Childs Nerv Syst (2015) 31:317–323 DOI 10.1007/s00381-014-2536-y
CASE REPORT
Intracranial Rosai-Dorfman disease mimicking multiple meningiomas in a child: a case report and review of the literature Yongji Tian & Junmei Wang & Jin zhao Ge & Zhenyu Ma & Ming Ge
Received: 28 July 2014 / Accepted: 18 August 2014 / Published online: 3 September 2014 # Springer-Verlag Berlin Heidelberg 2014
Abstract Objective Rosai-Dorfman disease (RDD) is a rare idiopathic, non-neoplastic histioproliferative disease. Central nervous system (CNS) manifestations are extremely rare. In this paper, we describe a 6-year-old boy with intracranial RDD mimicking multiple meningiomas both clinically and radiologically. We reviewed the literature to understand the clinical behaviour, clinicopathological features and treatment options. Methods A PubMed (US National Library of Medicine) search using the keywords ‘Rosai-Dorfman disease’ and ‘central nervous system’ was performed and citations were reviewed. Results Eighty-five cases of RDD involving the CNS have been reported until date, and only 7 cases involved children. Of the 85 cases, 16 cases mimicked multiple meningiomas. Our case is the first to involve multiple lesions in a child under 14 years old. Conclusion After reviewing the literature, we concluded that RDD should be considered as a differential diagnosis for Y. Tian : Z. Ma : M. Ge (*) Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China e-mail: [email protected] Y. Tian : Z. Ma : M. Ge China National Clinical Research Center for Neurological Diseases (NCRC-ND), Beijing 100050, China Y. Tian : Z. Ma : M. Ge Center for Brain Tumor, Beijing Institute for Brain Disorders, Beijing 100050, China Y. Tian : Z. Ma : M. Ge Beijing Key Laboratory for Brain Tumor, Beijing 100050, China J. Wang Department of Neuropathology, Beijing Neurosurgery Institute, Beijing 100050, China J. z. Ge Gui Yang Medical College, Guiyang 550004, China
lesions mimicking multiple meningiomas, especially in children. Resection of the intracranial lesion is the most effective treatment, and a definitive diagnosis should be based on histopathologic and immunocytochemical examinations. Keywords Rosai-Dorfman disease . Central nervous system . Child . Meningiomas . Sinus histiocytosis with massive lymphadenopathy
Introduction Rosai-Dorfman disease (RDD) is a rare, idiopathic, histiocytic proliferative disease. It may involve the orbits, superior airway, skeleton, skin, gastrointestinal tract, genitourinary tract, thyroid and central nervous system [1–11]. Intracranial RDD is rare and described in approximately 5 % of cases, the majority of which present with an isolated lesion [2, 3, 5, 12]. Patients with isolated intracranial RDD tend to be older (mean 39.4 years) than patients with systemic RDD [1]. In this paper, we describe a case of intracranial RDD mimicking multiple meningiomas both clinically and radiologically in a 6-year-old boy presenting with a 3-month history of fever. A literature review was also performed to investigate clinical data, laboratory and imaging results, treatment protocols and disease prognosis pertaining to RDD.
Case report Medical history A 6-year-old boy was admitted to the hospital with a 3-month history of fever and occasional headaches. The fever ranged from 37.5 to 38.5 °C even after treatment with antibiotics in local hospital. The patient did not experience seizures or loss
318
Childs Nerv Syst (2015) 31:317–323
of vision. Physical examinations revealed an enlarged head circumference (52 cm) and right-sided subcutaneous cervical lymphadenopathy. No hepatosplenomegaly or other focal lesions were observed. Neurological examinations revealed no abnormalities. Computed tomography (CT) scans revealed five hyperdense lesions in the frontal falx cerebri, lateral ventricles and both sides
Fig. 1 Axial (a), coronal (b) and sagittal (c) Gadolinium-enhanced T1-weighted MR image shows a heterogeneously enhancing mass lesion located in lateral ventricle (white arrow), tentorium of cerebellum in both sides (large white arrow) and in frontal falx (thin white arrow). d Postoperative Gadoliniumenhanced T1-weighted MR images showed the lesion in left ventricle had been resected completely. e Postoperative CT scan showed nearly hyperdense lesions in lesions in the frontal falx cerebrum, right lateral ventricule and drainage tube in left ventricule
A
B
C
D
E
of the tentorium cerebelli, symmetrically. No evidence of any calcification was noted within these lesions (Fig. 1e). Lesions were predominantly hypointense on T1-weighted magnetic resonance imaging (MRI) scans and near hyperintense with mild peritumoural oedema on T2-weighted images. On contrast administration, the lesions showed homogeneous enhancement with the dural tail sign (Fig. 1a–c).
Childs Nerv Syst (2015) 31:317–323
Laboratory examinations revealed leucocytosis with neutrophilia and thrombocytosis; increased erythrocyte sedimentation rate (ESR); and normal T4, prolactin and cortisol levels. Further routine haematological and biochemical tests were normal. Radiologically, the diagnosis was multiple meningioma. Surgical treatment and follow-up results The patient underwent surgical treatment for the largest mass in the left ventricle through a left occipitotemporal approach. Intraoperative views revealed a pale yellow, solid, hypervascular nodule with a clear boundary in the left ventricle that originated from the plexus choroid. During the operation, the lesion was markedly vascular. The lesion was completely removed, as confirmed by postoperative MRI (Fig. 1d). Histopathologic examinations revealed numerous histiocytes showing lymphophagocytosis in a background of numerous plasma cells. Immunohistochemical analysis indicated that most of the histiocytes were diffusely positive for CD68, lysozyme and S100 protein (Fig. 2a–d) and negative for CD1a. Thus, on the basis of these features, the lesions were diagnosed as intracranial RDD. The patient had an uneventful postoperative course and returned to hospital for resection of the other intracranial lesions.
