case_images: PMC6448025_01
case_images: Axial T1-weighted images between C3 and C4 of T1-weighted cervical spine magnetic resonance imaging (MRI) after gadolinium injection administration show dominant, patchy intense enhancing left-sided lesion within the spinal cord (arrows) 1 month after surgery, in pre-treatment with vemurafenib, 8 weeks after the beginning of vemurafenib, 2 months after vemurafenib discontinuation, and last follow-up (A). Sagittal post contrast T1-weighted images revealed lesions between C3 and C4 as well as C4 and C5 (arrows) 1 month after surgery, in pre-treatment, 8 weeks after beginning treatment, 2 months after discontinuation, and last follow-up (B). Sagittal T2-weighted images showing syringomyelia (arrowheads) rostral and caudal to the intramedullary tumor 1 month after surgery, at pre-treatment, 8 weeks after beginning treatment, 2 months after discontinuation, and last follow-up. Note the T2 hyperintensity in the spinal cord above and below the syringomyelia without associated enhancement (C).
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case_images: PMC6448025_01_fonc-09-00177-g0001.jpg
case_images: F1
case_images: Moreover, T2 hyperintensity was observed in the spinal cord above and below the syringomyelia without associated enhancement (Figure 1). Tumor measurements were then 36 x 12 mm, corresponding to a 50% increase in size (Figure 1). After 8 weeks of treatment, the patient was neurologically stable and brain MRI showed a >50% decrease in tumor size (Figure 1). Two months after stopping treatment, MRI revealed that the disease was stable and had not significantly progressed according to RANO criteria (Figure 1). Twenty-one months after vemurafenib discontinuation in October 2018, MRI and neurological examination showed stable disease (Figure 1) and the patient had no side effects.
case_images: Astrocytic glial cell population with grouped ganglion cells, marked in hematoxylin-Eosin-Saffron staining (original magnification X18) (A), neuronal components characterized by larger numbers of binucleated or multinucleated cells (arrowheads) with a cellular glial background evaluated by hematoxylin-Eosin-Saffron staining (original magnification X28) (B).
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case_images: PMC6448025_01_fonc-09-00177-g0002.jpg
case_images: F2
case_images: The histological examination of the lesion showed a tissue with mixed glial and neuronal components (Figures 2A,B), the presence of fusiform cells with anisonucleosis, sustained by blood vessels with thickened wall surrounded by lymphocytic cuffs, with eosinophilic granular bodies, and Rosenthal fibers.
case_images: Dermatological toxicities after 13 months of vemurafenib, representing a grade II maculopapular rash (A), microcysts (B), and hyperkeratosis as part of palmar-plantar erythrodysesthesia syndrome (C).
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case_images: PMC6448025_01_fonc-09-00177-g0003.jpg
case_images: F3
case_images: Adverse Events v4.0 and included grade I myalgia, arthralgia, and asthenia as well as grade I maculopapular rash (folliculitis with microcysts on legs and arms treated with topical retinoids). After 13 months of treatment, the patient decided to stop the treatment because of grade II photosensibility and other dermatological side effects (Figures 3A-C).