Systemic and Craniospinal Rosai Dorfman Disease with Intraparenchymal, Intramedullary and Leptomeningeal Disease

Authors

  • Yi Li MD Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, California, United States
  • Emily Sloan Department of Pathology, University of California San Francisco, San Francisco, California, United States
  • Andrew Bollen Department of Pathology, University of California San Francisco, San Francisco, California, United States
  • David Solomon Department of Pathology, University of California San Francisco, San Francisco, California, United States
  • Philip Theodosopoulos Department of Neurological Surgery, University of California, San Francisco, San Francisco, California, United States
  • Soonmee Cha Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, California, United States

DOI:

https://doi.org/10.18502/ijhoscr.v15i4.7482

Keywords:

Rosai dorfman disease; histiocytosis; BRAF

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.

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Published

2021-10-17

Issue

Volume (15) issue (4)

Section

Articles