Hospital Practice
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
Case Report of Intracranial Rosai-Dorfman Disease Mervat Wahba MD To cite this article: Mervat Wahba MD (2013) Case Report of Intracranial Rosai-Dorfman Disease, Hospital Practice, 41:4, 83-86 To link to this article: http://dx.doi.org/10.3810/hp.2013.10.1085
Published online: 13 Mar 2015.
Submit your article to this journal
Article views: 2
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ihop20 Download by: [Central Michigan University]
Date: 06 November 2015, At: 03:19
C a s e R e p o rt
Case Report of Intracranial Rosai-Dorfman Disease
Mervat Wahba, MD 1 Associate Professor of Neurology, Neurology Department, University of Tennessee Health Sciences Center, Memphis, TN
DOI: 10.3810/hp.2013.10.1085
Downloaded by [Central Michigan University] at 03:19 06 November 2015
1
Abstract: Rosai-Dorfman disease (RDD)—sinus histiocytosis with massive lymphadenopathy―represents a peculiar proliferation of histiocyte-like cells in patients. The condition was described by Rosai and Dorfman in 1969, after examining 4 cases, as an idiopathic histiocytic disorder. In 1972, they studied an additional 30 cases of patients with RDD. A histioproliferative disorder, RDD is characterized by bilateral, painless, cervical lymphadenopathy in 81% of patients. Fever, leukocytosis, elevated sedimentation rate, and polyclonal hypergammaglobulinemia may also be found. In 30% of patients, extranodal involvement is present and may include the skin, eye orbit, upper respiratory tract, or testes. Cases involving the central nervous system are rare and account for , 5% of patients with RDD. We report on a 78-year-old woman presenting with new-onset headache, dizziness, and imbalance, which had been present for a few weeks prior to admission. Magnetic resonance imaging of the brain showed 2 enhancing lesions within the right and left cerebellar hemispheres. Biopsy of the mass demonstrated a lymphohistiocytic infiltrate involving the cerebellum with foci of emperipolesis (phagocytosed lymphocytes). The adjacent cerebellum showed myelinated nerve fibers with reactive gliosis. A thorough work-up and histopathologic exam of the biopsied mass demonstrated lymphohistiocytic infiltrate with foci of emperipolesis (phagocytosed lymphocytes) consistent with RDD. Other differential considerations, such as primary or secondary neoplasms, infections, lymphoproliferative disorders, granulomatoses, Langerhans cell histiocytosis, and lymphocyterich meningioma were ruled out by additional histopathologic exam. Keywords: cervical lymphadenopathy; leukocytosis; phagocytosed lymphocytes; polyclonal hypergammaglobulinemia
Case Report
Correspondence: Mervat Wahba, MD, Associate Professor of Neurology, University of Tennessee Health Sciences Center, 855 Monroe Ave., Link Building, Room 415, Memphis, TN 38163. Tel: 901-448-6199 Fax: 901-448-7440 E-mail: [email protected]
A 78-year-old white woman, with no history of primary malignancy and prior medical history positive only for gastroesophageal reflux disease and hyperlipidemia, presented reporting a 2-week experience of subacute dizziness, headache, stumbling, and tendency to fall backwards. Upon presentation, neurologic examination revealed normal mental status, normal cranial nerve response, and normal motor strength. Physical examination of the patient was unremarkable; no lymphadenopathy was noted. On cerebellar testing, right finger-to-nose testing revealed dysmetria. Patient gait was wide based. Magnetic resonance imaging (MRI) of the head, with and without contrast, revealed 2 enhancing lesions: a 9-mm lesion in the right cerebellar hemisphere causing cerebellar ectopia and with surrounding vasogenic edema; a smaller left cerebellar enhancing lesion was seen as well. Additionally, dural enhancement was observed (Figure 1). Differential diagnosis based on MRI findings included primary and secondary tumors of the central nervous system (CNS), infectious etiologies, lymphoproliferative
© Hospital Practice,Volume 41, Issue 4, October/November 2013, ISSN – 2154-8331 83 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected]
11_Wahba.indd 83
10/11/13 3:14 PM
Mervat Wahba
Downloaded by [Central Michigan University] at 03:19 06 November 2015
Figure 1. Axial T1-weighted image with contrast showing an enhancing mass in the right cerebellar hemisphere and a smaller mass in the left cerebellar hemisphere.
