Centre de référence
des maladies rares
du pancréas

Solid Pseudopapillary Neoplasm (SPN) Or Frantz’s Tumour

What is SPN or Frantz’s tumour?

Solid pseudopapillary neoplasm (SPN) or  Frantz’s tumours are rare tumours described in 1959 by Frantz. The prevalence is unknown. Gender ratio is essentially female: 1/10. SPN occurs most commonly in black populations (>50%) and young women (<30 years of age). It is usually discovered by chance. Complications are possible in large tumours: intralesional haemorrhage and pain due to capsular pressure or rupture. Mean diameter at diagnosis is considerable: >9 cm. This type of benign exocrine tumour is mixed, solid and liquid. In 2/3 cases it is located in the pancreatic body and tail.

What are the treatment options?

Treatment is essentially early surgical resection. Complete ablation of the capsule (active tumour zone) is important to limit the risk of relapse, and to perform a bloc resectionso as to limit risk of dissemination.

Similarly, where SPN is suspected, it is highly inadvisable to perform needle biopsy, to limit the risk of rupture or dissemination.

The few documented cases report a low risk of malignity; nevertheless, 5% - 15% of metastasized cases were noted, and a 3% - 5% mortality rate. If resection is complete, with no rupture of the capsule, the risk of recurrence is virtually nil.

In cases of synchronous hepatic and/or peritoneal metastases, total resection of the lesions is advisable. The literature reports resections (+/- following chemotherapy depending on cases, no recommendations concerning protocol), and similarly in rare cases of liver transplant.