Fig. 2 Paraffin section showing (a) many contain phagocytized well-preserved lymphocytes within the abundant cytoplasm in histiocyte cells in the inflammatory background (haematoxylin and eosin, ×100). b Immunopositivity for S-100 protein (avidin biotin complex immunoperoxidase method ×100), c immunopositivity for CD68 (×100) and (d) Lysozyme- (×400)
319
Discussion RDD, also known as sinus histiocytosis with massive lymphadenopathy, was first recognized as a distinct clinicopathologic entity by Rosai and Dorfman in 1969. It is considered a benign self-limiting histiocytic disorder of presumed nonneoplastic nature; however, the aetiology and pathogenesis remain poorly understood [1, 6, 7, 13, 14]. Although extranodal involvement is known to occur, central nervous system (CNS) manifestations of RDD are rare (5 % of cases) and most commonly involve patients between 22 and 63 years of age, with a male predominance; the mean age at presentation is 39.4 years [1]. From the literature, 7 of 86 reported intracranial RDD cases were in children [15–22]. Only two children patients were treated for clinical manifestations of RDD [17, 22]. Our case is unique because it is the first to report multiple intracranial lesions mimicking meningiomas in a 6-year-old male patient. In total, 16 cases with multiple intracranial lesions have been reported (Table 1) [15, 23–35]. Common sites of RDD involvement in the CNS include the cerebral convexity, cranial base, parasagittal region, suprasellar region, cavernous sinus and petroclival region. Clinically, intracranial RDD causes a variety of symptoms including headache, nausea and vomiting, dizziness or epilepsy, fever, malaise, weight loss and night sweats and specific symptoms [1, 5–8]. Patients with lesions located in the sellar
70 59
53
71 72 22
2003 Chen KT. M 2003 Sato A, Sakurada K, Sonoda Y, F Saito S, Kayama T, Jokura H, Yoshimoto T, Nakazato Y.
M
M
M M
2006 McPherson CM, Brown J, Kim AW, DeMonte F.
2006 Gupta DK, Suri A, Mahapatra AK, Mehta VS, Garg A, Sarkar C, Ahmad FU.
2009 Russo N, Giangaspero F, Beccaglia MR, Santoro A.
2011 Zhang XJ, Piao YS, Chen L, M Tang GC, Wei LF, Yang H, Lu DH. F 2012 Catalucci A, Lanni G, Ventura L, M Ricci A, Galzio RJ, Gallucci M. 26 57
15
67
F
1999 Udono H, Fukuyama K, Okamoto H, Tabuchi K.
38
M
1996 Resnick DK, Johnson BL, Lovely TJ.
50
30
Bifrontal craniotomy to remove frontal convexity mass
Parietal craniotomy for the complete removal of both lesions Left retromastoid craniectomy
Subfrontal craniotomy
Treatment
Stable in 20 months follow-up
Stable for 2 years
Recovery well 6 months later
Treated with gentamycin, streptomucin and prednisone, no improvement
Follow-up
No detailed information No detailed information No detailed information 6-year history of progressive Multiple, bilateral extra-axial enhancing Resection of infratentorial right visual and hearing loss lesions, seven extra-axial and one petroclival right mass, through and tinnitus intraventricular lesion a right transpetrosal approach
No detailed information Not provided
1-year follow-up, MRI confirmed no recurrent lesion 14 months follow up, free of neurological symptoms No detailed information
(Paper in Japanese) Suprasellar region, right temporal Subtotal removal of the suprasellar No recurrence for 2 years convexity, left frontal convexity, left lesion, gamma knife cerebellopontine angle and C5 level radiosurgery to residual lesion of the spinal cord and the other intracranial lesions A lesion at the planum sphenoidale and Right orbitopterional craniotomy, Maintains full functional tuberculum sella compressing the optic nerve decompression abilities 10 months optic nerves, bilateral lesions from postoperation the cerebellopontine angle to the foramen magnum compressing the brainstem and a lesion at C-2 Bilateral petroclival Two stages craniotomy to remove 12 months follow-up, vision has tumours improved to 6/18 in both eyes and the cerebellar signs have resolved completely
Right frontal parasagittal lesion, multiple satellite lesions and an extracranial lesion in the pharynx
Left cerebellopontine angle, three frontal lesion in both sides
Two lesions in the right parietal convexity
Skull base of frontal, middle and posterior fossa in both sides
Site involved
Headache with vomiting and progressive visual deterioration in both eyes (left more than right) for 4 months Mild, slowly progressing upper Left frontal lesion measuring about 2.5 cm Elective frontal craniotomy right-arm paresis for 4 days in diameter, adherent to the dura 2 months of headache, Bilateral frontal lesions adhered to the Craniotomy to resect the two masses suddenly worsened convexity dura mater No detailed information No detailed information No detailed information
Decreased vision in right eye and diminished hearing in right ear
Bilateral visual impairment
6 years of seizure, loss of vision and hearing, facial palsy, speech difficulty and failing memory Left homonymous hemianopia and a left hemisensory inattention in the arm Transient visual loss, headaches, vomiting, deafness in left ear 3-week history of diplopia and headache
Gender Age Symptoms (years)
1989 Song SK, Schwartz IS, M Strauchen JA, Huang YP, Sachdev V, Daftary DR, Vas CJ. 1995 Kim M, Provias J, Bernstein M. M
Year Author
Table 1 Summary of intracranial Rosai–Dorfman’s disease mimicking multiple meningiomas
320 Childs Nerv Syst (2015) 31:317–323
A smooth postoperative course Resection of the large frontal lesion 43 M
A few months history of The left frontal region, 2 small lesions headache and mild dizziness with falcine attachment, a lesion in the vicinty of the anterior clinoid process and one attached to the anterosuperior border of the right petrous bone.
38 M
2013 Joubert C, Dagain A, Faivre A, Nguyen AT, Fesselet J, Figarella-Branger D. 2013 Abdel-Razek M, Matter GA, Azab WA, Katchy KC, Mallik AA.
Headache and frontal syndrome
Multiple extra-axial lesions, disseminated
Excision of the peripherally MRI 12 months demonstrated located left frontal lesion, 5-day resolution of all three lesions course of postoperative dexamethasone (Paper in French) (Paper in French) Intrinsic left frontal lobe, and right parietal and right inferior frontal white matter 31
