Selinexor is in clinical development for the treatment of adults with advanced unresectable dedifferentiated liposarcoma (DDLS). Liposarcoma is a malignancy of fat cells and represents the most common form of soft tissue sarcoma. The prognosis varies depending on tumour site, size, grade, and histologic subtype. DDLS occurs when a low-grade tumour changes and newer cells with higher grade arise in the tumour. DDLS is frequently found in the retroperitoneum and the extremities (arms and legs) and is characterised as a painless mass, though it can cause symptoms of obstruction, decrease in appetite and abdominal distention.
Selinexor works by selectively inhibiting nuclear export (SINE) compounds. By blocking a protein called Exportin 1 (XPO1), responsible for the transport of the major tumour suppressor proteins from the nucleus to the cytoplasm, the selinexor blocks the nuclear export of tumour suppressor, growth regulatory, and anti-inflammatory proteins, leading to accumulation of these proteins in the nucleus and enhancing their anticancer activity in the cell. If licensed, selinexor will offer a treatment option for patients with advanced unresectable DDLS