- Email: [email protected]
Rosai-Dorfman Disease of Rare Isolated Spinal Involvement: Report of 4 Cases and Literature Review
Case Report
Rosai-Dorfman Disease of Rare Isolated Spinal Involvement: Report of 4 Cases and Literature Review Bo Yuan Huang1, Hua Zhang2, Wen Jing Zong3, Yan Hui Sun2
Key words Isolated spinal involvement - Rosai-Dorfman disease -
Abbreviations and Acronyms CNS: Central nervous system EMA: epithelial membrane antigen LCH: Langerhans cell histiocytosis MRI: Magnetic resonance imaging RDD: Rosai-Dorfman disease From the 1Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing; 2Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing; and 3College of Traditional Chinese Medicine, Capital Medical University, Beijing, China To whom correspondence should be addressed: Yan Hui Sun, M.D.; Hua Zhang [E-mail: [email protected]; [email protected]] Citation: World Neurosurg. (2016) 85:367.e11-367.e16. http://dx.doi.org/10.1016/j.wneu.2015.09.097 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2016 Elsevier Inc. All rights reserved.
INTRODUCTION Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), was first reported by Rosai and Dorfman in 1969.1 RDD is a rare idiopathic histioproliferative disorder that is generally characterized with bilateral massive but painless cervical lymphadenopathy. Patients with RDD may also have other concomitant syndromes, such as fever, neutrophilia, increased sedimentation rate, and polyclonal hypergammaglobulinemia. In 30% of cases of RDD, extranodal involvement can be found, including the skin, orbit, upper respiratory tract, and bones.2 Central nervous system (CNS) involvements are rare and are usually associated with the dura or skull base.3 Isolated spinal RDD without other body involvements is even rarer. In this article, 4 cases of isolated spinal RDD are reported.
- BACKGROUND:
Rosai-Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system (CNS).
- CASE
DESCRIPTION: The diagnosis and treatment of 4 patients with isolated spinal RDD are discussed. All 4 patients were treated by total or subtotal surgical resection and none of them experienced recurrence. Histopathologic examination showed a characteristic emperipolesis, and the lymphocytes were engulfed in the S-100-protein-positive histiocytes with no expression of CD1a.
- CONCLUSIONS:
Preoperative diagnosis of spinal RDD is still challenging because the lesion is usually a dura-based lesion that mimics a meningioma. Surgical resection is an effective treatment, and radiotherapy, steroid therapy, and chemotherapy have not shown reliable therapeutic efficiency.
CASE REPORT Case 1 A 55-year-old man presented with a history of limb numbness for 1 month. The patient initially experienced numbness in the upper limbs and shoulder coupled with chest pain 1 month previously. He then developed numbness and atony of the lower limbs and hypesthesia of the skin below the level of the abdomen as well as gait instability. Before coming to our hospital, he had been treated with dexamethasone and mannitol in a local hospital, which slightly relieved his symptoms. In our hospital, physical examination showed that the patient had a decreased bilateral skin superficial sensation below the nipple. The power of the upper limbs was grade 3/5 and the power of the lower limbs was 4/5, with tendon hyperreflexia. Muscular tension was normal and the pathologic reflex was negative. No massive lymphadenopathy was found in the cervical, supraclavicular, or axillary regions. Routine laboratory examinations were also normal. Neuroimaging results showed that the lesion was isointense on T1-weighted magnetic resonance imaging (MRI) and slightly hyperintense on T2-weighted MRI, with homogeneous enhancement at the T1-T9 level of the epidural space (Figure 1AeD). On the basis of the patient’s clinical history and neuroimaging results, he was
WORLD NEUROSURGERY 85: 367.e11-367.e16, JANUARY 2016
preoperatively diagnosed as having lymphoma. Thus, a surgical plan of subtotal resection was made for pathologic diagnosis and intraspinal canal decompression. During the operation, a laminectomy of T1-T3 was performed and a dura-based pinkish-grey mass was found in the subdural space. The mass, which had adhered to the surrounding tissue, causing the surrounding arachnoid to disappear, was subtotally excised under a microscope. Pathologic examination after surgery showed that the lesion was composed of fibrous tissue infiltrated by lymphocytes, plasmocytes, and histiocytes. Characteristic emperipolesis, a phenomenon of wellpreserved lymphocytes engulfed by histiocytes, was also found (Figure 1F). Immunologic results showed that the lesion was positively expressed by S-100 protein, CD68, CD3, k, and l as well as focal positive expression of CD20 and MUM-1, with negative expression of CD1a. The Ki67 labeling index of the lesion was 20%e30%. The patient was pathologically diagnosed as having RDD. Postoperatively, the patient’s symptoms were partly relieved. The limb numbness was relieved and the power of the upper limbs recovered to grade 5/5. However, the power of the lower limbs remained at grade 4/5, with decreased bilateral skin superficial sensation. The patient refused radiotherapy and
www.WORLDNEUROSURGERY.org
367.E11
CASE REPORT BO YUAN HUANG ET AL.
ROSAI-DORFMAN DISEASE
Figure 1. Neuroimaging and pathologic findings of case 1. (A) Sagittal T1-weighted magnetic resonance imaging (MRI) and (B) sagittal T2-weighted MRI showed that the lesion was isointense on T1-weighted and hyperintense on T2-weighted imaging at the T1-T9 level of the epidural space. (C) Sagittal postcontrast T1-weighted MRI and (D) coronal postcontrast T1-weighted MRI showed that the lesion was homogeneously enhanced. (E) Postoperative MRI showed that the lesion was subtotally excised. (F) Pathologic examination showed typical emperipolesis.