Two focal seizures
321
2012 Camp SJ, Roncaroli F, F Apostolopoulos V, Weatherall M, Lim S, Nandi D.
Year Author
Table 1 (continued)
Gender Age Symptoms (years)
Site involved
Treatment
Follow-up
Childs Nerv Syst (2015) 31:317–323
region demonstrated endocrine disturbances, such as polydipsia, polyuria and amenorrhea. Our patient experienced headaches, fever and an increased head circumference caused by chronic elevated intracranial pressure. Clinical examinations of patients with RDD previously identified leucocytosis with neutrophilia, polyclonal hypergammaglobulinaemia, increased ESR and normocytic or microcytic anaemia [1, 5–8, 10, 11, 36, 37]. Imaging results are important for diagnosis of RDD and for exclusion of differential diagnoses. The typical imaging appearance of intracranial RDD is that of dural-based, extraaxial, well-circumscribed enhancing masses with possible perilesional cerebral oedema mimicking meningioma, together with mass effects involving contiguous structures and the midline [16, 26, 38–41]. On CT scans, lesions may appear as a homogeneous and lobulated isodense or hyperdense masses with marked contrast enhancement, and the adjacent bone might be eroded. On T1-weighted MRI, RDD lesions are hyperintense or isointense with clear borders hypointense relative to the brain parenchyma, while on T2-weighted images they have an isointense signal with possible intralesional foci of low signal intensity. MRI scans show strong inhomogeneous or homogeneous enhancement after gadolinium injection and demonstrate dural tail sign in all cases [26, 39–41]. Thus, our case closely mimics meningiomas clinically and radiologically, in agreement with other cases reported in the literature [2, 3, 42, 43]. Differential diagnosis may include other pathologies characterized by dural involvement and strong enhancement following contrast administration, such as meningioangiomatosis [38, 44], dural metastases [30], Wegener’s granulomatosis, sarcoidosis, Hodgkin’s lymphoma, inflammatory pseudotumour and Langerhans cell histiocytosis. Diagnosis can only be confirmed by histopathological or immunohistochemical examinations. Typically, histiocytes stain positive for S-100 protein and CD68, but negative for CD1a. Emperipolesis, signifying phagocytosis of autologous lymphocytes, is characteristic of RDD, but is present in only 70 % of cases [45]. RDD is generally considered a benign process that is typically self-limiting and often resolves spontaneously. However, the disease may be persistent and progressive [22]. Treatment options for RDD remain controversial. Various treatment modalities, including surgery, steroids, chemotherapy and radiation therapy, have been tried. Surgical resection is essential for diagnosis [8, 46]. It is also the most effective method of treatment and may be curative [1, 5–12, 15, 19, 29, 47, 48]. Surgery can relieve compression of vital structures, alleviate local damage and preserve function. RDD lesions in the CNS are firm, lobular, whitish-grey or yellowish-tan in colour and adherent to the dura. However, many lesions are difficult to resect completely because lesions
322
can adhere tightly to the cerebral cortex and may invade or surround other critical structures. The extent of surgical resection is strongly correlated with intracranial relapse of the disease [12, 29]. Petzold et al. [12] found intracranial tumour regrowth or recurrence of symptoms in 14 % of 29 patients with a mean follow-up period of 10.1 years. Postoperative treatment efforts should be directed at relapsing cases or those with subresected lesions. Corticosteroid therapy has been proposed and chemotherapy is another modality used to manage RDD, in cases of extensive or progressive disease. Jabali et al. [49] report that a combination of prednisone, 6-mercaptopurine, methotrexate and vinblastine was an effective and safe treatment for the disease. Radiation therapy has been employed for local disease control in such areas as the spinal cord and airway, or in cases of relapse [6, 12, 29, 43].
Conclusion For paediatric patients with multiple extra-axial lesions, a possible differential diagnosis of intracranial RDD should be considered and further investigations performed. Resection of the intracranial lesion is the most effective treatment. A definitive diagnosis should be based on histopathologic and immunocytochemical examinations. Furthermore, long-term follow-ups are required. Future efforts should be directed at investigating the aetiology of RDD and postoperative treatments for relapse or residual lesions. Acknowledgements Dr Yongji Tian (first author) was partially supported by the National Natural Science Foundation of China (No. 30900479), Beijing Nova-Plan Program (No. 2010B121), National Key Technology Research and Development Program of the Ministry of Science and Technology of China (2013BAI09B03) and BIBDPXM2013_014226_07_000084. Ethical standards This study was approved by the appropriate ethics committee and was performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki. Conflict of interest All authors declare that they have no conflicting interests and no authors were supported or funded by any drug company.
References 1. Foucar E, Rosai J, Dorfman R (1990) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 7:19–73 2. Konishi E, Ibayashi N, Yamamoto S, Scheithauer BW (2003) Isolated intracranial Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). AJNR Am J Neuroradiol 24:515–518 3. Wu M, Anderson AE, Kahn LB (2001) A report of intracranial RosaiDorfman disease with literature review. Ann Diagn Pathol 5:96–102
