Systemic and Craniospinal Rosai Dorfman Disease with Intraparenchymal, Intramedullary and Leptomeningeal Disease
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Abstract
Rosai Dorfman disease is a rare histiocytic disorder of over-production of non-Langerhans histiocytes, which typically manifests with massive lymphadenopathy and sinonasal involvement. We report a rare case of systemic and disseminated craniospinal Rosai-Dorfman disease with intraparenchymal and leptomeningeal involvement, but no sinus or dural-based disease. The diagnosis was established by biopsy of a hypothalamic mass. Additionally, UCSF500 Next Generation Sequencing demonstrated a solitary pathogenic alteration affecting the BRAF oncogene, which supports the morphologic and immunohistochemical diagnosis of Rosai-Dorfman disease.
1. La Barge D, Salzman KL, Harnsberger HR, et al. Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): Imaging Manifestations in the Head and Neck. AJR Am J Roentgenol . 2008;191(6):W299-306.
2. Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, et al. Rosai-dorfman disease of the central nervous system: Report of 6 cases and review of the literature. Medicine (Baltimore) . 2014;93(3):165-175.
3. Mar WA, Jonathan HY, Knuttinen MG, et al. Rosai-dorfman disease: Manifestations outside of the head and neck. AJR Am J Roentgenol . 2017;208(4):721-732.
4. Tan S, Ruan L, Jin K, et al. Systemic Rosai–Dorfman disease with central nervous system involvement. Int J Neurosci . 2018;128(2):192-197.
5. Symss N, Cugati G, Vasudevan MC, et al. Intracranial Rosai Dorfman Disease: Report of three cases and literature review. Asian J Neurosurg . 2010;5(2):19-30.
6. Brito J, Sousa V, Teixeira LS, et al. Rosai-Dorfman Disease Manifesting as a Mediastinal Mass with Calcifications. J Thorac Imaging . 2010;25(2):W30-2.
7. Ahuja J, Kanne JP, Meyer CA, et al. Histiocytic Disorders of the Chest: Imaging Findings. Radiographics. 2015;35(2):357-70.
8. O’Gallagher K, Dancy L, Sinha A, et al. Rosai-dorfman disease and the heart. Intractable Rare Dis Res. 2016;5(1):1-5.
9. Raslan OA, Schellingerhout D, Fuller GN, et al. Rosai-Dorfman disease in neuroradiology: Imaging findings in a series of 10 patients. AJR Am J Roentgenol . 2011;196(2):W187-93.
10. Huang BY, Liu HL, Yu CJ. Isolated Intramedullary Spinal Rosai-Dorfman Disease: A Case Report and Literature Review. World Neurosurg. 2016;88:694.e11-694.e15.
2. Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, et al. Rosai-dorfman disease of the central nervous system: Report of 6 cases and review of the literature. Medicine (Baltimore) . 2014;93(3):165-175.
3. Mar WA, Jonathan HY, Knuttinen MG, et al. Rosai-dorfman disease: Manifestations outside of the head and neck. AJR Am J Roentgenol . 2017;208(4):721-732.
4. Tan S, Ruan L, Jin K, et al. Systemic Rosai–Dorfman disease with central nervous system involvement. Int J Neurosci . 2018;128(2):192-197.
5. Symss N, Cugati G, Vasudevan MC, et al. Intracranial Rosai Dorfman Disease: Report of three cases and literature review. Asian J Neurosurg . 2010;5(2):19-30.
6. Brito J, Sousa V, Teixeira LS, et al. Rosai-Dorfman Disease Manifesting as a Mediastinal Mass with Calcifications. J Thorac Imaging . 2010;25(2):W30-2.
7. Ahuja J, Kanne JP, Meyer CA, et al. Histiocytic Disorders of the Chest: Imaging Findings. Radiographics. 2015;35(2):357-70.
8. O’Gallagher K, Dancy L, Sinha A, et al. Rosai-dorfman disease and the heart. Intractable Rare Dis Res. 2016;5(1):1-5.
9. Raslan OA, Schellingerhout D, Fuller GN, et al. Rosai-Dorfman disease in neuroradiology: Imaging findings in a series of 10 patients. AJR Am J Roentgenol . 2011;196(2):W187-93.
10. Huang BY, Liu HL, Yu CJ. Isolated Intramedullary Spinal Rosai-Dorfman Disease: A Case Report and Literature Review. World Neurosurg. 2016;88:694.e11-694.e15.
| Files | ||
| Issue | Vol 15, No 4 (2021) | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/ijhoscr.v15i4.7482 | |
| Keywords | ||
| Rosai Dorfman Disease Histiocytosis BRAF | ||
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Li Y, Sloan E, Bollen A, Solomon D, Theodosopoulos P, Cha S. Systemic and Craniospinal Rosai Dorfman Disease with Intraparenchymal, Intramedullary and Leptomeningeal Disease. Int J Hematol Oncol Stem Cell Res. 2021;15(4):260-262.