chemotherapy after surgery. The patient showed no progression over the next 6 months follow-up and had no recurrences. Case 2 A 40-year-old man presented with a history of neck and shoulder pain for 1 year and tumbling when walking for 6 months. On examination, he showed hypesthesia of the left limbs and trunk. The muscle power of the left leg was grade 3/5 and the muscle power of the right limbs was grade 5/5. Muscular tension was normal and the pathologic reflex was negative. His body temperature was high (37.6 C) and he had leukocytosis (11,500 white blood cells/mL, 68% neutrophils). He did not exhibit solitary palpable lymph nodes in the cervical, supraclavicular, or axillary regions. The neuroimaging results showed multiple mass lesions with homogeneous enhancement at the C3-C5 and C6 level of the epidural space, compressing the spinal cord (Figure 2AeD). On the basis of the patient’s clinical history and the neuroimaging results, he was preoperatively diagnosed as having spinal
367.E12
www.SCIENCEDIRECT.com
meningioma. During surgery, a laminectomy of C3-C6 was performed and 2 grayish firm lesions based on the dura were totally removed with intact dura. Pathologic examination of the lesions showed large pale histiocytes with numerous macrophages, focally distributed lymphocytes, and eosinophilic granulocytes. Emperipolesis, consisting of histiocytes and engulfed wellpreserved lymphocytes, was also found (Figure 2F). Immunologic results showed that the lesions were positively expressed by S-100 protein, with negative expression of CD1a. Accordingly, RDD was diagnosed based on the pathologic and immunologic results. Postoperatively, the patient’s preoperative symptoms were relieved, and thus, no radiotherapy or chemotherapy was given. The patient remained asymptomatic over the next 18 months follow-up and had no recurrences. Case 3 A 14-year-old girl came to our hospital with a history of bilateral leg pain for 4 months. On examination, she showed hypesthesia
of the right limbs. Her right knee showed a hypoactive knee jerk reflex. The muscle power of the limbs was grade 5 and muscle tone was normal. There was no evidence of massive lymphadenopathy in the cervical, supraclavicular, or axillary regions. Routine laboratory examinations were also normal. The neuroimaging results showed that the lesion was isointense on T1-weighted and T2-weighted MRI, with homogeneous enhancement at the S1-S2 level of the right intervertebral foramen (Figure 3AeD). On the basis of the patient’s clinical history and the neuroimaging results, she was preoperatively diagnosed as having a neurogenic tumor. The patient underwent a right-sided laminectomy of the S1-S2 vertebral plate. A pinkish-grey mass lesion of about 25 mm 13 mm 20 mm with intact dura was found in the S1-S2 intervertebral space. The lesion, which had adhered to the surrounding spinal nerve root, was totally removed under a microscope. Postoperatively, pathologic results showed an atypical lymphohistiocytic infiltration composed of CD68 and S-100 protein-positive histiocytes with large nuclei, prominent nucleoli and characteristic emperipolesis (Figure 2F). A diagnosis of RDD was established based on the pathologic results. Postoperatively, the patient’s symptoms were relieved and no radiotherapy or chemotherapy was given. The patient remained asymptomatic over the next 12 months follow-up and had no recurrences. Case 4 A 43-year-old man presented with a history of shoulder and back pain for 8 months coupled with numbness in the upper limbs for 2 months. On examination, the patient showed decreased superficial sensation of the skin in the bilateral shoulders, bilateral shoulder blades regions, and above the right upper part of the body. The muscle power of the limbs was grade 5 and muscle tone was normal. He did not have solitary palpable lymph nodes in the cervical, supraclavicular, or axillary regions. Routine laboratory examinations were also normal. Neuroimaging results showed a mass lesion of isointense on T1-weighted and T2-weighted MRI, with homogeneous enhancement at the C5-C6 level of the epidural space, compressing the spinal cord (Figure 4AeD). The patient was
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.09.097
CASE REPORT BO YUAN HUANG ET AL.
ROSAI-DORFMAN DISEASE
Figure 2. Neuroimaging and pathologic findings of case 2. (A) Sagittal T1-weighted magnetic resonance imaging (MRI) and (B) sagittal T2-weighted MRI showed that the lesion was multiple mass lesions isointense on T1-weighted and T2-weighted imaging at the C3-C5 and C6 level of the epidural space. (C) Sagittal postcontrast T1-weighted MRI and (D) coronal postcontrast T1-weighted MRI showed that the lesion was homogeneously enhanced. (E) Postoperative MRI showed that the lesion was totally excised. (F) Pathologic examination showed typical emperipolesis.
preoperatively diagnosed as having spinal meningioma. A laminectomy of C5-C6 was performed and a yellowish-white mass lesion of about 18 mm 13 mm 3 mm with intact dura was found in the subdural space. A dura-based lesion, which had slightly adhered to the surrounding tissue, causing the surrounding arachnoid to disappear, was totally excised with intact dura. Pathologic results showed typical emperipolesis, and immunologic results showed positive expression of CD68 and interspersed positive expression of lysozyme and S-100 protein with negative expression of epithelial membrane antigen (EMA) and CD1a (Figure 4F). Based on the pathologic and immunologic results, the patient was diagnosed as having RDD. Postoperatively, the patient’s preoperative symptoms were partially relieved and showed no progression over the next 12 months follow-up, with no recurrences. DISCUSSION RDD is a histioproliferative disorder that is characterized by bilateral cervical
lymphadenopathy, fever, neutrophilia, increased sedimentation rate, polyclonal hypergammaglobulinemia, and other extranodal involvements. RDD of the CNS without other extranodal involvements was observed in less than 5% of all patients with RDD.3 Furthermore, isolated spinal RDD was even rarer. A retrospective analysis showed that from 1969, when the disease was first described, to 2014, 210 cases of CNS involvement were reported, of which only 24 were isolated spinal RDD, compared with 167 cases of isolated intracranial involvements.4 Usually, intracranial RDD is an extra-axial durabased lesion (or multilesions) that mimics a dura-based meningioma.5-8 However, extremely rare cases of intraventricular and intraparenchymal lesions have also been reported.9-12 Similar to cases with intracranial involvements, most cases of isolated spinal RDD (22 cases) were also extramedullary dura-based lesions; only 2 cases were intramedullary lesions.4 In our cases, neuroimaging showed that 3 (cases 2, 3, and 4) were extramedullary lesions and the other (case 1) was an intramedullary lesion. Common symptoms of CNS
WORLD NEUROSURGERY 85: 367.e11-367.e16, JANUARY 2016
involvement are cephalgia, seizure, weakness, and paresthesia of the limbs and trunk, as well as cranial and spinal nerve deficiency based on the location of the lesions.4-8 CNS RDD is an uncommon entity and diagnostic neuroimaging usually shows a dura-based lesion. Accordingly, both intracranial and spinal RDD are commonly misdiagnosed as meningioma without biopsy. On T1-weighted MRI, intracranial or spinal RDD usually shows a single lesion or multiple well-defined mass lesions with isointense or hyperintense signal with distinct and homogeneous contrast enhancement. On T2-weighted imaging, the lesions usually present as an isointense signal.4-12 In a rare case, it was also reported that the lesion could be a high T2-signal mass and isointense on T1-weighted imaging with minimal contrast enhancement.8 In our cases, neuroimaging of 3 patients (cases 2, 3, and 4) showed extramedullary durabased lesions of isointense signal on T1-weighted and T2-weighted MRI and the other (case 1) showed an intramedullary lesion of isointense signal on T1-weighted MRI but a hyperintense signal on T2-weighted MRI. Accordingly, we assume that evidence of the lesion on T1-weighted and T2-weighted MRI may be associated with whether the lesion is extramedullary or intramedullary. The typical pathologic evidence of RDD is characterized by attenuated infiltration of lymphoplasma cells and pale histiocytes. These histiocytes are well defined, varied in size, and with round or oval vesicular nuclei, delicate nuclear membranes, and single prominent nucleoli. The emperipolesis, a characteristic pathologic sign of histiocytes engulfing well-preserved lymphocytes, can be seen in about 70% of RDD. On immunohistochemical examination, histiocytes in RDD are positively immunoreactive for CD68 and S-100 protein but negative for CD1a and EMA.3,13 The differential diagnosis of RDD includes meningioma, histiocytosis X, lymphoproliferative disorders, plasma cell granuloma, and infectious diseases. Both RDD and meningioma are dura-based lesions with homogeneous contrast enhancement, and even at microscopic levels, it is difficult to distinguish the 2 entities. During surgery, we found that the lesion had a distinct thickened and tough
www.WORLDNEUROSURGERY.org
367.E13
CASE REPORT BO YUAN HUANG ET AL.