Childs Nerv Syst (2015) 31:317–323 4. Wang Y, Gao X, Tang W, Jiang C (2010) Rosai-Dorfman disease isolated to the central nervous system: a report of six cases. Neuropathology: Off J Jpn Soc Neuropathol 30:154–158 5. Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV (2001) Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol: Off J US Can Acad Pathol, Inc 14: 172–178 6. Rosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol Lab Med 87:63–70 7. Foucar E, Rosai J, Dorfman RF (1978) Sinus histiocytosis with massive lymphadenopathy. Arch Otolaryngol 104:687–693 8. Adeleye AO, Amir G, Fraifeld S, Shoshan Y, Umansky F, Spektor S (2010) Diagnosis and management of Rosai-Dorfman disease involving the central nervous system. Neurol Res 32:572–578 9. Türe U, Seker A, Bozkurt SU, Uneri C, Sav A, Pamir MN (2004) Giant intracranial Rosai-Dorfman disease. J Clin Neurosci: Off J Neurosurg Soc Aust 11:563–566 10. Said R, Abi-Fadel F, Talwar J, Attallah JP, Dilawari A (2011) Intracranial Rosai-Dorfman: a clinical challenge. Neurologist 17: 117–119 11. Eiras Jda C, Schettini A, Lima LL, Tubilla LH, Oliveira RM (2010) Cutaneous Rosai-Dorfman disease: a case report. An Bras Dermatol 85:687–690 12. Petzold A, Thom M, Powell M, Plant GT (2001) Relapsing intracranial Rosai-Dorfman disease. J Neurol Neurosurg Psychiatr 71:538– 541 13. Middel P, Hemmerlein B, Fayyazi A, Kaboth U, Radzun HJ (1999) Sinus histiocytosis with massive lymphadenopathy: evidence for its relationship to macrophages and for a cytokine-related disorder. Histopathology 35:525–533 14. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES (1992) Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis 166:291–295 15. Gupta DK, Suri A, Mahapatra AK et al (2006) Intracranial Rosai–Dorfman disease in a child mimicking bilateral giant petroclival meningiomas: a case report and review of literature. Child's Nerv Syst: ChNS: Off J Int Soc Pediatr Neurosurg 22: 1194–1200 16. Theeler BJ, Keylock JB, Yoest SM (2008) Teaching NeuroImage: isolated intracranial Rosai-Dorfman disease mimicking a meningioma. Neurology 70:e42 17. Shaver EG, Rebsamen SL, Yachnis AT, Sutton LN (1993) Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy. Case report. J Neurosurg 79:769–773 18. Woodcock RJ Jr, Mandell J, Lipper MH (1999) Sinus histiocytosis (Rosai-Dorfman disease) of the suprasellar region: MR imaging findings—a case report. Radiology 213:808–810 19. Griffiths SJ, Tang W, Parameswaran R, Kelsey A, West CG (2004) Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. Br J Neurosurg 18:293–297 20. Lungren MP, Petrella JR, Cummings TJ, Grant GA (2009) Isolated intracranial Rosai-Dorfman disease in a child. AJNR Am J Neuroradiol 30:E148–149 21. Gupta K, Bagdi N, Sunitha P, Ghosal N (2011) Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child: a case report and review of the literature. Br J Radiol 84:e138–141 22. Di Rocco F, Garnett MR, Puget S, Pueyerredon F, Roujeau T, Jaubert F, Sainte-Rose C (2007) Cerebral localization of Rosai-Dorfman disease in a child. Case report. J Neurosurg 107:147–151 23. Song SK, Schwartz IS, Strauchen JA, Huang YP, Sachdev V, Daftary DR, Vas CJ (1989) Meningeal nodules with features of extranodal sinus histiocytosis with massive lymphadenopathy. Am J Surg Pathol 13:406–412
Childs Nerv Syst (2015) 31:317–323 24. Kim M, Provias J, Bernstein M (1995) Rosai-Dorfman disease mimicking multiple meningioma: case report. Neurosurgery 36:1185–1187 25. Resnick DK, Johnson BL, Lovely TJ (1996) Rosai-Dorfman disease presenting with multiple orbital and intracranial masses. Acta Neuropathologica 91:554–557 26. Udono H, Fukuyama K, Okamoto H, Tabuchi K (1999) RosaiDorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report. J Neurosurg 91:335–339 27. Chen KT (2003) Crush cytology of Rosai-Dorfman disease of the central nervous system. A report of 2 cases. Acta Cytologica 47: 1111–1115 28. Sato A, Sakurada K, Sonoda Y, Saito S, Kayama T, Jokura H, Yoshimoto T, Nakazato Y (2003) Rosai-Dorfman disease presenting with multiple intracranial and intraspinal masses: a case report. No Shinkei Geka Neurol Surg 31:1199–1204 29. McPherson CM, Brown J, Kim AW, DeMonte F (2006) Regression of intracranial Rosai-Dorfman disease following corticosteroid therapy. Case report. J Neurosurg 104:840–844 30. Russo N, Giangaspero F, Beccaglia MR, Santoro A (2009) Intracranial dural histiocytosis. Br J Neurosurg 23:449–454 31. Zhang XJ, Piao YS, Chen L, Tang GC, Wei LF, Yang H, Lu DH (2011) Multiple intracranial lesions: a clinicalpathologic study of 62 cases. Zhonghua Bing Li Xue Za Zhi Chin J Pathol 40:599–603 32. Catalucci A, Lanni G, Ventura L, Ricci A, Galzio RJ, Gallucci M (2012) A rare case of intracranial Rosai-Dorfman disease mimicking multiple meningiomas. A case report and review of the literature. Neuroradiol J 25:569–574 33. Camp SJ, Roncaroli F, Apostolopoulos V, Weatherall M, Lim S, Nandi D (2012) Intracerebral multifocal Rosai-Dorfman disease. J Clin Neurosc: Off J Neurosurg Soc Australas 19:1308–1310 34. Joubert C, Dagain A, Faivre A, Nguyen AT, Fesselet J, FigarellaBranger D (2013) Intracranial Rosai-Dorfman disease mimicking multiple meningiomas. Rev Med Brux 34:112–114 35. Abdel-Razek M, Matter GA, Azab WA, Katchy KC, Mallik AA (2013) Isolated intracranial Rosai-Dorfman disease: report of two cases and a review of the literature. Turk Neurosurg 23:509–513 36. Foucar E, Rosai J, Dorfman RF, Brynes RK (1982) The neurologic manifestations of sinus histiocytosis with massive lymphadenopathy. Neurology 32:365–372 37. Gaitonade S (2007) Multifocal, extranodal sinus histiocytosis with massive lymphadenopathy: an overview. Arch Pathol Lab Med 131: 1117–1121
323 38. Chen YY, Tiang XY, Li Z, Luo BN, Huang Q (2010) Sporadic meningioangiomatosis-associated atypical meningioma mimicking parenchymal invasion of brain: a case report and review of the literature. Diagn Pathol 18:39 39. Raslan OA, Schellingerhout D, Fuller GN, Ketonen LM (2011) Rosai-Dorfman disease in neuroradiology: imaging findings in a series of 10 patients. AJR Am J Roentgenol 196:W187–193 40. Geara AR, Ayoubi MA, Achram MC, Chamseddine NM (2004) Rosai-Dorfman disease mimicking neurofibromatosis: case presentation and review of the literature. Clin Radiol 59:625–630 41. Toh CH, Lin CY, Wong HF, Wei KC, Ng SH, Wan YL (2005) RosaiDorfman disease with dural sinus invasion. Report of two cases. J Neurosurg 102:550–554 42. Sharma MS, Padua MD, Jha AN (2005) Rosai-Dorfman disease mimicking a sphenoid wing meningioma. Neurol India 53:110–111 43. Kidd DP, Revesz T, Miller NR (2006) Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement. Neurology 67:1551–1555 44. Kremer S, Grand S, Rémy C, Pasquier B, Benabid AL, Bracard S, Le Bas JF (2004) Contribution of dynamic contrast MR imaging to the differentiation between dural metastasis and meningioma. Neuroradiology 46:642–648 45. Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, Bucsky P, Egeler RM, Elinder G, Gadner H, Gresik M, Henter JI, Imashuku S, Janka-Schaub G, Jaffe R, Ladisch S, Nezelof C, Pritchard J (1997) Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol 29:157–166 46. Zhang JT, Tian HJ, Lang SY, Wang XQ (2010) Primary intracerebral Rosai-Dorfman disease. J Clin Neurosci: Off J Neurosurg Soc Australas 17:1286–1288 47. Wan S, Teng X, Zhan R, Yu J, Gu J, Zhang K (2008) Isolated intracranial Rosai-Dorfman disease mimicking suprasellar meningioma: case report with review of the literature. J Int Med Res 36:1134– 1139 48. Gaetani P, Tancioni F, Di Rocco M, Rodriguez y Baena R (2000) Isolated cerebellar involvement in Rosai-Dorfman disease: case report. Neurosurgery 46:479–481 49. Jabali Y, Smrcka V, Pradna J (2005) Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report. Int J Surg Pathol 13:285–289