disorders, plasma cell granulomas, Langerhans cell histiocytosis and lymphocyte-rich meningioma. Antineuronal nuclear antibodies (Hu antibodies), antiPurkinje cell (Yo antibodies), and anti-Ri antibodies were undetectable by serum immunofluorescent assay. Carcinoembryonic antigen level was 0.7 ng/mL (normal range, 0.0 to , 2.6); cancer antigen 19-9 level was 5.4 U/mL (normal range, 0.0 to 34.9). A drug screen was negative. Tuberculin skin test result was negative. A chest radiograph was normal. Cerebellar biopsy fungus culture and acid-fast bacillus stain and culture revealed no presence or growth of mycobacteria or fungi in 8 weeks. A metastatic work-up in the form of computerized tomography (CT) imaging of the chest, abdomen, and pelvis revealed no sources of malignancy. A mammogram was negative for breast masses. A radionuclide bone scan was negative for masses. The patient was started on oral dexamethasone. The neurosurgery team was consulted and it was decided to proceed with a debulking surgery with a biopsy of the lesions. The patient underwent suboccipital craniectomy for excision and biopsy of the cerebellar masses followed by placement of a dural patch graft. Surgical pathology of the mass demonstrated a lymphohistiocytic infiltrate with foci of emperipolesis (phagocytosed lymphocytes). Immunoperoxidase stains for S-100 and cluster of differentiation (CD) 68 demonstrated a population of histiocytic cells. Immunoperoxidase stains for CD3 and CD20 demonstrated a population of lymphoid cells with predominance of T lymphocytes. Stains for CD15 and CD30 were negative, thus indicating the absence of Hodgkin disease and anaplastic large-cell lymphoma. An additional CD1a 84
11_Wahba.indd 84
stain was completed to rule out Langerhans histiocytosis, evidenced by no immune reactivity to CD1a. An epithelial membrane antigen (EMA) stain was negative, thus ruling out a lymphocyte-rich meningioma. Material submitted to flow cytometry predominantly demonstrated T cells expressing normal T-cell antigens CD2, CD3, CD5, and CD7, with a CD4:CD8 ratio of 50:47. Natural killer (NK) cells were not increased. Beta cells (β) were a minority, expressing CD19 and 20, showing no light-chain restriction. The flow cytometry findings constituted polyclonal T and B cells and were nonspecific. No malignancy was identified. Special stains for acid-fast bacilli and Grocott’s methenamine silver stain for fungi were negative. Patient’s findings were consistent with RDD. Hematology and oncology services were consulted; it was recommended that the patient continue taking dexamethasone and that patient blood levels be monitored for carcinoembryonic antigen, cancer antigen 19-9 levels, and antineuronal nuclear antibodies (Hu, Yo, and anti-Ri antibodies). During her postoperative stay, the patient reported that her headache resolved. Her gait imbalance also improved significantly. A follow-up MRI of the head with contrast showed a very minimal enhancement surrounding the operative cavity. The patient was discharged home in stable condition and was to follow up with neurosurgery, hematology/oncology, and with her primary care physician. Follow up brain MRI with contrast was scheduled.
Discussion
In 1969, Rosai and Dorfman were the first to describe 4 patient cases of sinus histiocytosis with massive lymphadenopathy.1,2 Classic patient presentation of RDD includes massive, painless cervical lymphadenopathy, fever, and polyclonal hypergammaglobulinemia.3,4 Clinical presentation may include nasal obstruction, occasional episodes of epistaxis, tonsillar enlargement, or hearing abnormalities. Extranodal involvement has been reported in other anatomic sites, notably the eye orbit, upper respiratory tract, and the soft tissue.4,5 Involvement of the CNS occurs in , 5% of patients.6 Central nervous system involvement typically occurs in the epidural or subdural space of the skull base or the spine.7–12 It has been suggested that RDD represents an autoimmune disease or a reactive proliferation to an infection without a true neoplastic process, although the evidence is not conclusive. Biopsy of the brain mass in our patient demonstrated a lymphohistiocytic infiltrate with foci of emperipolesis (phagocytosed lymphocytes). Immunoperoxidase stains for
© Hospital Practice, Volume 41, Issue 4, October/November 2013, ISSN – 2154-8331 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected]
10/11/13 3:14 PM
Case Report: Intracranial Rosai-Dorfman Disease
Downloaded by [Central Michigan University] at 03:19 06 November 2015
Figure 2. Collection of histiocytes (h arrow) with abundant pink cytoplasm, in a lymphoplasmacytic background (i arrow).