ROSAI-DORFMAN DISEASE
Figure 3. Neuroimaging and pathologic findings of case 3. (A) Sagittal T1-weighted magnetic resonance imaging (MRI) and (B) sagittal T2-weighted MRI showed that the lesion was isointense on T1-weighted and T2-weighted MRI. (C) Sagittal postcontrast T1-weighted MRI and (D) axial postcontrast T1-weighted MRI showed that the lesion was homogeneously enhanced at the S1-S2 level of the right intervertebral foramen. (E) Postoperative MRI showed that the lesion was totally excised. (F) Pathologic examination showed typical emperipolesis.
dura. This was different from a meningioma and made us suspicious of the imaging diagnosis. The pathologic results substantiated our suspicion. Moreover, meningioma, which has positive EMA, can be readily differentiated by immunohistochemical staining. Plasma cell granuloma, characterizing a mixed inflammatory infiltrate with polyclonal plasma cells, is usually confused with RDD, because it can present as a discrete, dura-based inflammatory mass. However, emperipolesis and expression of S-100 protein is negative in the pathology of plasma cell granuloma. Langerhans cell histiocytosis (LCH) may also present as a dura-based mass. Furthermore, the S-100 protein-positive histiocytes can also be found in this lesion. However, LCH can usually be found on pathologic examination of characteristic longitudinal nuclear grooves, conspicuous eosinophils, and pathognomonic Birbeck granules, in the absence of lymphophagocytosis in histiocytes. Lymphoproliferative disorders may also be misdiagnosed as
367.E14
www.SCIENCEDIRECT.com
RDD because the histiocytes present a phagocytic phenomenon and positive S-100 protein. However, erythrophagocytosis can differentiate the lesion from RDD. As in our cases, the immunologic results of 3 patients (cases 1, 3, and 4) showed a concurrent positive expression of S-100 protein and CD68. The other patient (case 2), although her immunologic results showed negative expression of S-100 protein, was also diagnosed as having RDD based on the characteristic emperipolesis. Moreover, all 4 cases showed negative expression of CD1a in immunohistochemistry. The histiocytes in RDD show S-100 proteinpositive expression and absence of CD1a. As noted earlier, both RDD and LCH can present as positive expression of S-100 protein; however, CD1a, which is reliably expressed on histiocytes of LCH, is negative in RDD.3 Accordingly, immunologic staining of CD1a is also significant for the diagnosis of RDD. Generally, treatment of systemic RDD is advised only in patients with symptomatic
lesions or masses that threaten the function of the vital organs, because this disease is considered to be a benign, nonneoplastic lymphoproliferative disorder.4 Furthermore, complete spontaneous resolution has been reported in about 20% of cases of systemic RDD.14 However, in CNS RDD, complete spontaneous resolution has not been reported. Accordingly, surgery is still the first choice of treatment of intracranial and spinal RDD for both diagnostic purposes and rapid improving neurologic symptoms.15,16 Complete resection should be attempted, and when lesions are adhered to contiguous critical structures, subtotal resection should also be performed for pathologic diagnosis and symptomatic remission. After surgery, most patients’ disease stabilizes and a wait-and-watch treatment is advised.17 Radiotherapy, chemotherapy, and corticosteroid therapy have been given to patients whose neurologic symptoms were not relieved after surgery.18-22 Both fractionated radiotherapy and stereotactic radiotherapy have been used in CNS RDD after surgery; however, results varied and some patients showed no improvement.17,20,23 Moreover, although steroid treatment has had some therapeutic benefit on the treatment of patients with systemic RDD with rapid growing lymph node or other constitutional symptoms and some patients with CNS involvement,17,20,24 randomized trials are still needed to support its efficiency. Chemotherapy has also been adopted in systemic RDD and CNS RDD. Pulsoni et al.25 retrospectively analyzed therapeutic outcomes of 12 patients with systemic RDD who were treated with anthracyclines, vinca alkaloids, and alkylating agents. Only 2 patients achieved complete relief. Rivera et al.22 adopted a CHOP (cyclophosphamide, vincristine, doxorubicin, and prednisone) regimen to treat 2 patients with recurrent intracranial RDD. Both patients experienced recurrence within 2 years after the first subtotal resection surgery. After the second treatment of chemotherapy, they were free from recurrence for 7 years.22 Although adjuvant chemotherapy has been recommended after subtotal resection,22,26 clinical trials are still needed to establish the therapeutic approaches. In our cases, the lesions were totally or subtotally removed by surgery and no
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.09.097
CASE REPORT BO YUAN HUANG ET AL.
