Usually PCNSMZL presents as extra-axial dural-based masses; it is rarely localized in glomus of the choroid plexus in lateral ventricles, cortex, basal ganglia, cerebellopontine angle and retina. Symptoms are related to the tumour mass effect and they are often long standing. The most common MRI presentation is a single mass with sharp margins and ample dura implantation. Clinical-radiological suspicion of PCNSMZL should be always confirmed by histopathological examination. Staging workup should include MRI of the encephalon and/or spinal cord, CSF examination, PET, total-body CT scan and bone marrow biopsy. Surgery, radiotherapy and chemotherapy in different combinations were effective in obtaining CR in most of the treated patients. A therapeutic algorithm has been proposed, but standard of care still remains to be defined.

Safety and Efficacy of CAR-T and BsAb in prior allo-HCT in RRMM.

Advanced systemic mastocytosis (SM) includes aggressive SM (ASM), SM with associated myeloid neoplasm (SM-AMN) and mast cell leukaemia (MCL). MCL has the worst prognosis and requires the immediate institution of cladribine, midostaurin or avapritinib, followed by an allogeneic haematopoietic stem cell transplant (AHSCT). Prognosis in SM-AMN depends on the prognosis of the associated myeloid neoplasm as well as the nature of the SM component (i.e. indolent or aggressive), but often requires AHSCT to secure long-term survival. ASM is initially managed with drugs but might ultimately require AHSCT, depending on treatment response.