علاج الورم النخاعي عندما يفشل خط العلاج الأول عالي الخطورة في تحقيق الانكماش الكافي للورم

References

DOI: 10.1016/S1470-2045(19)30669-2

• High risk • Residual tumour >1·5 cm² • >M1 • Large cell/anaplastic histology • Group 4 molecular subgroup • SHH TP53mut
• Second surgery should be done if a total resection appears possible and in cases of disseminated tumour if symptoms can be relieved (level III A; eg, if the spinal cord is compressed by solid intraspinal metastases).
• In selected paediatric cases who initially received reduced dose craniospinal irradiation, salvage treatment with a second craniospinal irradiation appears feasible.
• In cases of focal relapse, focal radiotherapy can be used also in adults (level III B).
• Intravenous chemotherapy with carboplatin and etoposide was explored in the HIT-REZ-2005 study (NCT00749723); an oral alternative consists of a combination chemotherapy with etoposide and trofosfamide.
• Other regimens, such as metronomic and targeted anti-angiogenesis therapy (NCT01356290), topotecan and temozolomide (NCT00918320) or temozolomide and irinotecan, some of them supplemented with bevacizumab can be considered on an individual basis.
• If molecular data are available, sonidegib or vismodegib, with or without cytostatic therapy, can be considered in recurrent or refractory adult patients with SHH medulloblastoma on the basis of their biology, despite the scarce available data that show individual objective responses in small cohorts.