Solid pseudopapillary tumour (Frantzs tumour) of the pancreas in childhood: successful management of late liver metastases with sunitinib and chemoembolisation

The patient is a girl aged 17 years who originally presented at age 11 years with a solid pseudopapillary tumour (SPT) in the head of the pancreas treated by an R0 pylorus-preserving Whipple procedure. The patient underwent surveillance CT every 3 months for the first year followed by MRI every 6 months. She was noted to have a new liver lesion in Couinaud segment VI highly suspicious for metastasis at 30 months. Liver wedge biopsy confirmed metastatic SPT. Two months later two new lesions were noted in Couinaud segment VII. The family preferred medical management to surgery resulting in a treatment combination of the tyrosine kinase inhibitor sunitinib and hepatic artery embolisation. The patient developed a hepatic abscess following embolisation but recovered with antibiotics. The patient has subsequently been followed with serial MRIs every 3 months, and 20 months following chemoembolisation, she has no evidence of recurrence of the metastases.

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