S-100 and CD68 demonstrated a population of histiocytic cells. Immunophenotypical testing for CD68 was positive in the macrophages. No malignancy was identified. Special stains for acid-fast bacilli and fungi were negative. The findings were consistent with RDD (Figure 2, 3). The differential diagnosis of a lesion containing numerous, large histiocytes includes granulomatous diseases, such as Wegener granulomatosis, sarcoidosis, Hodgkin disease, Langerhans histiocytosis, and lymphocyte-rich meningiomas. The lack of sheet-like necrobiotic necrosis and necrotizing capillaritis excluded Wegener granulomatosis; the absence of small, well-formed granulomas made sarcoidosis unlikely. The morphologic features of the histiocytes in Langerhans histiocytosis include xanthomatous cytoplasm and longitudinal nuclear grooves, in distinction to the pale histiocytes characteristic of RDD. Patients with Hodgkin disease may show a mixed inflammatory infiltrate and fibrosis but lesions lack the histiocytes of RDD. Reed-Sternberg cells are S-100 protein negative, and positive for CD15 and CD30―in contrast to Rosai-Dorfman histiocytes, which are S-100 protein positive, and negative for CD15 and CD30.13 Lymphoproliferative disorders may show phagocytic properties and can be S-100 protein positive. Lymphoproliferative disorders, however, show erythrophagocytosis rather than lymphophagocytosis. Also, lymphoproliferative disorders will manifest with malignant cytologic features.7 Plasma cell granulomas and necrotic abscesses exhibit neither emperipolesis nor S-100 protein positivity.8 Distinction of RDD from a lymphoplasmacytic-rich meningioma is also possible by performing an EMA stain.9 An immunoperoxidase stain for S-100 in our patient’s biopsy specimen was strongly positive within the histiocyte population.
Rosai-Dorfman disease is an uncommon disorder that typically manifests with systemic symptoms and lymphadenopathy. Extranodal intracranial disease is uncommon. A review by Andriko et al,6 described 11 cases of RDD isolated to the CNS without nodal disease. The disease may mimic meningiomas,7,11 lymphomas, and chronic inflammation.9,10 Magnetic resonance images of patients with RDD show low signal intensity on T2-weighted MRI, unlike most meningiomas.11 Rosai-Dorfman disease has been reported as a cause of myelopathy.14 Leptomeningeal RRD has been described in case reports. We have previously reported a case of leptomeningeal RDD that improved with surgical resection.15 Intracranial RDD is typically treated with surgical resection. In cases of multi-organ, progressive disorder, immunosuppressive treatments, including cytoxan, corticosteroids, vincristine, or radiation may be administered to the patient. Patients should be closely monitored for recurrence of intracranial RDD and should be offered repeat resections or radiosurgery accompanied by or preceded with other treatment modalities, such as systemic corticosteroids, radiation therapy, anti-metabolites, or immunomodulatory therapies.16–21
Conclusion
The histopathological picture of our case suggests that RDD may manifest as an intracranial mass. No infectious, neoplastic, lymphoproliferative, eosinophilic granuloma, or otherwise granulomatous etiology was found in our patient. Rosai-Dorfman disease should be considered in the differential diagnosis of patients with intracranial masses, duralbased masses, and diffuse leptomeningeal enhancement. Given the rarity of occurrence of RDD, histopathological Figure 3. Immunohistochemical staining for CD68 confirms that large cells with abundant pink cytoplasm are indeed histiocytes.
© Hospital Practice, Volume 41, Issue 4, October/November 2013, ISSN – 2154-8331 85 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected]
11_Wahba.indd 85
10/11/13 3:14 PM
Mervat Wahba
and immunocytochemical assessments of the patient should permit a correct diagnosis.
