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

Abstract

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.

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

“…SPTs are slow growing biologically. In this case, if the initial recommended surveillance programme was followed,2 waiting another 6 months may have resulted in detection of the three total lesions and saved an intermediate difficult wedge biopsy step. At that time right hepatectomy would have been recommended more strongly.…”

“…The authors recommend serial CT or MRI every 3 months during the first year, 6 months for the second year and yearly thereafter, for a total of 5 years of surveillance following successful treatment of metastatic Frantz’s tumour in paediatric patients 2. The authors still recommend the surveillance programme as outlined originally for primary tumours .…”

“…As noted previously, Frantz’s tumour in children is rare and pathologically and clinically distinct from adults 2. Malignant potential and late metastases is exceedingly rare in paediatric patients, and mortality resulting from this type of tumour is approximately 1%–1.2% 3 4.…”

“…The unique clinical and pathological features of this paediatric case prompted the senior surgeon (MAE) to collaboratively prepare a case summary and review of the literature comparing paediatric with adult presentations of SPN of the pancreas 2. Based on the review of the literature, the authors recommended: serial CT or MRI every 3 months for the first year spacing out to 6 months during the second year and yearly thereafter for a total of 5 years 2…”

Frantz’s tumor in a female patient with diarrhea: case report of a rare pancreatic neoplasm

“…The disease predominantly affects young women [1][2][3]6 . Nevertheless, there are reported cases of the disease in children, male patients and old-aged 11,12 . Differential diagnoses include a variety of pancreatic tumours including non-functioning islet tumour, mucinous cystic neoplasm, serous cystadenomas, acinar cell cancer, lymphoma, pancreatoblastoma and pseudocyst.…”