ROSAI-DORFMAN DISEASE
Dorfman granuloma mimicking LhermitteDuclos disease. Case report. J Neurosurg Pediatr. 2009;4:118-120. 9. Juric G, Jakic-Razumovic J, Rotim K, Zarkovic K. Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma. Acta Neurochir (Wien). 2003;145:145-149. 10. Gaetani P, Tancioni F, Di Rocco M, Rodriguez y Baena R. Isolated cerebellar involvement in RosaiDorfman disease: case report. Neurosurgery. 2000; 46:479-481. 11. Natarajan S, Post KD, Strauchen J, Morgello S. Primary intracerebral Rosai-Dorfman disease: a case report. J Neurooncol. 2000;47:73-77. 12. Morandi X, Godey B, Riffaud L, Heresbach N, Brassier G. Isolated Rosai-Dorfman disease of the fourth ventricle. Case illustration. J Neurosurg. 2000;92:890. 13. Tubbs RS, Kelly DR, Mroczek-Musulman EC, Hammers YA, Berkow RL, Oakes WJ, et al. Spinal cord compression as a result of Rosai-Dorfman disease of the upper cervical spine in a child. Childs Nerv Syst. 2005;21:951-954. 14. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7:19-73.
Figure 4. Neuroimaging and pathologic findings of case 4. (A) Sagittal T1-weighted magnetic resonance imaging (MRI) and (B) sagittal T2-weighted MRI showed that the lesion was isointense on T1-weighted and T2-weighted imaging at the C5-C6 level of the epidural space. (C) Sagittal postcontrast T1-weighted MRI and (D) coronal postcontrast T1-weighted MRI showed that the lesion was homogeneously enhanced. (E) Postoperative MRI showed that the lesion was totally excised. (F) Pathologic examination showed typical emperipolesis.
recurrences were observed after 6e18 months follow-up.
CONCLUSIONS Isolated spinal RDD is an exceedingly rare disease and its diagnosis is still challenging. Histologic evidence and immunophenotypical characteristics are still the only reliable basis for diagnosis. Surgical resection has been shown to be an effective treatment approach, and other treatments such as radiotherapy, steroid therapy, and chemotherapy need further research to clarify usage and efficiency. REFERENCES 1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol. 1969;87:63-70. 2. Symss NP, Cugati G, Vasudevan MC, Ramamurthi R, Pande A. Intracranial Rosai
15. Kayali H, Onguru O, Erdogan E, Sirin S, Timurkaynak E. Isolated intracranial RosaiDorfman disease mimicking meningioma. Clin Neuropathol. 2004;23:204-208.
Dorfman disease: report of three cases and literature review. Asian J Neurosurg. 2010;5:19-30. 3. Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV. Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol. 2001;14:172-178. 4. Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, Velazquez-Vega J, Borja MJ, Rosenberg S, et al. Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. Medicine (Baltimore). 2014;93: 165-175. 5. Kim M, Provias J, Bernstein M. Rosai-Dorfman disease mimicking multiple meningioma: case report. Neurosurgery. 1995;36:1185-1187. 6. Simos M, Dimitrios P, Philip T. A new clinical entity mimicking meningioma diagnosed pathologically as Rosai-Dorfman disease. Skull Base Surg. 1998;8:87-92. 7. Di Rocco F, Garnett MR, Puget S, Pueyerredon F, Roujeau T, Jaubert F, et al. Cerebral localization of Rosai-Dorfman disease in a child. Case report. J Neurosurg. 2007;107(2 Suppl):147-151. 8. Johnston JM, Limbrick DD, Ray WZ, Brown S, Shimony J, Park TS. Isolated cerebellar Rosai-
WORLD NEUROSURGERY 85: 367.e11-367.e16, JANUARY 2016
16. Griffiths SJ, Tang W, Parameswaran R, Kelsey A, West CG. Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. Br J Neurosurg. 2004;18:293-297. 17. Cooper SL, Jenrette JM. Rosai-Dorfman disease: management of CNS and systemic involvement. Clin Adv Hematol Oncol. 2012;10:199-202. 18. Franco-Paredes C, Martin K. Extranodal RosaiDorfman disease involving the meninges. South Med J. 2002;95:1101-1102. 19. Sato A, Sakurada K, Sonoda Y, Saito S, Kayama T, Jokura H, et al. Rosai-Dorfman disease presenting with multiple intracranial and intraspinal masses: a case report. No Shinkei Geka. 2003;31:1199-1204. 20. Hadjipanayis CG, Bejjani G, Wiley C, Hasegawa T, Maddock M, Kondziolka D. Intracranial Rosai-Dorfman disease treated with microsurgical resection and stereotactic radiosurgery. Case report. J Neurosurg. 2003;98:165-168. 21. Hinduja A, Aguilar LG, Steineke T, Nochlin D, Landolfi JC. Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion. J Neurooncol. 2009;92:117-120. 22. Rivera D, Pérez-Castillo M, Fernández B, Stoeter P. Long-term follow-up in two cases of intracranial Rosai-Dorfman disease complicated by incomplete resection and recurrence. Surg Neurol Int. 2014;5:30.
www.WORLDNEUROSURGERY.org
367.E15
CASE REPORT BO YUAN HUANG ET AL.
23. Kidd DP, Revesz T, Miller NR. Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement. Neurology. 2006;67: 1551-1555. 24. Shaver EG, Rebsamen SL, Yachnis AT, Sutton LN. Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy. Case report. J Neurosurg. 1993; 79:769-773. 25. Pulsoni A, Anghel G, Falcucci P, Matera R, Pescarmona E, Ribersani M, et al. Treatment of sinus histiocytosis with massive lymphadenopathy
367.E16
www.SCIENCEDIRECT.com
ROSAI-DORFMAN DISEASE
(Rosai-Dorfman disease): report of a case and literature review. Am J Hematol. 2002;69:67-71. 26. Le Guenno G, Galicier L, Uro-Coste E, Petitcolin V, Rieu V, Ruivard M. Successful treatment with azathioprine of relapsing RosaiDorfman disease of the central nervous system. J Neurosurg. 2012;117:486-489.