Acknowledgments
I acknowledge, with gratitude, the efforts of Lauar Salama, MD, and Caitlin Carr, for their contributions in reviewing the manuscript.
Conflict of Interest Statement
Mervat Wahba, MD, declares no conflicts of interest.
18. Horneff G, Jurgens H, Hort W, Karitzky D, Göbel U. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine. Med Pediatr Oncol. 1996; 27(3):187–192. 19. Palomera L, Domingo M, Soria J, Gutiérrez M. Long term survival in a patient with aggressive Rosai-Dorfman disease treated with interferon alpha. Med Clin (Barc). 2001;116(20):797–798. 20. Tasso M, Esquembre C, Blanco E, Moscardó, Niveiro M, Payá A. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine. Pediatr Blood Cancer. 2006;47(5):612–615. 21. Utikal J, Ugurel S, Kurzen H, et al. Imatinib as a treatment option for systemic non-Langerhans cell histiocytoses. Arch Dermatol. 2007;143(6):736–740.
Downloaded by [Central Michigan University] at 03:19 06 November 2015
References
1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63–70. 2. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7(1):19–73. 3. Haas RJ, Helmig MSE, Pretchel K. Sinus histiocytosis with massive lymphadenopathy and paraparesis: remission with chemotherapy. Cancer. 1978;42(1):77–80. 4. Sanchez R, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: an analysis of 113 cases with special emphasis on its extranodal manifestation. Lab Invest. 1977;36:21–22. 5. Puppin D Jr, Chavaz P, Harms M. Histiocytic lymphophagocytic panniculitis (Rosai-Dorfman disease): a case report. Dermatology. 1992;184(4):317–320 6. Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV. RosaiDorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol. 2001;14(3):172–178. 7. Siadati A, Powell SZ, Shahab I, Valadka AB, Parker JR. Pathologic quiz case: a 48 year-old woman with a dural–based intracranial tumor. Arch Pathol Lab Med. 2001;125(8):1115–1116. 8. Wu M, Anderson AE, Kahn LB. A report of intracranial Rosai-Dorfman disease with literature review. Ann Diagn Pathol. 2001;5(2):96–102. 9. Trudel M. Dural involvement in sinus histiocytosis with massive lymphadenopathy. Case report. J Neurosurg. 1984;60(4):850–852. 10. Cannella DM, Prezyna AP, Kapp JP. Primary intracranial plasma-cell granuloma. Case report. J Neurosurg. 1988;69(5):785–788. 11. Kattner KA, Stroink AR, Roth TC, Lee JM. Rosai-Dorfman disease mimicking parasagittal meningioma: case presentation and review of literature. Surg Neurol. 2000;53(5):452–457; discussion 457. 12. Udono H, Fukuyama K, Okamoto H, Tabuchi K. Rosai-Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report. J Neurosurg. 1999;91(2):335–339. 13. Rosai J, ed. Differential diagnosis in surgical pathology. Rosai and Ackerman’s Surgical Pathology, 10th ed. Philadelphia, PA: Elsevier Mosby; 2011. 14. Hollowell JP, Wolfla CE, Shah NC, Mark LP, Whittaker MH. RosaiDorfman disease causing cervical myelopathy. Spine (Phila Pa 1976). 2000;25(11):1453–1456. 15. Wahba M, Mansoor S, Foreman A, Shokouh-Amiri M, Bauer D. RosaiDorfman disease involving the meninges. Int J Heart & Brain. 2011;4(1 & 2):49–51. 16. Oka M, Kamo T, Goto N, et al. Successful treatment of Rosai-Dorfman disease with low-dose oral corticosteroid. J Dermatol. 2009; 36(4):237–240. 17. Hadjipanyayis CG, Bejjani G, Wiley C, Hasegawa T, Maddock M, Kondziolka D. Intracranial Rosai-Dorfman disease treated with microsurgical resection and stereotactic surgery. J Neurosurg. 2003;98(1):165–168.
