| Identifier |
walsh_2020_s2_c4 |
| Title |
Tissue Is the Issue |
| Creator |
Christine Greer; Jasmine Francis; Eli Diamond; Charles Eberhart; Marc Dinkin |
| Affiliation |
(CG) (MD) Weill Cornell Medical College, New york, New York; (JF) (ED) Memorial Sloan Kettering Cancer Center, New York, New York; (CE) Johns Hopkins University School of Medicine, Baltimore, Maryland |
| Subject |
Optic Neuritis, MRI, Horizontal Gaze Palsy |
| History |
The pathologic specimen demonstrated atypical histiocytic proliferation and infiltration with abundant cytoplasm, CD68+, S100+, consistent with Rosai Dorfman (RDD). The patient had been treated with steroids with a partial response and disease stabilization with radiation therapy with subsequent progression. He was treated with cobimetinib as a part of the clinical trial #15-216 with a partial response. He progressed off treatment and was treated with cladribine with a complete response of his parenchymal disease in early 2019. He was readmitted with vision loss while off treatment. In light of this history, we presumed that the optic nerve and chiasm disease in this case was infiltration by RDD. He was treated with steroids and re-initiated of MEK inhibitor therapy. |
| Disease/Diagnosis |
The pathologic specimen demonstrated atypical histiocytic proliferation and infiltration with abundant cytoplasm, CD68+, S100+, consistent with Rosai Dorfman (RDD). The patient had been treated with steroids with a partial response and disease stabilization with radiation therapy with subsequent progression. He was treated with cobimetinib as a part of the clinical trial #15-216 with a partial response. He progressed off treatment and was treated with cladribine with a complete response of his parenchymal disease in early 2019. He was readmitted with vision loss while off treatment. In light of this history, we presumed that the optic nerve and chiasm disease in this case was infiltration by RDD. He was treated with steroids and re-initiated of MEK inhibitor therapy. |
| Date |
2019-03 |
| References |
1.AlWadani, S, Robinson, S, Myers, R, Akpek, EK, Eberhart, CG. No increase in IgG4-positive plasma cells in limbal Rosai-Dorfman disease. Cornea. 33:844-847, 2014. 2. Cavouto K, Galor A, Dubovy SR, Gregori N, McCarthy M. Subconjunctival masses associated with central nervous system rosai-dorfman disease.Cornea. 30(2), 237-240, 2011. 3. Dalia S, Sagatys E, Sokol L, Kubal T. Rosai-dorfman disease: Tumor biology, clinical features, pathology, and treatment.Cancer control : journal of the Moffitt Cancer Center. 21(4), 322-327, 2014. 4. Diamond, E.L., Durham, B.H., Ulaner, G.A. et al. Efficacy of MEK inhibition in patients with histiocytic neoplasms. Nature 567, 521-524, 2019. 5. Foucar E, Rosai J, Dorfman RF. The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. American Journal of Ophthalmology, 87(3), 354-367, 1979. 6. 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. 93(3),165-175, 2014. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
2020 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting 2020: Frank B. Walsh Session 1 |
| Collection |
Neuro-Ophthalmology Virtual Education Library - Walsh Session Annual Meeting Archives https://novel.utah.edu/Walsh/index3.html |
| Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-6037 |
| Rights Management |
Copyright 2020. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6ht7wwh |
| Setname |
ehsl_novel_fbw |
| ID |
1534162 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6ht7wwh |
