No abstract

staging imaging, and the patient was started on treatment with pembrolizumab. A review of the existing literature showed approximately 45 case reports to date, with the first in 1901. Treatment approaches have been varied, with surgery as a mainstay, either alone or with adjuvant chemotherapy or immunotherapy. Prognosis is generally accepted to be poor. Characterisation of molecular features has been limited. Given the rarity of this entity, there is no consensus for standard therapy, and evidence to date is largely anecdotal.