86
11_Wahba.indd 86
© Hospital Practice, Volume 41, Issue 4, October/November 2013, ISSN – 2154-8331 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected]
10/11/13 3:14 PM
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
Case Report of Intracranial Rosai-Dorfman Disease Mervat Wahba MD To cite this article: Mervat Wahba MD (2013) Case Report of Intracranial Rosai-Dorfman Disease, Hospital Practice, 41:4, 83-86 To link to this article: http://dx.doi.org/10.3810/hp.2013.10.1085
Published online: 13 Mar 2015.
Submit your article to this journal
Article views: 2
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ihop20 Download by: [Central Michigan University]
Date: 06 November 2015, At: 03:19
C a s e R e p o rt
Case Report of Intracranial Rosai-Dorfman Disease
Mervat Wahba, MD 1 Associate Professor of Neurology, Neurology Department, University of Tennessee Health Sciences Center, Memphis, TN
DOI: 10.3810/hp.2013.10.1085
Downloaded by [Central Michigan University] at 03:19 06 November 2015
1
Abstract: Rosai-Dorfman disease (RDD)—sinus histiocytosis with massive lymphadenopathy―represents a peculiar proliferation of histiocyte-like cells in patients. The condition was described by Rosai and Dorfman in 1969, after examining 4 cases, as an idiopathic histiocytic disorder. In 1972, they studied an additional 30 cases of patients with RDD. A histioproliferative disorder, RDD is characterized by bilateral, painless, cervical lymphadenopathy in 81% of patients. Fever, leukocytosis, elevated sedimentation rate, and polyclonal hypergammaglobulinemia may also be found. In 30% of patients, extranodal involvement is present and may include the skin, eye orbit, upper respiratory tract, or testes. Cases involving the central nervous system are rare and account for , 5% of patients with RDD. We report on a 78-year-old woman presenting with new-onset headache, dizziness, and imbalance, which had been present for a few weeks prior to admission. Magnetic resonance imaging of the brain showed 2 enhancing lesions within the right and left cerebellar hemispheres. Biopsy of the mass demonstrated a lymphohistiocytic infiltrate involving the cerebellum with foci of emperipolesis (phagocytosed lymphocytes). The adjacent cerebellum showed myelinated nerve fibers with reactive gliosis. A thorough work-up and histopathologic exam of the biopsied mass demonstrated lymphohistiocytic infiltrate with foci of emperipolesis (phagocytosed lymphocytes) consistent with RDD. Other differential considerations, such as primary or secondary neoplasms, infections, lymphoproliferative disorders, granulomatoses, Langerhans cell histiocytosis, and lymphocyterich meningioma were ruled out by additional histopathologic exam. Keywords: cervical lymphadenopathy; leukocytosis; phagocytosed lymphocytes; polyclonal hypergammaglobulinemia
Case Report
Correspondence: Mervat Wahba, MD, Associate Professor of Neurology, University of Tennessee Health Sciences Center, 855 Monroe Ave., Link Building, Room 415, Memphis, TN 38163. Tel: 901-448-6199 Fax: 901-448-7440 E-mail: [email protected]
A 78-year-old white woman, with no history of primary malignancy and prior medical history positive only for gastroesophageal reflux disease and hyperlipidemia, presented reporting a 2-week experience of subacute dizziness, headache, stumbling, and tendency to fall backwards. Upon presentation, neurologic examination revealed normal mental status, normal cranial nerve response, and normal motor strength. Physical examination of the patient was unremarkable; no lymphadenopathy was noted. On cerebellar testing, right finger-to-nose testing revealed dysmetria. Patient gait was wide based. Magnetic resonance imaging (MRI) of the head, with and without contrast, revealed 2 enhancing lesions: a 9-mm lesion in the right cerebellar hemisphere causing cerebellar ectopia and with surrounding vasogenic edema; a smaller left cerebellar enhancing lesion was seen as well. Additionally, dural enhancement was observed (Figure 1). Differential diagnosis based on MRI findings included primary and secondary tumors of the central nervous system (CNS), infectious etiologies, lymphoproliferative
© Hospital Practice,Volume 41, Issue 4, October/November 2013, ISSN – 2154-8331 83 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected]
11_Wahba.indd 83
10/11/13 3:14 PM
Mervat Wahba
Downloaded by [Central Michigan University] at 03:19 06 November 2015
Figure 1. Axial T1-weighted image with contrast showing an enhancing mass in the right cerebellar hemisphere and a smaller mass in the left cerebellar hemisphere.
