case_images: PMC10598752_01
case_images: Treatment timeline in a girl with progressive adamantinomatous craniopharyngioma. The timeline for treatment interventions for progressieve adamaninomatous craniopharyngioma from 11 till 16,5 years old is shown in the graph. Four neurosurgical interventions are shown with arrows, while the radiotherapy period is depicted with a lightning bold pictogram. The systemic treatment was composed of biweekly 800 mg tocilizumab intravenously.
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case_images: A 15-year-old girl showed ongoing cystic progression of residual ACP with treatment interventions shown in  Figure 1 .
case_images: Course of craniopharyngioma on MR imaging. The MRI at diagnosis performed in a child of 11 years old shows a suprasellar and sellar cystic-solid lesion with calcifications, measuring a maximum of 5,2 cm in cranio-caudal direction visible on a sagittal image, a T1 3 mm 0,3 gap with gadolinium (A) and on a coronal image, T2 TSE 3 mm 0,3 gap (B) consistent with the diagnosis of a craniopharyngeoma. After four previous neurosurgical resections a cystic lesion close to the right optic nerve increases up to 1,1 cm visible on a sagittal image after contrast (C) and on the axial image T2 TSE, 3 mm without gap (D).
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case_images: The size was 5.2 cm craniocaudal x 3.3 cm anterior-posterior growing into the third ventricle, infiltrating the hypothalamus, compressing the thalamus, and stretching the chiasm anterior and downwards ( Figures 2A, B ). Because of the slight decrease in the BCVA of her left eye to 0.1 decimal (1.0 LogMAR) combined with the subsequent fifth progression with new cystic growth until 0.9 cm x 1.0 cm x 1.0 cm ( Figures 2C, D ) at 15 years of age, we decided to start experimental therapy with the anti-interleukin-6 agent tocilizumab (TCZ) (20 mg/ml) 800 mg every 2 weeks intravenously during 1 h.
case_images: Visual field changes in a patient with cystic craniopharyngioma. Visual field of the left eye restricted to the inferionasal kwadrant measured with Goldman method at start of treatment (A) with tocilizumab and at end of treatment (B).
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case_images: Post-operatively, her vision deteriorated with an episode of complete blindness, where after the right eye remained without light perception and in the left eye, a best corrected visual acuity (BCVA) of 0.16 decimal (LogMAR, 0.8) was measured combined with a large visual field defect represented by an absolute temporal hemifield and superonasal deficit ( Figure 3A ). Despite the TCZ treatment, longitudinal analyses of visual functions showed a small decrease in BCVA to 1.2 LogMAR and an additional constriction of her visual field to an inferonasal remnant ( Figure 3B ).
case_images: (A-C) (HE): histological features adamantinomatous craniopharyngioma. (A): squamous epithelial tumour with basal palisading of tumour cells, stellate reticulum, and wet keratin (asterisk). (B): prominent calcifications in the cyst wall (arrows). (C): interface of adamantinomatous craniopharyngioma and brain tissue with Rosenthal fibers present in the reactive piloid gliosis surrounding the tumor (arrows and insert). (D-H): Immunohistochemistry. (D) (GFAP) shows reactive piloid gliosis. (E, F) (CD68) shows macrophages (arrows) in the cyst wall (E) and around the tumor (F). (G, H) (CD3): T-lymphocytes (arrows) located around and focally (H) within the tumor. Scale bars: (A-C), 100 microm; (D-H), 50 microm.
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case_images: Extensive histopathological analysis of the ACP was performed with the tissue of the latest surgery showing confluent epithelial fields of multilayer and matured squamous epithelial cells with basal palisades and variable degrees of keratinization and focal calcification ( Figure 4 ).