A rare case of metastatic atypical meningioma that highlights the shortcomings of treatment options at present.

We report a case of a locally invasive recurrent atypical meningioma in the temporal region with late onset of meningioma lung metastasis. The patient was diagnosed in early adolescence with an atypical meningioma believed to be radiotherapy induced following treatment of a benign pilocytic astrocytoma in the hypothalamus region at 6 years of age. Even though the patient underwent several surgical and radiotherapy treatments, the intracranial meningioma kept growing and was locally invasive. The patient received experimental treatment with bevacizumab, a vascular endothelial growth factor A (VEGF-A)-inhibitor, for 4 years from age 26. Treatment was withdrawn after proven tumor growth on routine control MRI. A DOTA-TOC PET-CT-scan was performed to evaluate the DOTA-TOC somatostatin receptor number for possible SSTR (somatostatin receptor targeted therapy). In the included scan plan multiple lung metastasis were detected and later verified. Genomic tumor sequencing was performed, but no targeted treatment options were found. Instead, the patient finally, as the last treatment option, underwent 4 series of SSTR-targeted therapy (Lutetium DOTA-TOC). Unfortunately, the intracranial tumor component significantly progressed during the final stages of the treatment and the patient died less than a year after treatment was withdrawn at age 32. This case story illustrates the shortcomings of atypical/anaplastic meningioma treatment strategies at present and highlights the possibility of extracranial metastasis.