disorders, plasma cell granulomas, Langerhans cell histiocytosis and lymphocyte-rich meningioma. Antineuronal nuclear antibodies (Hu antibodies), antiPurkinje cell (Yo antibodies), and anti-Ri antibodies were undetectable by serum immunofluorescent assay. Carcinoembryonic antigen level was 0.7 ng/mL (normal range, 0.0 to , 2.6); cancer antigen 19-9 level was 5.4 U/mL (normal range, 0.0 to 34.9). A drug screen was negative. Tuberculin skin test result was negative. A chest radiograph was normal. Cerebellar biopsy fungus culture and acid-fast bacillus stain and culture revealed no presence or growth of mycobacteria or fungi in 8 weeks. A metastatic work-up in the form of computerized tomography (CT) imaging of the chest, abdomen, and pelvis revealed no sources of malignancy. A mammogram was negative for breast masses. A radionuclide bone scan was negative for masses. The patient was started on oral dexamethasone. The neurosurgery team was consulted and it was decided to proceed with a debulking surgery with a biopsy of the lesions. The patient underwent suboccipital craniectomy for excision and biopsy of the cerebellar masses followed by placement of a dural patch graft. Surgical pathology of the mass demonstrated a lymphohistiocytic infiltrate with foci of emperipolesis (phagocytosed lymphocytes). Immunoperoxidase stains for S-100 and cluster of differentiation (CD) 68 demonstrated a population of histiocytic cells. Immunoperoxidase stains for CD3 and CD20 demonstrated a population of lymphoid cells with predominance of T lymphocytes. Stains for CD15 and CD30 were negative, thus indicating the absence of Hodgkin disease and anaplastic large-cell lymphoma. An additional CD1a 84
11_Wahba.indd 84
stain was completed to rule out Langerhans histiocytosis, evidenced by no immune reactivity to CD1a. An epithelial membrane antigen (EMA) stain was negative, thus ruling out a lymphocyte-rich meningioma. Material submitted to flow cytometry predominantly demonstrated T cells expressing normal T-cell antigens CD2, CD3, CD5, and CD7, with a CD4:CD8 ratio of 50:47. Natural killer (NK) cells were not increased. Beta cells (β) were a minority, expressing CD19 and 20, showing no light-chain restriction. The flow cytometry findings constituted polyclonal T and B cells and were nonspecific. No malignancy was identified. Special stains for acid-fast bacilli and Grocott’s methenamine silver stain for fungi were negative. Patient’s findings were consistent with RDD. Hematology and oncology services were consulted; it was recommended that the patient continue taking dexamethasone and that patient blood levels be monitored for carcinoembryonic antigen, cancer antigen 19-9 levels, and antineuronal nuclear antibodies (Hu, Yo, and anti-Ri antibodies). During her postoperative stay, the patient reported that her headache resolved. Her gait imbalance also improved significantly. A follow-up MRI of the head with contrast showed a very minimal enhancement surrounding the operative cavity. The patient was discharged home in stable condition and was to follow up with neurosurgery, hematology/oncology, and with her primary care physician. Follow up brain MRI with contrast was scheduled.
Discussion
In 1969, Rosai and Dorfman were the first to describe 4 patient cases of sinus histiocytosis with massive lymphadenopathy.1,2 Classic patient presentation of RDD includes massive, painless cervical lymphadenopathy, fever, and polyclonal hypergammaglobulinemia.3,4 Clinical presentation may include nasal obstruction, occasional episodes of epistaxis, tonsillar enlargement, or hearing abnormalities. Extranodal involvement has been reported in other anatomic sites, notably the eye orbit, upper respiratory tract, and the soft tissue.4,5 Involvement of the CNS occurs in , 5% of patients.6 Central nervous system involvement typically occurs in the epidural or subdural space of the skull base or the spine.7–12 It has been suggested that RDD represents an autoimmune disease or a reactive proliferation to an infection without a true neoplastic process, although the evidence is not conclusive. Biopsy of the brain mass in our patient demonstrated a lymphohistiocytic infiltrate with foci of emperipolesis (phagocytosed lymphocytes). Immunoperoxidase stains for
© Hospital Practice, Volume 41, Issue 4, October/November 2013, ISSN – 2154-8331 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected]
10/11/13 3:14 PM
Case Report: Intracranial Rosai-Dorfman Disease
Downloaded by [Central Michigan University] at 03:19 06 November 2015
Figure 2. Collection of histiocytes (h arrow) with abundant pink cytoplasm, in a lymphoplasmacytic background (i arrow).
