case_images: PMC10764088_01
case_images: Radiological intracranial evolution. a CT scan at first presentation showing a cystic PXA. b MRI of the brain showing first recurrence of the PXA 6 months after initial surgical resection. c The patient was followed by MRI which showed no evidence of disease during 14 years after gross total resection. d MRI of the brain revealing a second recurrence after 20 years of progression free survival. e MRI of the brain showing a third recurrence 3 months after third surgical resection. f MRI of the brain showing a fourth recurrence with subcutaneous after fourth surgical resection and radiotherapy (2 x 20 Gy), accompanied by multiple extracranial metastases (the latter not visible on the depicted image). g MRI of the brain showing a complete intracranial response 2 months after initiation of BRAF/MEK inhibition therapy.
case_images: cro-2024-0017-0001-534731_F01.jpg
case_images: PMC10764088_01_cro-2024-0017-0001-534731_F01.jpg
case_images: F1
case_images: A partially cystic right occipital lesion with significant mass effect was discovered on imaging of the brain (shown in Fig. 1). MRI revealed tumor recurrence with extra-cerebral soft tissue extension (shown in Fig. 1). Tumor recurrence (shown in Fig. 1) was confirmed on MRI, and a fourth surgical resection was performed. MRI of the brain performed 2 months after treatment initiation confirmed a decrease of contrast enhancement (shown in Fig. 1). [18F]FDG-PET/CT at 3 months showed an extracranial near-complete remission with disappearance of all metabolically active subcutaneous, bone, lung metastases as well as cervical and hilar adenopathies (shown in Fig. 3).
case_images: Microscopic findings. Microscopic features of the resection specimen shows a highly cellular pleomorphic proliferation with several multinucleated astrocytes (H&E, x20). There are several mitoses (arrows) and the proliferation index is above 60% (inset, Ki67, x20).
case_images: cro-2024-0017-0001-534731_F02.jpg
case_images: PMC10764088_01_cro-2024-0017-0001-534731_F02.jpg
case_images: F2
case_images: The anatomopathological report indicated transformation to a WHO grade 3 anaplastic PXA (microscopic findings are shown in Fig. 2).
case_images: Extracranial evolution assessed by [18F]FDG-PET/CT. a Baseline [18F] FDG-PET/CT showing metabolically active bone metastases left parietal, cervical C3, C4, C6 (with paravertebral extension), C7, and Th1; subcutaneous metastases from right parietal to retro-auricular right; left and right cervical adenopathies; bilateral lung metastases; and bilateral hilar adenopathies (arrows). b [18F] FDG-PET/CT 3 months after initiating therapy with BRAF/MEK inhibitors: near complete remission with residual weak metabolic activity in one cervical adenopathy at the left side (arrow).
case_images: cro-2024-0017-0001-534731_F03.jpg
case_images: PMC10764088_01_cro-2024-0017-0001-534731_F03.jpg
case_images: F3
case_images: MRI of the brain performed 2 months after treatment initiation confirmed a decrease of contrast enhancement (shown in Fig. 1). [18F]FDG-PET/CT at 3 months showed an extracranial near-complete remission with disappearance of all metabolically active subcutaneous, bone, lung metastases as well as cervical and hilar adenopathies (shown in Fig. 3).