Stabilisation of alveolar soft part sarcoma with pazopanib as single-agent therapy and renewed effects post therapy break

Abstract

This case study presents a 16-year-old female who was originally diagnosed with Alveolar Soft Part Sarcoma of the right shoulder in 2008, which was completely excised. During routine surveillance, pulmonary metastases were diagnosed in October 2013. A trial of chemotherapy including anthracycline had no benefit, and she was monitored throughout 2014. In early 2015, she presented with a sudden onset of blindness. Multiple enhancing cranial metastases were diagnosed with significant vasogenic oedema. The known pulmonary metastases had also increased. She was initially stabilised with steroids and received whole brain irradiation with adjuvant temozolamide. An MRI in March 2015 demonstrated an increase in size of cerebral metastases and extent of oedema. She was commenced on Pazopanib in April 2015 with initial reduction in size of all metastases. ByOctober 2015 steroids were able to be weaned with the aid of a VP shunt. Over the next 12 months, three monthlyMRI scans demonstrated no new lesions with relatively stable disease. Tolerance eventually developed to pazopanib with disease progression evident, and itwas decided to enrol her on a clinical trial involving pembrolizumab in late 2016. Unfortunately this was not able to stem the progression of the cerebellar lesion and a new lesion in the pelvis was also found. However, she remained clinically stable and surgery was able to be performed on her cerebellar metastasis and the pelvic mass. Pazopanibwas subsequently recommenced in early 2017 and the most recent scans show stable disease again with no local recurrence. It appears the tumour has had renewed sensitivity to single-agent therapy when allowed to have a break from the immunotherapy. This case study demonstrates the significant effects of immunotherapy on stabilising disease. Re-challenging in the setting of previous tolerance should be considered as there may be renewed sensitivity.L6193514292017-11-27 <br />