S-100 and CD68 demonstrated a population of histiocytic cells. Immunophenotypical testing for CD68 was positive in the macrophages. No malignancy was identified. Special stains for acid-fast bacilli and fungi were negative. The findings were consistent with RDD (Figure 2, 3). The differential diagnosis of a lesion containing numerous, large histiocytes includes granulomatous diseases, such as Wegener granulomatosis, sarcoidosis, Hodgkin disease, Langerhans histiocytosis, and lymphocyte-rich meningiomas. The lack of sheet-like necrobiotic necrosis and necrotizing capillaritis excluded Wegener granulomatosis; the absence of small, well-formed granulomas made sarcoidosis unlikely. The morphologic features of the histiocytes in Langerhans histiocytosis include xanthomatous cytoplasm and longitudinal nuclear grooves, in distinction to the pale histiocytes characteristic of RDD. Patients with Hodgkin disease may show a mixed inflammatory infiltrate and fibrosis but lesions lack the histiocytes of RDD. Reed-Sternberg cells are S-100 protein negative, and positive for CD15 and CD30―in contrast to Rosai-Dorfman histiocytes, which are S-100 protein positive, and negative for CD15 and CD30.13 Lymphoproliferative disorders may show phagocytic properties and can be S-100 protein positive. Lymphoproliferative disorders, however, show erythrophagocytosis rather than lymphophagocytosis. Also, lymphoproliferative disorders will manifest with malignant cytologic features.7 Plasma cell granulomas and necrotic abscesses exhibit neither emperipolesis nor S-100 protein positivity.8 Distinction of RDD from a lymphoplasmacytic-rich meningioma is also possible by performing an EMA stain.9 An immunoperoxidase stain for S-100 in our patient’s biopsy specimen was strongly positive within the histiocyte population.
Rosai-Dorfman disease is an uncommon disorder that typically manifests with systemic symptoms and lymphadenopathy. Extranodal intracranial disease is uncommon. A review by Andriko et al,6 described 11 cases of RDD isolated to the CNS without nodal disease. The disease may mimic meningiomas,7,11 lymphomas, and chronic inflammation.9,10 Magnetic resonance images of patients with RDD show low signal intensity on T2-weighted MRI, unlike most meningiomas.11 Rosai-Dorfman disease has been reported as a cause of myelopathy.14 Leptomeningeal RRD has been described in case reports. We have previously reported a case of leptomeningeal RDD that improved with surgical resection.15 Intracranial RDD is typically treated with surgical resection. In cases of multi-organ, progressive disorder, immunosuppressive treatments, including cytoxan, corticosteroids, vincristine, or radiation may be administered to the patient. Patients should be closely monitored for recurrence of intracranial RDD and should be offered repeat resections or radiosurgery accompanied by or preceded with other treatment modalities, such as systemic corticosteroids, radiation therapy, anti-metabolites, or immunomodulatory therapies.16–21
Conclusion
The histopathological picture of our case suggests that RDD may manifest as an intracranial mass. No infectious, neoplastic, lymphoproliferative, eosinophilic granuloma, or otherwise granulomatous etiology was found in our patient. Rosai-Dorfman disease should be considered in the differential diagnosis of patients with intracranial masses, duralbased masses, and diffuse leptomeningeal enhancement. Given the rarity of occurrence of RDD, histopathological Figure 3. Immunohistochemical staining for CD68 confirms that large cells with abundant pink cytoplasm are indeed histiocytes.
© Hospital Practice, Volume 41, Issue 4, October/November 2013, ISSN – 2154-8331 85 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected]
11_Wahba.indd 85
10/11/13 3:14 PM
Mervat Wahba
and immunocytochemical assessments of the patient should permit a correct diagnosis.