commercial or financial relationships that could be construed as a potential conflict of interest. Received 26 August 2015; accepted 26 September 2015 Citation: World Neurosurg. (2016) 85:367.e11-367.e16. http://dx.doi.org/10.1016/j.wneu.2015.09.097 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2016 Elsevier Inc. All rights reserved.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.09.097
Rosai-Dorfman Disease of Rare Isolated Spinal Involvement: Report of 4 Cases and Literature Review Bo Yuan Huang1, Hua Zhang2, Wen Jing Zong3, Yan Hui Sun2
Key words Isolated spinal involvement - Rosai-Dorfman disease -
Abbreviations and Acronyms CNS: Central nervous system EMA: epithelial membrane antigen LCH: Langerhans cell histiocytosis MRI: Magnetic resonance imaging RDD: Rosai-Dorfman disease From the 1Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing; 2Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing; and 3College of Traditional Chinese Medicine, Capital Medical University, Beijing, China To whom correspondence should be addressed: Yan Hui Sun, M.D.; Hua Zhang [E-mail: [email protected]; [email protected]] Citation: World Neurosurg. (2016) 85:367.e11-367.e16. http://dx.doi.org/10.1016/j.wneu.2015.09.097 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2016 Elsevier Inc. All rights reserved.
INTRODUCTION Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), was first reported by Rosai and Dorfman in 1969.1 RDD is a rare idiopathic histioproliferative disorder that is generally characterized with bilateral massive but painless cervical lymphadenopathy. Patients with RDD may also have other concomitant syndromes, such as fever, neutrophilia, increased sedimentation rate, and polyclonal hypergammaglobulinemia. In 30% of cases of RDD, extranodal involvement can be found, including the skin, orbit, upper respiratory tract, and bones.2 Central nervous system (CNS) involvements are rare and are usually associated with the dura or skull base.3 Isolated spinal RDD without other body involvements is even rarer. In this article, 4 cases of isolated spinal RDD are reported.
- BACKGROUND:
Rosai-Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system (CNS).
- CASE
DESCRIPTION: The diagnosis and treatment of 4 patients with isolated spinal RDD are discussed. All 4 patients were treated by total or subtotal surgical resection and none of them experienced recurrence. Histopathologic examination showed a characteristic emperipolesis, and the lymphocytes were engulfed in the S-100-protein-positive histiocytes with no expression of CD1a.
- CONCLUSIONS:
Preoperative diagnosis of spinal RDD is still challenging because the lesion is usually a dura-based lesion that mimics a meningioma. Surgical resection is an effective treatment, and radiotherapy, steroid therapy, and chemotherapy have not shown reliable therapeutic efficiency.
CASE REPORT Case 1 A 55-year-old man presented with a history of limb numbness for 1 month. The patient initially experienced numbness in the upper limbs and shoulder coupled with chest pain 1 month previously. He then developed numbness and atony of the lower limbs and hypesthesia of the skin below the level of the abdomen as well as gait instability. Before coming to our hospital, he had been treated with dexamethasone and mannitol in a local hospital, which slightly relieved his symptoms. In our hospital, physical examination showed that the patient had a decreased bilateral skin superficial sensation below the nipple. The power of the upper limbs was grade 3/5 and the power of the lower limbs was 4/5, with tendon hyperreflexia. Muscular tension was normal and the pathologic reflex was negative. No massive lymphadenopathy was found in the cervical, supraclavicular, or axillary regions. Routine laboratory examinations were also normal. Neuroimaging results showed that the lesion was isointense on T1-weighted magnetic resonance imaging (MRI) and slightly hyperintense on T2-weighted MRI, with homogeneous enhancement at the T1-T9 level of the epidural space (Figure 1AeD). On the basis of the patient’s clinical history and neuroimaging results, he was
WORLD NEUROSURGERY 85: 367.e11-367.e16, JANUARY 2016
preoperatively diagnosed as having lymphoma. Thus, a surgical plan of subtotal resection was made for pathologic diagnosis and intraspinal canal decompression. During the operation, a laminectomy of T1-T3 was performed and a dura-based pinkish-grey mass was found in the subdural space. The mass, which had adhered to the surrounding tissue, causing the surrounding arachnoid to disappear, was subtotally excised under a microscope. Pathologic examination after surgery showed that the lesion was composed of fibrous tissue infiltrated by lymphocytes, plasmocytes, and histiocytes. Characteristic emperipolesis, a phenomenon of wellpreserved lymphocytes engulfed by histiocytes, was also found (Figure 1F). Immunologic results showed that the lesion was positively expressed by S-100 protein, CD68, CD3, k, and l as well as focal positive expression of CD20 and MUM-1, with negative expression of CD1a. The Ki67 labeling index of the lesion was 20%e30%. The patient was pathologically diagnosed as having RDD. Postoperatively, the patient’s symptoms were partly relieved. The limb numbness was relieved and the power of the upper limbs recovered to grade 5/5. However, the power of the lower limbs remained at grade 4/5, with decreased bilateral skin superficial sensation. The patient refused radiotherapy and
www.WORLDNEUROSURGERY.org
367.E11
CASE REPORT BO YUAN HUANG ET AL.
ROSAI-DORFMAN DISEASE
Figure 1. Neuroimaging and pathologic findings of case 1. (A) Sagittal T1-weighted magnetic resonance imaging (MRI) and (B) sagittal T2-weighted MRI showed that the lesion was isointense on T1-weighted and hyperintense on T2-weighted imaging at the T1-T9 level of the epidural space. (C) Sagittal postcontrast T1-weighted MRI and (D) coronal postcontrast T1-weighted MRI showed that the lesion was homogeneously enhanced. (E) Postoperative MRI showed that the lesion was subtotally excised. (F) Pathologic examination showed typical emperipolesis.
