-axial CNS RDD, as stated in some reports [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003e29 Reported cases of isolated intra-axial Rosai Dorfman disease\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"7\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c7\" colnum=\"7\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCase\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eAuthors\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eAge/Sex\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eLocation\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003ePresentation\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTreatment\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c7\"\u003e \u003cp\u003eOutcomes\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eBeros et al.\u003c/p\u003e \u003cp\u003e(2011) [\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e41/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eR cerebellum\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eataxia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e3 years\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eFukushima et al.\u003c/p\u003e \u003cp\u003e(2011) [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e33/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eR frontal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e5 months\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eGaetani et al.\u003c/p\u003e \u003cp\u003e(2000) [\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e67/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eR cerebellum\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eataxia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e1 month\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eGui et al.\u003c/p\u003e \u003cp\u003e(2014) [\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e60/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMultiple lesions,\u003c/p\u003e \u003cp\u003eR cerebellum, corpus callosum\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache, vomiting\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e3 months\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eGui et al.\u003c/p\u003e \u003cp\u003e(2014) [\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e54/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eL fronto-parietal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eseizure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e3 months\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eHong et al.\u003c/p\u003e \u003cp\u003e(2016) [\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e59/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eR cerebellum\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003edizziness, blurred vision, falls, seizure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e2 months\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eJohnston et al.\u003c/p\u003e \u003cp\u003e(2009) [\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e14/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eR cerebellum\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache, emesis,\u003c/p\u003e \u003cp\u003eataxia, seizure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e12 months\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eJoshi et al.\u003c/p\u003e \u003cp\u003e(2019) [\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e42/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eL occipito-parietal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eright sided weakness, memory disturbances, urgency of micturition\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eSubtotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003eMarked reduction in the size of the lesion\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eJuric et al.\u003c/p\u003e \u003cp\u003e(2003) [\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e39/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eR temporal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003evisual disturbances, vertigo, syncope\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e10 months\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e10\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eLi et al.\u003c/p\u003e \u003cp\u003e(2012) [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e40/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eR occipital\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache,\u003c/p\u003e \u003cp\u003epsychological disorders\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e2 years\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e11\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMahzoni et al.\u003c/p\u003e \u003cp\u003e(2012) [\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e33/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eL temporal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache, ataxia, unconsciousness\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e14 months\u003c/p\u003e \u003cp\u003eAlive\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e12\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMorandi et al.\u003c/p\u003e \u003cp\u003e(2000) [\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e22/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eFloor of fourth ventricle\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003ediplopia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e3 years\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e13\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNatarajan et al.\u003c/p\u003e \u003cp\u003e(2000) [\u003cspan citationid=\"CR29\" class=\"CitationRef\"\u003e29\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e45/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eR frontal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eseizure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e5 months\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e14\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003ePatwardhan et al.