Solid pseudopapillary tumour (Frantz's 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.

Solid pseudopapillary tumour (Frantz's tumour) of the pancreas in childhood.

An 11-year-old girl presented with acute pancreatitis and mass in the head of the pancreas. MRI revealed a heterogeneous right-upper quadrant retroperitoneal mass measuring 6.8×6.1×5.5 cm arising from the pancreatic head. Endoscopic ultrasound with fine-needle aspirate revealed a solid pseudopapillary tumour (SPT) of the pancreas. The patient underwent a pylorus-preserving Whipple procedure. Pathology confirmed SPT. First described by Frantz, SPT represents less than 3% of all exocrine tumours. It is especially rare in children and shows different clinical features compared with adults. In our patient, tumour cells were arranged at the periphery of fibrovascular cores, but they did not show definite gland formation, keratinisation or cytoplasmic pigment accumulation. A periodic acid-Schiff stain without diastase did not show appreciable glycogen within the tumour cells, classic for Frantz's tumour. The literature, diagnosis, management and pathogenesis on this rare entity in children are reviewed and discussed.

Oesophageal atresia associated with congenital oesophageal stenosis.

Congenital esophageal stenosis (CES) is associated with oesophageal atresia and tracheoesophageal fistula (EA-TEF). Tracheobronchial (cartilaginous) rests are typically found within the area of stenosis. The authors present a case report of EA-TEF associated with CES and present a modification of a novel technique to facilitate the management of CES. The impact of gastro-oesophageal reflux disease is discussed in this unique entity. Finally, the literature is reviewed for this unique entity.