chemotherapy after surgery. The patient showed no progression over the next 6 months follow-up and had no recurrences. Case 2 A 40-year-old man presented with a history of neck and shoulder pain for 1 year and tumbling when walking for 6 months. On examination, he showed hypesthesia of the left limbs and trunk. The muscle power of the left leg was grade 3/5 and the muscle power of the right limbs was grade 5/5. Muscular tension was normal and the pathologic reflex was negative. His body temperature was high (37.6 C) and he had leukocytosis (11,500 white blood cells/mL, 68% neutrophils). He did not exhibit solitary palpable lymph nodes in the cervical, supraclavicular, or axillary regions. The neuroimaging results showed multiple mass lesions with homogeneous enhancement at the C3-C5 and C6 level of the epidural space, compressing the spinal cord (Figure 2AeD). On the basis of the patient’s clinical history and the neuroimaging results, he was preoperatively diagnosed as having spinal
367.E12
www.SCIENCEDIRECT.com
meningioma. During surgery, a laminectomy of C3-C6 was performed and 2 grayish firm lesions based on the dura were totally removed with intact dura. Pathologic examination of the lesions showed large pale histiocytes with numerous macrophages, focally distributed lymphocytes, and eosinophilic granulocytes. Emperipolesis, consisting of histiocytes and engulfed wellpreserved lymphocytes, was also found (Figure 2F). Immunologic results showed that the lesions were positively expressed by S-100 protein, with negative expression of CD1a. Accordingly, RDD was diagnosed based on the pathologic and immunologic results. Postoperatively, the patient’s preoperative symptoms were relieved, and thus, no radiotherapy or chemotherapy was given. The patient remained asymptomatic over the next 18 months follow-up and had no recurrences. Case 3 A 14-year-old girl came to our hospital with a history of bilateral leg pain for 4 months. On examination, she showed hypesthesia
of the right limbs. Her right knee showed a hypoactive knee jerk reflex. The muscle power of the limbs was grade 5 and muscle tone was normal. There was no evidence of massive lymphadenopathy in the cervical, supraclavicular, or axillary regions. Routine laboratory examinations were also normal. The neuroimaging results showed that the lesion was isointense on T1-weighted and T2-weighted MRI, with homogeneous enhancement at the S1-S2 level of the right intervertebral foramen (Figure 3AeD). On the basis of the patient’s clinical history and the neuroimaging results, she was preoperatively diagnosed as having a neurogenic tumor. The patient underwent a right-sided laminectomy of the S1-S2 vertebral plate. A pinkish-grey mass lesion of about 25 mm 13 mm 20 mm with intact dura was found in the S1-S2 intervertebral space. The lesion, which had adhered to the surrounding spinal nerve root, was totally removed under a microscope. Postoperatively, pathologic results showed an atypical lymphohistiocytic infiltration composed of CD68 and S-100 protein-positive histiocytes with large nuclei, prominent nucleoli and characteristic emperipolesis (Figure 2F). A diagnosis of RDD was established based on the pathologic results. Postoperatively, the patient’s symptoms were relieved and no radiotherapy or chemotherapy was given. The patient remained asymptomatic over the next 12 months follow-up and had no recurrences. Case 4 A 43-year-old man presented with a history of shoulder and back pain for 8 months coupled with numbness in the upper limbs for 2 months. On examination, the patient showed decreased superficial sensation of the skin in the bilateral shoulders, bilateral shoulder blades regions, and above the right upper part of the body. The muscle power of the limbs was grade 5 and muscle tone was normal. He did not have solitary palpable lymph nodes in the cervical, supraclavicular, or axillary regions. Routine laboratory examinations were also normal. Neuroimaging results showed a mass lesion of isointense on T1-weighted and T2-weighted MRI, with homogeneous enhancement at the C5-C6 level of the epidural space, compressing the spinal cord (Figure 4AeD). The patient was
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.09.097
CASE REPORT BO YUAN HUANG ET AL.
ROSAI-DORFMAN DISEASE
Figure 2. Neuroimaging and pathologic findings of case 2. (A) Sagittal T1-weighted magnetic resonance imaging (MRI) and (B) sagittal T2-weighted MRI showed that the lesion was multiple mass lesions isointense on T1-weighted and T2-weighted imaging at the C3-C5 and C6 level of the epidural space. (C) Sagittal postcontrast T1-weighted MRI and (D) coronal postcontrast T1-weighted MRI showed that the lesion was homogeneously enhanced. (E) Postoperative MRI showed that the lesion was totally excised. (F) Pathologic examination showed typical emperipolesis.
preoperatively diagnosed as having spinal meningioma. A laminectomy of C5-C6 was performed and a yellowish-white mass lesion of about 18 mm 13 mm 3 mm with intact dura was found in the subdural space. A dura-based lesion, which had slightly adhered to the surrounding tissue, causing the surrounding arachnoid to disappear, was totally excised with intact dura. Pathologic results showed typical emperipolesis, and immunologic results showed positive expression of CD68 and interspersed positive expression of lysozyme and S-100 protein with negative expression of epithelial membrane antigen (EMA) and CD1a (Figure 4F). Based on the pathologic and immunologic results, the patient was diagnosed as having RDD. Postoperatively, the patient’s preoperative symptoms were partially relieved and showed no progression over the next 12 months follow-up, with no recurrences. DISCUSSION RDD is a histioproliferative disorder that is characterized by bilateral cervical
lymphadenopathy, fever, neutrophilia, increased sedimentation rate, polyclonal hypergammaglobulinemia, and other extranodal involvements. RDD of the CNS without other extranodal involvements was observed in less than 5% of all patients with RDD.3 Furthermore, isolated spinal RDD was even rarer. A retrospective analysis showed that from 1969, when the disease was first described, to 2014, 210 cases of CNS involvement were reported, of which only 24 were isolated spinal RDD, compared with 167 cases of isolated intracranial involvements.4 Usually, intracranial RDD is an extra-axial durabased lesion (or multilesions) that mimics a dura-based meningioma.5-8 However, extremely rare cases of intraventricular and intraparenchymal lesions have also been reported.9-12 Similar to cases with intracranial involvements, most cases of isolated spinal RDD (22 cases) were also extramedullary dura-based lesions; only 2 cases were intramedullary lesions.4 In our cases, neuroimaging showed that 3 (cases 2, 3, and 4) were extramedullary lesions and the other (case 1) was an intramedullary lesion. Common symptoms of CNS
WORLD NEUROSURGERY 85: 367.e11-367.e16, JANUARY 2016
involvement are cephalgia, seizure, weakness, and paresthesia of the limbs and trunk, as well as cranial and spinal nerve deficiency based on the location of the lesions.4-8 CNS RDD is an uncommon entity and diagnostic neuroimaging usually shows a dura-based lesion. Accordingly, both intracranial and spinal RDD are commonly misdiagnosed as meningioma without biopsy. On T1-weighted MRI, intracranial or spinal RDD usually shows a single lesion or multiple well-defined mass lesions with isointense or hyperintense signal with distinct and homogeneous contrast enhancement. On T2-weighted imaging, the lesions usually present as an isointense signal.4-12 In a rare case, it was also reported that the lesion could be a high T2-signal mass and isointense on T1-weighted imaging with minimal contrast enhancement.8 In our cases, neuroimaging of 3 patients (cases 2, 3, and 4) showed extramedullary durabased lesions of isointense signal on T1-weighted and T2-weighted MRI and the other (case 1) showed an intramedullary lesion of isointense signal on T1-weighted MRI but a hyperintense signal on T2-weighted MRI. Accordingly, we assume that evidence of the lesion on T1-weighted and T2-weighted MRI may be associated with whether the lesion is extramedullary or intramedullary. The typical pathologic evidence of RDD is characterized by attenuated infiltration of lymphoplasma cells and pale histiocytes. These histiocytes are well defined, varied in size, and with round or oval vesicular nuclei, delicate nuclear membranes, and single prominent nucleoli. The emperipolesis, a characteristic pathologic sign of histiocytes engulfing well-preserved lymphocytes, can be seen in about 70% of RDD. On immunohistochemical examination, histiocytes in RDD are positively immunoreactive for CD68 and S-100 protein but negative for CD1a and EMA.3,13 The differential diagnosis of RDD includes meningioma, histiocytosis X, lymphoproliferative disorders, plasma cell granuloma, and infectious diseases. Both RDD and meningioma are dura-based lesions with homogeneous contrast enhancement, and even at microscopic levels, it is difficult to distinguish the 2 entities. During surgery, we found that the lesion had a distinct thickened and tough
www.WORLDNEUROSURGERY.org
367.E13
CASE REPORT BO YUAN HUANG ET AL.