\u003c/p\u003e \u003cp\u003e(2018) [\u003cspan citationid=\"CR30\" class=\"CitationRef\"\u003e30\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e40/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eintraventricular\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003eAsymptomatic\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e15\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSundaram et al.\u003c/p\u003e \u003cp\u003e(2005) [\u003cspan citationid=\"CR31\" class=\"CitationRef\"\u003e31\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e35/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMultiple lesions,\u003c/p\u003e \u003cp\u003ebilateral parietal,\u003c/p\u003e \u003cp\u003eoccipital\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection of\u003c/p\u003e \u003cp\u003eR parietal lesion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e21.5 years\u003c/p\u003e \u003cp\u003eAsymptomatic\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e16\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eVaran et al.\u003c/p\u003e \u003cp\u003e(2015) [\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003edorsal pons\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003ediplopia, L ptosis,\u003c/p\u003e \u003cp\u003eataxia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eSubtotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e3 months\u003c/p\u003e \u003cp\u003eAsymptomatic,\u003c/p\u003e \u003cp\u003eresolution of the lesion\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e17\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eZhu et al.\u003c/p\u003e \u003cp\u003e(2013) [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMultiple lesions,\u003c/p\u003e \u003cp\u003ebilateral cerebral and cerebellar\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eseizure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eSurgery\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e8 months\u003c/p\u003e \u003cp\u003eStable condition\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e18\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCamp et al.\u003c/p\u003e \u003cp\u003e(2012) [\u003cspan citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e31/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMultiple lesions,\u003c/p\u003e \u003cp\u003eL frontal, R frontal,\u003c/p\u003e \u003cp\u003eR parietal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eseizure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTotal resection of\u003c/p\u003e \u003cp\u003eL frontal lesion\u003c/p\u003e \u003cp\u003e+ steroid therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e1 year\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eLudemann et al.\u003c/p\u003e \u003cp\u003e(2015) [\u003cspan citationid=\"CR35\" class=\"CitationRef\"\u003e35\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMultiple lesions,\u003c/p\u003e \u003cp\u003eboth frontal lobes ,\u003c/p\u003e \u003cp\u003eintraventricular\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eleg weakness, fever\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eSteroid therapy\u003c/p\u003e \u003cp\u003e\u0026thinsp;+\u0026thinsp;followed subtotal resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e16 months\u003c/p\u003e \u003cp\u003eAsymptomatic\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e20\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSymss et al.\u003c/p\u003e \u003cp\u003e(2010) [\u003cspan citationid=\"CR36\" class=\"CitationRef\"\u003e36\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e17/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMultiple lesions,\u003c/p\u003e \u003cp\u003esubfrontal lobe extending into suprasellar region,\u003c/p\u003e \u003cp\u003eL temporal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache, emesis, amenorrhea, weight gain, memory lapses,\u003c/p\u003e \u003cp\u003edecreased visual acuity\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eSubtotal resection\u003c/p\u003e \u003cp\u003eof subfrontal lesion\u003c/p\u003e \u003cp\u003e+ radiation therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e2 years\u003c/p\u003e \u003cp\u003eAsymptomatic,\u003c/p\u003e \u003cp\u003eresolution of the lesion\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e21\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eBing et al.\u003c/p\u003e \u003cp\u003e(2009) [\u003cspan citationid=\"CR37\" class=\"CitationRef\"\u003e37\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e32/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eL parieto-occipital\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eseizure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eBiopsy\u003c/p\u003e \u003cp\u003e+ steroid therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e5 years\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e22\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eChivukula et al. (2015) [\u003cspan citationid=\"CR38\" class=\"CitationRef\"\u003e38\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e66/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003ehypothalamus\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache, diplopia, confusion,\u003c/p\u003e \u003cp\u003ebilateral partial abducens\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eBiopsy\u003c/p\u003e \u003cp\u003e+ radiation therapy\u003c/p\u003e \u003cp\u003e+ steroid therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e19 months\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e23\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eEl Majodoub et al.