Acknowledgments
I acknowledge, with gratitude, the efforts of Lauar Salama, MD, and Caitlin Carr, for their contributions in reviewing the manuscript.
Conflict of Interest Statement
Mervat Wahba, MD, declares no conflicts of interest.
18. Horneff G, Jurgens H, Hort W, Karitzky D, Göbel U. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine. Med Pediatr Oncol. 1996; 27(3):187–192. 19. Palomera L, Domingo M, Soria J, Gutiérrez M. Long term survival in a patient with aggressive Rosai-Dorfman disease treated with interferon alpha. Med Clin (Barc). 2001;116(20):797–798. 20. Tasso M, Esquembre C, Blanco E, Moscardó, Niveiro M, Payá A. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine. Pediatr Blood Cancer. 2006;47(5):612–615. 21. Utikal J, Ugurel S, Kurzen H, et al. Imatinib as a treatment option for systemic non-Langerhans cell histiocytoses. Arch Dermatol. 2007;143(6):736–740.
Downloaded by [Central Michigan University] at 03:19 06 November 2015
References
1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63–70. 2. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7(1):19–73. 3. Haas RJ, Helmig MSE, Pretchel K. Sinus histiocytosis with massive lymphadenopathy and paraparesis: remission with chemotherapy. Cancer. 1978;42(1):77–80. 4. Sanchez R, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: an analysis of 113 cases with special emphasis on its extranodal manifestation. Lab Invest. 1977;36:21–22. 5. Puppin D Jr, Chavaz P, Harms M. Histiocytic lymphophagocytic panniculitis (Rosai-Dorfman disease): a case report. Dermatology. 1992;184(4):317–320 6. Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV. RosaiDorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol. 2001;14(3):172–178. 7. Siadati A, Powell SZ, Shahab I, Valadka AB, Parker JR. Pathologic quiz case: a 48 year-old woman with a dural–based intracranial tumor. Arch Pathol Lab Med. 2001;125(8):1115–1116. 8. Wu M, Anderson AE, Kahn LB. A report of intracranial Rosai-Dorfman disease with literature review. Ann Diagn Pathol. 2001;5(2):96–102. 9. Trudel M. Dural involvement in sinus histiocytosis with massive lymphadenopathy. Case report. J Neurosurg. 1984;60(4):850–852. 10. Cannella DM, Prezyna AP, Kapp JP. Primary intracranial plasma-cell granuloma. Case report. J Neurosurg. 1988;69(5):785–788. 11. Kattner KA, Stroink AR, Roth TC, Lee JM. Rosai-Dorfman disease mimicking parasagittal meningioma: case presentation and review of literature. Surg Neurol. 2000;53(5):452–457; discussion 457. 12. Udono H, Fukuyama K, Okamoto H, Tabuchi K. Rosai-Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report. J Neurosurg. 1999;91(2):335–339. 13. Rosai J, ed. Differential diagnosis in surgical pathology. Rosai and Ackerman’s Surgical Pathology, 10th ed. Philadelphia, PA: Elsevier Mosby; 2011. 14. Hollowell JP, Wolfla CE, Shah NC, Mark LP, Whittaker MH. RosaiDorfman disease causing cervical myelopathy. Spine (Phila Pa 1976). 2000;25(11):1453–1456. 15. Wahba M, Mansoor S, Foreman A, Shokouh-Amiri M, Bauer D. RosaiDorfman disease involving the meninges. Int J Heart & Brain. 2011;4(1 & 2):49–51. 16. Oka M, Kamo T, Goto N, et al. Successful treatment of Rosai-Dorfman disease with low-dose oral corticosteroid. J Dermatol. 2009; 36(4):237–240. 17. Hadjipanyayis CG, Bejjani G, Wiley C, Hasegawa T, Maddock M, Kondziolka D. Intracranial Rosai-Dorfman disease treated with microsurgical resection and stereotactic surgery. J Neurosurg. 2003;98(1):165–168.
86
11_Wahba.indd 86
© Hospital Practice, Volume 41, Issue 4, October/November 2013, ISSN – 2154-8331 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected]
10/11/13 3:14 PM
