case_images: PMC11131344_01
case_images: Preoperative and postoperative MRI imaging.. Preoperative mid-sagittal (A) and coronal (B) postcontrast T1 WI showing a lobulated solid enhancing 3rd intraventricular mass, displacing the optic chiasm (Ch) downwards and forwards, bowing the floor of the third ventricle (3V) and sparing the pituitary stalk, infundibulum and the chiasmatic cistern (Ch cs), associated with subsequent obstructive hydrocephalus (asterisks). Serial mid-sagittal postcontrast T1 WI images (C) immediately following debulking of the 3rd intraventricular mass showing residual enhancing component (arrow) and resolution of the hydrocephalus (asterisks), (D) significant progression of the mass without treatment on the 4-month postoperative follow-up scan. Vemurafenib and cobimetinib combination therapy was started within 2 weeks and the 3-month post combination therapy shows significant reduction in the size of the residual mass (E). (F) 11-days after the treatment is held, the residual mass continues to shrink and show cavitary changes. Most recent mid-sagittal (G) and coronal (H) postcontrast T1 WI showing near complete resolution of the mass with a subcentimetric residual enhancing focus which remained stable for 1 year since it attained this size (85 weeks since treatment was withheld).
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case_images: PMC11131344_01_ICNS_A_2336805_F0001_C.jpg
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case_images: MRI showed a 3.1 cm homogenously enhancing mass within the third ventricle with moderate enlargement of lateral ventricles and mass effect on the optic chiasm (Figure 1). MRI was consistent with disease progression four months after initial surgery (Figure 1D). MRI findings indicated enlargement of the suprasellar craniopharyngioma from 2.4 x 2.0 x 1.7 cm to 2.3 x 2.3 x 3.0 cm, additional 0.6 cm diameter increase of the third ventricles bilaterally, and mild mass effect on the optic chiasm and infundibulum. After 1 cycle of the combination therapy, the MRI showed a significant decrease in the size of the lesion from 2.3 x 2.3 x 3.0 cm to 1.0 x 1.0 x 1.2 cm and stable size in bilateral lateral ventricles (Figure 1E). MRI has since demonstrated lesion size stability with the mass measuring 0.4 x 0.3 x 0.3 cm (Figure 1H) now 29 months from holding treatment.
case_images: Histopathological findings.. Histopathology of the tumor, with H&E histology at 40x magnification (A), 400x magnification (B) and BRAF V600E immunohistochemistry at 400x magnification (C).. An epithelium-lined mass with papillary configuration is noted (A) consisting of well-differentiated stratified squamous epithelium (B) overall similar to the appearance of a squamous papilloma, and in this anatomic location, characteristic of a papillary craniopharyngioma. Mutation specific immunohistochemistry for BRAF V600E was positive (C), also typical of papillary craniopharyngioma.
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case_images: PMC11131344_01_ICNS_A_2336805_F0002_C.jpg
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case_images: Notably, pathology findings indicated a papillary craniopharyngioma with a BRAF V600E mutation (Figure 2).