\u003c/p\u003e \u003cp\u003e(2009) [\u003cspan citationid=\"CR39\" class=\"CitationRef\"\u003e39\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e10/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eL white matter and basal ganglia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eheadache, nausea, vomiting, weight loss, dizziness\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eBiopsy\u003c/p\u003e \u003cp\u003e+ radiation therapy\u003c/p\u003e \u003cp\u003e+ steroid therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e49 months\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e24\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMiletic et al.\u003c/p\u003e \u003cp\u003e(2008) [\u003cspan citationid=\"CR40\" class=\"CitationRef\"\u003e40\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e8/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eL insula and thalamus\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003esymptoms of raised intracranial pressure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eBiopsy\u003c/p\u003e \u003cp\u003e+ steroid therapy\u003c/p\u003e \u003cp\u003e+ radiation therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e3 years\u003c/p\u003e \u003cp\u003eComplete remission\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e25\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eImada et al.\u003c/p\u003e \u003cp\u003e(2015) [\u003cspan citationid=\"CR41\" class=\"CitationRef\"\u003e41\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e68/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003ebrainstem\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003evisual disturbances,\u003c/p\u003e \u003cp\u003erespiratory failure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eSteroid therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e3 years\u003c/p\u003e \u003cp\u003eDeath\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e26\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRichardson et al.\u003c/p\u003e \u003cp\u003e(2018) [\u003cspan citationid=\"CR42\" class=\"CitationRef\"\u003e42\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e64/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMultiple lesions, brainstem,\u003c/p\u003e \u003cp\u003eR cerebellar,\u003c/p\u003e \u003cp\u003emultiple cerebral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003edouble vision\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eBiopsy\u003c/p\u003e \u003cp\u003e+ cladribine\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e2 years\u003c/p\u003e \u003cp\u003eDeath\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e27\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003ePresent case\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e6x/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMultiple lesions,\u003c/p\u003e \u003cp\u003eR frontal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eL hemiplegia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eBiopsy\u003c/p\u003e \u003cp\u003e+ steroid therapy\u003c/p\u003e \u003cp\u003e+ radiation therapy\u003c/p\u003e \u003cp\u003e+ cladribine\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e30 months\u003c/p\u003e \u003cp\u003eDeath\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003ePurav et al.\u003c/p\u003e \u003cp\u003e(2005) [\u003cspan citationid=\"CR43\" class=\"CitationRef\"\u003e43\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e50/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMultiple lesions,\u003c/p\u003e \u003cp\u003elocation unspecified\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003espastic hemiparesis,\u003c/p\u003e \u003cp\u003emild dysphasia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eBiopsy\u003c/p\u003e \u003cp\u003eR parietal lesion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e10 days\u003c/p\u003e \u003cp\u003eDeath from cardiac disease\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e29\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eDeshayes et al.\u003c/p\u003e \u003cp\u003e(2013) [\u003cspan citationid=\"CR44\" class=\"CitationRef\"\u003e44\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e51/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eFloor of third ventricle\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003ememory disturbance\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eBiopsy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003eNot reported\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eOutcomes and treatment of isolated intra-axial Rosai Dorfman disease\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"4\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eOutcomes\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eTreatment\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eCase\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eNumber (%)\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003econtrolled\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eResection alone\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1\u0026ndash;17\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e17 (59%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSubtotal resection\u0026thinsp;+\u0026thinsp;additional therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e18\u0026ndash;20\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3 (10%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eBiopsy\u0026thinsp;+\u0026thinsp;additional therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e21\u0026ndash;24\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (14%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eintractable\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eDeath of unresectable lesions \u003c/p\u003e \u003cp\u003e+ additional therapy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e25\u0026ndash;27\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3 (10%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eothers\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eDeath of another cause\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (3%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNot reported\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e29\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (3%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eIn our case, the tumor was in primary motor area and hard to resect. The first 4 courses of cladribine were temporally effective, however the residual regrowing lesions were treated with steroids, radiotherapy and additional cladribine, but to no avail. It may have been necessary to consider total resection when the tumor began to grow again even though it was in the primary motor area. Since intra-axial CNS RDD itself has rarely been reported, further study is needed for its treatment.