CASE REPORT
Intracranial Rosai-Dorfman disease mimicking multiple meningiomas in a child: a case report and review of the literature Yongji Tian & Junmei Wang & Jin zhao Ge & Zhenyu Ma & Ming Ge
Received: 28 July 2014 / Accepted: 18 August 2014 / Published online: 3 September 2014 # Springer-Verlag Berlin Heidelberg 2014
Abstract Objective Rosai-Dorfman disease (RDD) is a rare idiopathic, non-neoplastic histioproliferative disease. Central nervous system (CNS) manifestations are extremely rare. In this paper, we describe a 6-year-old boy with intracranial RDD mimicking multiple meningiomas both clinically and radiologically. We reviewed the literature to understand the clinical behaviour, clinicopathological features and treatment options. Methods A PubMed (US National Library of Medicine) search using the keywords ‘Rosai-Dorfman disease’ and ‘central nervous system’ was performed and citations were reviewed. Results Eighty-five cases of RDD involving the CNS have been reported until date, and only 7 cases involved children. Of the 85 cases, 16 cases mimicked multiple meningiomas. Our case is the first to involve multiple lesions in a child under 14 years old. Conclusion After reviewing the literature, we concluded that RDD should be considered as a differential diagnosis for Y. Tian : Z. Ma : M. Ge (*) Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China e-mail: [email protected] Y. Tian : Z. Ma : M. Ge China National Clinical Research Center for Neurological Diseases (NCRC-ND), Beijing 100050, China Y. Tian : Z. Ma : M. Ge Center for Brain Tumor, Beijing Institute for Brain Disorders, Beijing 100050, China Y. Tian : Z. Ma : M. Ge Beijing Key Laboratory for Brain Tumor, Beijing 100050, China J. Wang Department of Neuropathology, Beijing Neurosurgery Institute, Beijing 100050, China J. z. Ge Gui Yang Medical College, Guiyang 550004, China
lesions mimicking multiple meningiomas, especially in children. Resection of the intracranial lesion is the most effective treatment, and a definitive diagnosis should be based on histopathologic and immunocytochemical examinations. Keywords Rosai-Dorfman disease . Central nervous system . Child . Meningiomas . Sinus histiocytosis with massive lymphadenopathy
Introduction Rosai-Dorfman disease (RDD) is a rare, idiopathic, histiocytic proliferative disease. It may involve the orbits, superior airway, skeleton, skin, gastrointestinal tract, genitourinary tract, thyroid and central nervous system [1–11]. Intracranial RDD is rare and described in approximately 5 % of cases, the majority of which present with an isolated lesion [2, 3, 5, 12]. Patients with isolated intracranial RDD tend to be older (mean 39.4 years) than patients with systemic RDD [1]. In this paper, we describe a case of intracranial RDD mimicking multiple meningiomas both clinically and radiologically in a 6-year-old boy presenting with a 3-month history of fever. A literature review was also performed to investigate clinical data, laboratory and imaging results, treatment protocols and disease prognosis pertaining to RDD.
Case report Medical history A 6-year-old boy was admitted to the hospital with a 3-month history of fever and occasional headaches. The fever ranged from 37.5 to 38.5 °C even after treatment with antibiotics in local hospital. The patient did not experience seizures or loss
318
Childs Nerv Syst (2015) 31:317–323
of vision. Physical examinations revealed an enlarged head circumference (52 cm) and right-sided subcutaneous cervical lymphadenopathy. No hepatosplenomegaly or other focal lesions were observed. Neurological examinations revealed no abnormalities. Computed tomography (CT) scans revealed five hyperdense lesions in the frontal falx cerebri, lateral ventricles and both sides
Fig. 1 Axial (a), coronal (b) and sagittal (c) Gadolinium-enhanced T1-weighted MR image shows a heterogeneously enhancing mass lesion located in lateral ventricle (white arrow), tentorium of cerebellum in both sides (large white arrow) and in frontal falx (thin white arrow). d Postoperative Gadoliniumenhanced T1-weighted MR images showed the lesion in left ventricle had been resected completely. e Postoperative CT scan showed nearly hyperdense lesions in lesions in the frontal falx cerebrum, right lateral ventricule and drainage tube in left ventricule
A
B
C
D
E
of the tentorium cerebelli, symmetrically. No evidence of any calcification was noted within these lesions (Fig. 1e). Lesions were predominantly hypointense on T1-weighted magnetic resonance imaging (MRI) scans and near hyperintense with mild peritumoural oedema on T2-weighted images. On contrast administration, the lesions showed homogeneous enhancement with the dural tail sign (Fig. 1a–c).
Childs Nerv Syst (2015) 31:317–323
Laboratory examinations revealed leucocytosis with neutrophilia and thrombocytosis; increased erythrocyte sedimentation rate (ESR); and normal T4, prolactin and cortisol levels. Further routine haematological and biochemical tests were normal. Radiologically, the diagnosis was multiple meningioma. Surgical treatment and follow-up results The patient underwent surgical treatment for the largest mass in the left ventricle through a left occipitotemporal approach. Intraoperative views revealed a pale yellow, solid, hypervascular nodule with a clear boundary in the left ventricle that originated from the plexus choroid. During the operation, the lesion was markedly vascular. The lesion was completely removed, as confirmed by postoperative MRI (Fig. 1d). Histopathologic examinations revealed numerous histiocytes showing lymphophagocytosis in a background of numerous plasma cells. Immunohistochemical analysis indicated that most of the histiocytes were diffusely positive for CD68, lysozyme and S100 protein (Fig. 2a–d) and negative for CD1a. Thus, on the basis of these features, the lesions were diagnosed as intracranial RDD. The patient had an uneventful postoperative course and returned to hospital for resection of the other intracranial lesions.