ROSAI-DORFMAN DISEASE
Figure 3. Neuroimaging and pathologic findings of case 3. (A) Sagittal T1-weighted magnetic resonance imaging (MRI) and (B) sagittal T2-weighted MRI showed that the lesion was isointense on T1-weighted and T2-weighted MRI. (C) Sagittal postcontrast T1-weighted MRI and (D) axial postcontrast T1-weighted MRI showed that the lesion was homogeneously enhanced at the S1-S2 level of the right intervertebral foramen. (E) Postoperative MRI showed that the lesion was totally excised. (F) Pathologic examination showed typical emperipolesis.
dura. This was different from a meningioma and made us suspicious of the imaging diagnosis. The pathologic results substantiated our suspicion. Moreover, meningioma, which has positive EMA, can be readily differentiated by immunohistochemical staining. Plasma cell granuloma, characterizing a mixed inflammatory infiltrate with polyclonal plasma cells, is usually confused with RDD, because it can present as a discrete, dura-based inflammatory mass. However, emperipolesis and expression of S-100 protein is negative in the pathology of plasma cell granuloma. Langerhans cell histiocytosis (LCH) may also present as a dura-based mass. Furthermore, the S-100 protein-positive histiocytes can also be found in this lesion. However, LCH can usually be found on pathologic examination of characteristic longitudinal nuclear grooves, conspicuous eosinophils, and pathognomonic Birbeck granules, in the absence of lymphophagocytosis in histiocytes. Lymphoproliferative disorders may also be misdiagnosed as
367.E14
www.SCIENCEDIRECT.com
RDD because the histiocytes present a phagocytic phenomenon and positive S-100 protein. However, erythrophagocytosis can differentiate the lesion from RDD. As in our cases, the immunologic results of 3 patients (cases 1, 3, and 4) showed a concurrent positive expression of S-100 protein and CD68. The other patient (case 2), although her immunologic results showed negative expression of S-100 protein, was also diagnosed as having RDD based on the characteristic emperipolesis. Moreover, all 4 cases showed negative expression of CD1a in immunohistochemistry. The histiocytes in RDD show S-100 proteinpositive expression and absence of CD1a. As noted earlier, both RDD and LCH can present as positive expression of S-100 protein; however, CD1a, which is reliably expressed on histiocytes of LCH, is negative in RDD.3 Accordingly, immunologic staining of CD1a is also significant for the diagnosis of RDD. Generally, treatment of systemic RDD is advised only in patients with symptomatic
lesions or masses that threaten the function of the vital organs, because this disease is considered to be a benign, nonneoplastic lymphoproliferative disorder.4 Furthermore, complete spontaneous resolution has been reported in about 20% of cases of systemic RDD.14 However, in CNS RDD, complete spontaneous resolution has not been reported. Accordingly, surgery is still the first choice of treatment of intracranial and spinal RDD for both diagnostic purposes and rapid improving neurologic symptoms.15,16 Complete resection should be attempted, and when lesions are adhered to contiguous critical structures, subtotal resection should also be performed for pathologic diagnosis and symptomatic remission. After surgery, most patients’ disease stabilizes and a wait-and-watch treatment is advised.17 Radiotherapy, chemotherapy, and corticosteroid therapy have been given to patients whose neurologic symptoms were not relieved after surgery.18-22 Both fractionated radiotherapy and stereotactic radiotherapy have been used in CNS RDD after surgery; however, results varied and some patients showed no improvement.17,20,23 Moreover, although steroid treatment has had some therapeutic benefit on the treatment of patients with systemic RDD with rapid growing lymph node or other constitutional symptoms and some patients with CNS involvement,17,20,24 randomized trials are still needed to support its efficiency. Chemotherapy has also been adopted in systemic RDD and CNS RDD. Pulsoni et al.25 retrospectively analyzed therapeutic outcomes of 12 patients with systemic RDD who were treated with anthracyclines, vinca alkaloids, and alkylating agents. Only 2 patients achieved complete relief. Rivera et al.22 adopted a CHOP (cyclophosphamide, vincristine, doxorubicin, and prednisone) regimen to treat 2 patients with recurrent intracranial RDD. Both patients experienced recurrence within 2 years after the first subtotal resection surgery. After the second treatment of chemotherapy, they were free from recurrence for 7 years.22 Although adjuvant chemotherapy has been recommended after subtotal resection,22,26 clinical trials are still needed to establish the therapeutic approaches. In our cases, the lesions were totally or subtotally removed by surgery and no
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.09.097
CASE REPORT BO YUAN HUANG ET AL.