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNone.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics declarations\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll procedures performed in studies were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConflict of interest\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no conflicts of interest.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthical approval\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe patient family gave informed consent for publication.\u0026nbsp;\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eRosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 87:63-70\u003c/li\u003e\n\u003cli\u003eLa Barge DV 3rd, Salzman KL, Harnsberger HR et al (2008) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): imaging manifestations in the head and neck. AJR Am J Roentgenol 191:W299-306\u003c/li\u003e\n\u003cli\u003eBruce-Brand C, Schneider JW, Schubert P (2020) Rosai-Dorfman disease: an overview. J Clin Pathol 73:697-705\u003c/li\u003e\n\u003cli\u003eSathyanarayanan V, Issa A, Pinto R et al (2019) Rosai-Dorfman Disease: The MD Anderson Cancer Center Experience. Clin Lymphoma Myeloma Leuk 19:709-14\u003c/li\u003e\n\u003cli\u003eSandoval-Sus JD, Sandoval-Leon AC, Chapman JR et al (2014) Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. 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Acta Neurochir (Wien) 145:145-9\u003c/li\u003e\n\u003cli\u003eLi Y, Sun H, Zhang Y et al (2012) Isolated intracranial Rosai-Dorfman disease presenting as mental deterioration. Clin Neurol Neurosurg 114:1070-3\u003c/li\u003e\n\u003cli\u003eMahzoni P, Zavareh MH, Bagheri M et al (2012) Intracranial ROSAI-DORFMAN Disease. J Res Med Sci 17:304-7\u003c/li\u003e\n\u003cli\u003eMorandi X, Godey B, Riffaud L et al (2000) Isolated Rosai-Dorfman disease of the fourth ventricle. Case illustration. J Neurosurg 92:890\u003c/li\u003e\n\u003cli\u003eNatarajan S, Post KD, Strauchen J et al (2000) Primary intracerebral rosai-dorfman disease: a case report. J Neurooncol 47:73-7\u003c/li\u003e\n\u003cli\u003ePatwardhan PP, Goel NA (2018) Isolated Intraventricular Rosai-Dorfman Disease. Asian J Neurosurg 13:1285-7\u003c/li\u003e\n\u003cli\u003eSundaram C, Uppin SG, Prasad BC et al (2005) Isolated Rosai Dorfman disease of the central nervous system presenting as dural-based and intraparenchymal lesions. Clin Neuropathol 24:112-7\u003c/li\u003e\n\u003cli\u003eVaran A, Şen H, Akalan N et al (2015) Pontine Rosai-Dorfman disease in a child. Childs Nerv Syst 31:971-5\u003c/li\u003e\n\u003cli\u003eZhu F, Zhang JT, Xing XW et al (2013) Rosai-Dorfman disease: a retrospective analysis of 13 cases. Am J Med Sci 345:200-10\u003c/li\u003e\n\u003cli\u003eCamp SJ, Roncaroli F, Apostolopoulos V et al (2012) Intracerebral multifocal Rosai-Dorfman disease. J Clin Neurosci 19:1308-10\u003c/li\u003e\n\u003cli\u003eL\u0026uuml;demann W, Banan R, Samii A et al (2015) Cerebral Rosai-Dorfman disease. Childs Nerv Syst 31:529-32\u003c/li\u003e\n\u003cli\u003eSymss NP, Cugati G, Vasudevan MC et al (2010) Intracranial Rosai Dorfman Disease: report of three cases and literature review. Asian J Neurosurg 5:19-30\u003c/li\u003e\n\u003cli\u003eBing F, Brion JP, Grand S et al (2009) Tumor arising in the periventricular region. 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Clin Nucl Med 38:e50-2\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Rosai-Dorfman disease, intra-axial tumor, Cladribine","lastPublishedDoi":"10.21203/rs.3.rs-6620676/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6620676/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eRosai-Dorfman disease (RDD) is a rare histiocytic disorder that often presents with fever, painless cervical lymphadenopathy and leukemia. Most cases of RDD have a favorable outcome. RDD involving only the central nervous system (CNS) is very rare, and intra-axial CNS RDD is little. We report a case of intra-axial intractable CNS RDD with an atypical presentation. The case reported here is that of a woman in her 60s. Magnetic resonance imaging (MRI) showed a solid tumor 30 mm in diameter in the right frontal lobe. Because the tumor was in the primary motor area, a biopsy was performed for diagnosis. The tumor was ultimately diagnosed as RDD. Although the steroids and cladribine temporarily reduced the size of the tumor, it was ultimately unable to control the growth of the tumor. The patient finally died about two and a half years after the onset. In previous reports, two cases of death directly caused by intra-axial CNS RDD had been reported in the past, because in both cases surgical treatment was not possible. In our case, the tumor was in primary motor area and hard to resect. Total surgical resection is likely to be required to cure intra-axial CNS RDD.\u003c/p\u003e","manuscriptTitle":"An Intractable Intra-Axial central nervous system Rosai-Dorfman Disease: A Case Report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-06-09 10:25:34","doi":"10.21203/rs.3.rs-6620676/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"1fd6c62e-3066-4aea-94b4-de6a9baefae6","owner":[],"postedDate":"June 9th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-06-22T16:41:29+00:00","versionOfRecord":[],"versionCreatedAt":"2025-06-09 10:25:34","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-6620676","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6620676","identity":"rs-6620676","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}