Fig. 2 Paraffin section showing (a) many contain phagocytized well-preserved lymphocytes within the abundant cytoplasm in histiocyte cells in the inflammatory background (haematoxylin and eosin, ×100). b Immunopositivity for S-100 protein (avidin biotin complex immunoperoxidase method ×100), c immunopositivity for CD68 (×100) and (d) Lysozyme- (×400)
319
Discussion RDD, also known as sinus histiocytosis with massive lymphadenopathy, was first recognized as a distinct clinicopathologic entity by Rosai and Dorfman in 1969. It is considered a benign self-limiting histiocytic disorder of presumed nonneoplastic nature; however, the aetiology and pathogenesis remain poorly understood [1, 6, 7, 13, 14]. Although extranodal involvement is known to occur, central nervous system (CNS) manifestations of RDD are rare (5 % of cases) and most commonly involve patients between 22 and 63 years of age, with a male predominance; the mean age at presentation is 39.4 years [1]. From the literature, 7 of 86 reported intracranial RDD cases were in children [15–22]. Only two children patients were treated for clinical manifestations of RDD [17, 22]. Our case is unique because it is the first to report multiple intracranial lesions mimicking meningiomas in a 6-year-old male patient. In total, 16 cases with multiple intracranial lesions have been reported (Table 1) [15, 23–35]. Common sites of RDD involvement in the CNS include the cerebral convexity, cranial base, parasagittal region, suprasellar region, cavernous sinus and petroclival region. Clinically, intracranial RDD causes a variety of symptoms including headache, nausea and vomiting, dizziness or epilepsy, fever, malaise, weight loss and night sweats and specific symptoms [1, 5–8]. Patients with lesions located in the sellar
70 59
53
71 72 22
2003 Chen KT. M 2003 Sato A, Sakurada K, Sonoda Y, F Saito S, Kayama T, Jokura H, Yoshimoto T, Nakazato Y.
M
M
M M
2006 McPherson CM, Brown J, Kim AW, DeMonte F.
2006 Gupta DK, Suri A, Mahapatra AK, Mehta VS, Garg A, Sarkar C, Ahmad FU.
2009 Russo N, Giangaspero F, Beccaglia MR, Santoro A.
2011 Zhang XJ, Piao YS, Chen L, M Tang GC, Wei LF, Yang H, Lu DH. F 2012 Catalucci A, Lanni G, Ventura L, M Ricci A, Galzio RJ, Gallucci M. 26 57
15
67
F
1999 Udono H, Fukuyama K, Okamoto H, Tabuchi K.
38
M
1996 Resnick DK, Johnson BL, Lovely TJ.
50
30
Bifrontal craniotomy to remove frontal convexity mass
Parietal craniotomy for the complete removal of both lesions Left retromastoid craniectomy
Subfrontal craniotomy
Treatment
Stable in 20 months follow-up
Stable for 2 years
Recovery well 6 months later
Treated with gentamycin, streptomucin and prednisone, no improvement
Follow-up
No detailed information No detailed information No detailed information 6-year history of progressive Multiple, bilateral extra-axial enhancing Resection of infratentorial right visual and hearing loss lesions, seven extra-axial and one petroclival right mass, through and tinnitus intraventricular lesion a right transpetrosal approach
No detailed information Not provided
1-year follow-up, MRI confirmed no recurrent lesion 14 months follow up, free of neurological symptoms No detailed information
(Paper in Japanese) Suprasellar region, right temporal Subtotal removal of the suprasellar No recurrence for 2 years convexity, left frontal convexity, left lesion, gamma knife cerebellopontine angle and C5 level radiosurgery to residual lesion of the spinal cord and the other intracranial lesions A lesion at the planum sphenoidale and Right orbitopterional craniotomy, Maintains full functional tuberculum sella compressing the optic nerve decompression abilities 10 months optic nerves, bilateral lesions from postoperation the cerebellopontine angle to the foramen magnum compressing the brainstem and a lesion at C-2 Bilateral petroclival Two stages craniotomy to remove 12 months follow-up, vision has tumours improved to 6/18 in both eyes and the cerebellar signs have resolved completely
Right frontal parasagittal lesion, multiple satellite lesions and an extracranial lesion in the pharynx
Left cerebellopontine angle, three frontal lesion in both sides
Two lesions in the right parietal convexity
Skull base of frontal, middle and posterior fossa in both sides
Site involved
Headache with vomiting and progressive visual deterioration in both eyes (left more than right) for 4 months Mild, slowly progressing upper Left frontal lesion measuring about 2.5 cm Elective frontal craniotomy right-arm paresis for 4 days in diameter, adherent to the dura 2 months of headache, Bilateral frontal lesions adhered to the Craniotomy to resect the two masses suddenly worsened convexity dura mater No detailed information No detailed information No detailed information
Decreased vision in right eye and diminished hearing in right ear
Bilateral visual impairment
6 years of seizure, loss of vision and hearing, facial palsy, speech difficulty and failing memory Left homonymous hemianopia and a left hemisensory inattention in the arm Transient visual loss, headaches, vomiting, deafness in left ear 3-week history of diplopia and headache
Gender Age Symptoms (years)
1989 Song SK, Schwartz IS, M Strauchen JA, Huang YP, Sachdev V, Daftary DR, Vas CJ. 1995 Kim M, Provias J, Bernstein M. M
Year Author
Table 1 Summary of intracranial Rosai–Dorfman’s disease mimicking multiple meningiomas
320 Childs Nerv Syst (2015) 31:317–323
A smooth postoperative course Resection of the large frontal lesion 43 M
A few months history of The left frontal region, 2 small lesions headache and mild dizziness with falcine attachment, a lesion in the vicinty of the anterior clinoid process and one attached to the anterosuperior border of the right petrous bone.
38 M
2013 Joubert C, Dagain A, Faivre A, Nguyen AT, Fesselet J, Figarella-Branger D. 2013 Abdel-Razek M, Matter GA, Azab WA, Katchy KC, Mallik AA.
Headache and frontal syndrome
Multiple extra-axial lesions, disseminated
Excision of the peripherally MRI 12 months demonstrated located left frontal lesion, 5-day resolution of all three lesions course of postoperative dexamethasone (Paper in French) (Paper in French) Intrinsic left frontal lobe, and right parietal and right inferior frontal white matter 31