ROSAI-DORFMAN DISEASE
Dorfman granuloma mimicking LhermitteDuclos disease. Case report. J Neurosurg Pediatr. 2009;4:118-120. 9. Juric G, Jakic-Razumovic J, Rotim K, Zarkovic K. Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma. Acta Neurochir (Wien). 2003;145:145-149. 10. Gaetani P, Tancioni F, Di Rocco M, Rodriguez y Baena R. Isolated cerebellar involvement in RosaiDorfman disease: case report. Neurosurgery. 2000; 46:479-481. 11. Natarajan S, Post KD, Strauchen J, Morgello S. Primary intracerebral Rosai-Dorfman disease: a case report. J Neurooncol. 2000;47:73-77. 12. Morandi X, Godey B, Riffaud L, Heresbach N, Brassier G. Isolated Rosai-Dorfman disease of the fourth ventricle. Case illustration. J Neurosurg. 2000;92:890. 13. Tubbs RS, Kelly DR, Mroczek-Musulman EC, Hammers YA, Berkow RL, Oakes WJ, et al. Spinal cord compression as a result of Rosai-Dorfman disease of the upper cervical spine in a child. Childs Nerv Syst. 2005;21:951-954. 14. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7:19-73.
Figure 4. Neuroimaging and pathologic findings of case 4. (A) Sagittal T1-weighted magnetic resonance imaging (MRI) and (B) sagittal T2-weighted MRI showed that the lesion was isointense on T1-weighted and T2-weighted imaging at the C5-C6 level of the epidural space. (C) Sagittal postcontrast T1-weighted MRI and (D) coronal postcontrast T1-weighted MRI showed that the lesion was homogeneously enhanced. (E) Postoperative MRI showed that the lesion was totally excised. (F) Pathologic examination showed typical emperipolesis.
recurrences were observed after 6e18 months follow-up.
CONCLUSIONS Isolated spinal RDD is an exceedingly rare disease and its diagnosis is still challenging. Histologic evidence and immunophenotypical characteristics are still the only reliable basis for diagnosis. Surgical resection has been shown to be an effective treatment approach, and other treatments such as radiotherapy, steroid therapy, and chemotherapy need further research to clarify usage and efficiency. REFERENCES 1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol. 1969;87:63-70. 2. Symss NP, Cugati G, Vasudevan MC, Ramamurthi R, Pande A. Intracranial Rosai
15. Kayali H, Onguru O, Erdogan E, Sirin S, Timurkaynak E. Isolated intracranial RosaiDorfman disease mimicking meningioma. Clin Neuropathol. 2004;23:204-208.
Dorfman disease: report of three cases and literature review. Asian J Neurosurg. 2010;5:19-30. 3. Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV. Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol. 2001;14:172-178. 4. Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, Velazquez-Vega J, Borja MJ, Rosenberg S, et al. Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. Medicine (Baltimore). 2014;93: 165-175. 5. Kim M, Provias J, Bernstein M. Rosai-Dorfman disease mimicking multiple meningioma: case report. Neurosurgery. 1995;36:1185-1187. 6. Simos M, Dimitrios P, Philip T. A new clinical entity mimicking meningioma diagnosed pathologically as Rosai-Dorfman disease. Skull Base Surg. 1998;8:87-92. 7. Di Rocco F, Garnett MR, Puget S, Pueyerredon F, Roujeau T, Jaubert F, et al. Cerebral localization of Rosai-Dorfman disease in a child. Case report. J Neurosurg. 2007;107(2 Suppl):147-151. 8. Johnston JM, Limbrick DD, Ray WZ, Brown S, Shimony J, Park TS. Isolated cerebellar Rosai-
WORLD NEUROSURGERY 85: 367.e11-367.e16, JANUARY 2016
16. Griffiths SJ, Tang W, Parameswaran R, Kelsey A, West CG. Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. Br J Neurosurg. 2004;18:293-297. 17. Cooper SL, Jenrette JM. Rosai-Dorfman disease: management of CNS and systemic involvement. Clin Adv Hematol Oncol. 2012;10:199-202. 18. Franco-Paredes C, Martin K. Extranodal RosaiDorfman disease involving the meninges. South Med J. 2002;95:1101-1102. 19. Sato A, Sakurada K, Sonoda Y, Saito S, Kayama T, Jokura H, et al. Rosai-Dorfman disease presenting with multiple intracranial and intraspinal masses: a case report. No Shinkei Geka. 2003;31:1199-1204. 20. Hadjipanayis CG, Bejjani G, Wiley C, Hasegawa T, Maddock M, Kondziolka D. Intracranial Rosai-Dorfman disease treated with microsurgical resection and stereotactic radiosurgery. Case report. J Neurosurg. 2003;98:165-168. 21. Hinduja A, Aguilar LG, Steineke T, Nochlin D, Landolfi JC. Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion. J Neurooncol. 2009;92:117-120. 22. Rivera D, Pérez-Castillo M, Fernández B, Stoeter P. Long-term follow-up in two cases of intracranial Rosai-Dorfman disease complicated by incomplete resection and recurrence. Surg Neurol Int. 2014;5:30.
www.WORLDNEUROSURGERY.org
367.E15
CASE REPORT BO YUAN HUANG ET AL.
23. Kidd DP, Revesz T, Miller NR. Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement. Neurology. 2006;67: 1551-1555. 24. Shaver EG, Rebsamen SL, Yachnis AT, Sutton LN. Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy. Case report. J Neurosurg. 1993; 79:769-773. 25. Pulsoni A, Anghel G, Falcucci P, Matera R, Pescarmona E, Ribersani M, et al. Treatment of sinus histiocytosis with massive lymphadenopathy
367.E16
www.SCIENCEDIRECT.com
ROSAI-DORFMAN DISEASE
(Rosai-Dorfman disease): report of a case and literature review. Am J Hematol. 2002;69:67-71. 26. Le Guenno G, Galicier L, Uro-Coste E, Petitcolin V, Rieu V, Ruivard M. Successful treatment with azathioprine of relapsing RosaiDorfman disease of the central nervous system. J Neurosurg. 2012;117:486-489.
commercial or financial relationships that could be construed as a potential conflict of interest. Received 26 August 2015; accepted 26 September 2015 Citation: World Neurosurg. (2016) 85:367.e11-367.e16. http://dx.doi.org/10.1016/j.wneu.2015.09.097 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2016 Elsevier Inc. All rights reserved.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.09.097