Two focal seizures
321
2012 Camp SJ, Roncaroli F, F Apostolopoulos V, Weatherall M, Lim S, Nandi D.
Year Author
Table 1 (continued)
Gender Age Symptoms (years)
Site involved
Treatment
Follow-up
Childs Nerv Syst (2015) 31:317–323
region demonstrated endocrine disturbances, such as polydipsia, polyuria and amenorrhea. Our patient experienced headaches, fever and an increased head circumference caused by chronic elevated intracranial pressure. Clinical examinations of patients with RDD previously identified leucocytosis with neutrophilia, polyclonal hypergammaglobulinaemia, increased ESR and normocytic or microcytic anaemia [1, 5–8, 10, 11, 36, 37]. Imaging results are important for diagnosis of RDD and for exclusion of differential diagnoses. The typical imaging appearance of intracranial RDD is that of dural-based, extraaxial, well-circumscribed enhancing masses with possible perilesional cerebral oedema mimicking meningioma, together with mass effects involving contiguous structures and the midline [16, 26, 38–41]. On CT scans, lesions may appear as a homogeneous and lobulated isodense or hyperdense masses with marked contrast enhancement, and the adjacent bone might be eroded. On T1-weighted MRI, RDD lesions are hyperintense or isointense with clear borders hypointense relative to the brain parenchyma, while on T2-weighted images they have an isointense signal with possible intralesional foci of low signal intensity. MRI scans show strong inhomogeneous or homogeneous enhancement after gadolinium injection and demonstrate dural tail sign in all cases [26, 39–41]. Thus, our case closely mimics meningiomas clinically and radiologically, in agreement with other cases reported in the literature [2, 3, 42, 43]. Differential diagnosis may include other pathologies characterized by dural involvement and strong enhancement following contrast administration, such as meningioangiomatosis [38, 44], dural metastases [30], Wegener’s granulomatosis, sarcoidosis, Hodgkin’s lymphoma, inflammatory pseudotumour and Langerhans cell histiocytosis. Diagnosis can only be confirmed by histopathological or immunohistochemical examinations. Typically, histiocytes stain positive for S-100 protein and CD68, but negative for CD1a. Emperipolesis, signifying phagocytosis of autologous lymphocytes, is characteristic of RDD, but is present in only 70 % of cases [45]. RDD is generally considered a benign process that is typically self-limiting and often resolves spontaneously. However, the disease may be persistent and progressive [22]. Treatment options for RDD remain controversial. Various treatment modalities, including surgery, steroids, chemotherapy and radiation therapy, have been tried. Surgical resection is essential for diagnosis [8, 46]. It is also the most effective method of treatment and may be curative [1, 5–12, 15, 19, 29, 47, 48]. Surgery can relieve compression of vital structures, alleviate local damage and preserve function. RDD lesions in the CNS are firm, lobular, whitish-grey or yellowish-tan in colour and adherent to the dura. However, many lesions are difficult to resect completely because lesions
322
can adhere tightly to the cerebral cortex and may invade or surround other critical structures. The extent of surgical resection is strongly correlated with intracranial relapse of the disease [12, 29]. Petzold et al. [12] found intracranial tumour regrowth or recurrence of symptoms in 14 % of 29 patients with a mean follow-up period of 10.1 years. Postoperative treatment efforts should be directed at relapsing cases or those with subresected lesions. Corticosteroid therapy has been proposed and chemotherapy is another modality used to manage RDD, in cases of extensive or progressive disease. Jabali et al. [49] report that a combination of prednisone, 6-mercaptopurine, methotrexate and vinblastine was an effective and safe treatment for the disease. Radiation therapy has been employed for local disease control in such areas as the spinal cord and airway, or in cases of relapse [6, 12, 29, 43].
Conclusion For paediatric patients with multiple extra-axial lesions, a possible differential diagnosis of intracranial RDD should be considered and further investigations performed. Resection of the intracranial lesion is the most effective treatment. A definitive diagnosis should be based on histopathologic and immunocytochemical examinations. Furthermore, long-term follow-ups are required. Future efforts should be directed at investigating the aetiology of RDD and postoperative treatments for relapse or residual lesions. Acknowledgements Dr Yongji Tian (first author) was partially supported by the National Natural Science Foundation of China (No. 30900479), Beijing Nova-Plan Program (No. 2010B121), National Key Technology Research and Development Program of the Ministry of Science and Technology of China (2013BAI09B03) and BIBDPXM2013_014226_07_000084. Ethical standards This study was approved by the appropriate ethics committee and was performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki. Conflict of interest All authors declare that they have no conflicting interests and no authors were supported or funded by any drug company.
References 1. Foucar E, Rosai J, Dorfman R (1990) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 7:19–73 2. Konishi E, Ibayashi N, Yamamoto S, Scheithauer BW (2003) Isolated intracranial Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). AJNR Am J Neuroradiol 24:515–518 3. Wu M, Anderson AE, Kahn LB (2001) A report of intracranial RosaiDorfman disease with literature review. Ann Diagn Pathol 5:96–102
Childs Nerv Syst (2015) 31:317–323 4. Wang Y, Gao X, Tang W, Jiang C (2010) Rosai-Dorfman disease isolated to the central nervous system: a report of six cases. Neuropathology: Off J Jpn Soc Neuropathol 30:154–158 5. Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV (2001) Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol: Off J US Can Acad Pathol, Inc 14: 172–178 6. Rosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol Lab Med 87:63–70 7. Foucar E, Rosai J, Dorfman RF (1978) Sinus histiocytosis with massive lymphadenopathy. Arch Otolaryngol 104:687–693 8. Adeleye AO, Amir G, Fraifeld S, Shoshan Y, Umansky F, Spektor S (2010) Diagnosis and management of Rosai-Dorfman disease involving the central nervous system. Neurol Res 32:572–578 9. Türe U, Seker A, Bozkurt SU, Uneri C, Sav A, Pamir MN (2004) Giant intracranial Rosai-Dorfman disease. J Clin Neurosci: Off J Neurosurg Soc Aust 11:563–566 10. Said R, Abi-Fadel F, Talwar J, Attallah JP, Dilawari A (2011) Intracranial Rosai-Dorfman: a clinical challenge. Neurologist 17: 117–119 11. Eiras Jda C, Schettini A, Lima LL, Tubilla LH, Oliveira RM (2010) Cutaneous Rosai-Dorfman disease: a case report. An Bras Dermatol 85:687–690 12. Petzold A, Thom M, Powell M, Plant GT (2001) Relapsing intracranial Rosai-Dorfman disease. J Neurol Neurosurg Psychiatr 71:538– 541 13. Middel P, Hemmerlein B, Fayyazi A, Kaboth U, Radzun HJ (1999) Sinus histiocytosis with massive lymphadenopathy: evidence for its relationship to macrophages and for a cytokine-related disorder. Histopathology 35:525–533 14. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES (1992) Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis 166:291–295 15. Gupta DK, Suri A, Mahapatra AK et al (2006) Intracranial Rosai–Dorfman disease in a child mimicking bilateral giant petroclival meningiomas: a case report and review of literature. Child's Nerv Syst: ChNS: Off J Int Soc Pediatr Neurosurg 22: 1194–1200 16. Theeler BJ, Keylock JB, Yoest SM (2008) Teaching NeuroImage: isolated intracranial Rosai-Dorfman disease mimicking a meningioma. Neurology 70:e42 17. Shaver EG, Rebsamen SL, Yachnis AT, Sutton LN (1993) Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy. Case report. J Neurosurg 79:769–773 18. Woodcock RJ Jr, Mandell J, Lipper MH (1999) Sinus histiocytosis (Rosai-Dorfman disease) of the suprasellar region: MR imaging findings—a case report. Radiology 213:808–810 19. Griffiths SJ, Tang W, Parameswaran R, Kelsey A, West CG (2004) Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. Br J Neurosurg 18:293–297 20. Lungren MP, Petrella JR, Cummings TJ, Grant GA (2009) Isolated intracranial Rosai-Dorfman disease in a child. AJNR Am J Neuroradiol 30:E148–149 21. Gupta K, Bagdi N, Sunitha P, Ghosal N (2011) Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child: a case report and review of the literature. Br J Radiol 84:e138–141 22. Di Rocco F, Garnett MR, Puget S, Pueyerredon F, Roujeau T, Jaubert F, Sainte-Rose C (2007) Cerebral localization of Rosai-Dorfman disease in a child. Case report. J Neurosurg 107:147–151 23. Song SK, Schwartz IS, Strauchen JA, Huang YP, Sachdev V, Daftary DR, Vas CJ (1989) Meningeal nodules with features of extranodal sinus histiocytosis with massive lymphadenopathy. Am J Surg Pathol 13:406–412
Childs Nerv Syst (2015) 31:317–323 24. Kim M, Provias J, Bernstein M (1995) Rosai-Dorfman disease mimicking multiple meningioma: case report. Neurosurgery 36:1185–1187 25. Resnick DK, Johnson BL, Lovely TJ (1996) Rosai-Dorfman disease presenting with multiple orbital and intracranial masses. Acta Neuropathologica 91:554–557 26. Udono H, Fukuyama K, Okamoto H, Tabuchi K (1999) RosaiDorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report. J Neurosurg 91:335–339 27. Chen KT (2003) Crush cytology of Rosai-Dorfman disease of the central nervous system. A report of 2 cases. Acta Cytologica 47: 1111–1115 28. Sato A, Sakurada K, Sonoda Y, Saito S, Kayama T, Jokura H, Yoshimoto T, Nakazato Y (2003) Rosai-Dorfman disease presenting with multiple intracranial and intraspinal masses: a case report. No Shinkei Geka Neurol Surg 31:1199–1204 29. McPherson CM, Brown J, Kim AW, DeMonte F (2006) Regression of intracranial Rosai-Dorfman disease following corticosteroid therapy. Case report. J Neurosurg 104:840–844 30. Russo N, Giangaspero F, Beccaglia MR, Santoro A (2009) Intracranial dural histiocytosis. Br J Neurosurg 23:449–454 31. Zhang XJ, Piao YS, Chen L, Tang GC, Wei LF, Yang H, Lu DH (2011) Multiple intracranial lesions: a clinicalpathologic study of 62 cases. Zhonghua Bing Li Xue Za Zhi Chin J Pathol 40:599–603 32. Catalucci A, Lanni G, Ventura L, Ricci A, Galzio RJ, Gallucci M (2012) A rare case of intracranial Rosai-Dorfman disease mimicking multiple meningiomas. A case report and review of the literature. Neuroradiol J 25:569–574 33. Camp SJ, Roncaroli F, Apostolopoulos V, Weatherall M, Lim S, Nandi D (2012) Intracerebral multifocal Rosai-Dorfman disease. J Clin Neurosc: Off J Neurosurg Soc Australas 19:1308–1310 34. Joubert C, Dagain A, Faivre A, Nguyen AT, Fesselet J, FigarellaBranger D (2013) Intracranial Rosai-Dorfman disease mimicking multiple meningiomas. Rev Med Brux 34:112–114 35. Abdel-Razek M, Matter GA, Azab WA, Katchy KC, Mallik AA (2013) Isolated intracranial Rosai-Dorfman disease: report of two cases and a review of the literature. Turk Neurosurg 23:509–513 36. Foucar E, Rosai J, Dorfman RF, Brynes RK (1982) The neurologic manifestations of sinus histiocytosis with massive lymphadenopathy. Neurology 32:365–372 37. Gaitonade S (2007) Multifocal, extranodal sinus histiocytosis with massive lymphadenopathy: an overview. Arch Pathol Lab Med 131: 1117–1121
323 38. Chen YY, Tiang XY, Li Z, Luo BN, Huang Q (2010) Sporadic meningioangiomatosis-associated atypical meningioma mimicking parenchymal invasion of brain: a case report and review of the literature. Diagn Pathol 18:39 39. Raslan OA, Schellingerhout D, Fuller GN, Ketonen LM (2011) Rosai-Dorfman disease in neuroradiology: imaging findings in a series of 10 patients. AJR Am J Roentgenol 196:W187–193 40. Geara AR, Ayoubi MA, Achram MC, Chamseddine NM (2004) Rosai-Dorfman disease mimicking neurofibromatosis: case presentation and review of the literature. Clin Radiol 59:625–630 41. Toh CH, Lin CY, Wong HF, Wei KC, Ng SH, Wan YL (2005) RosaiDorfman disease with dural sinus invasion. Report of two cases. J Neurosurg 102:550–554 42. Sharma MS, Padua MD, Jha AN (2005) Rosai-Dorfman disease mimicking a sphenoid wing meningioma. Neurol India 53:110–111 43. Kidd DP, Revesz T, Miller NR (2006) Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement. Neurology 67:1551–1555 44. Kremer S, Grand S, Rémy C, Pasquier B, Benabid AL, Bracard S, Le Bas JF (2004) Contribution of dynamic contrast MR imaging to the differentiation between dural metastasis and meningioma. Neuroradiology 46:642–648 45. Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, Bucsky P, Egeler RM, Elinder G, Gadner H, Gresik M, Henter JI, Imashuku S, Janka-Schaub G, Jaffe R, Ladisch S, Nezelof C, Pritchard J (1997) Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol 29:157–166 46. Zhang JT, Tian HJ, Lang SY, Wang XQ (2010) Primary intracerebral Rosai-Dorfman disease. J Clin Neurosci: Off J Neurosurg Soc Australas 17:1286–1288 47. Wan S, Teng X, Zhan R, Yu J, Gu J, Zhang K (2008) Isolated intracranial Rosai-Dorfman disease mimicking suprasellar meningioma: case report with review of the literature. J Int Med Res 36:1134– 1139 48. Gaetani P, Tancioni F, Di Rocco M, Rodriguez y Baena R (2000) Isolated cerebellar involvement in Rosai-Dorfman disease: case report. Neurosurgery 46:479–481 49. Jabali Y, Smrcka V, Pradna J (2005) Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report. Int J Surg Pathol 13:285–289
