Solid pseudopapillary neoplasm of the pancreas in children: Hacettepe experience

Solid pseudopapillary neoplasm of the pancreas (SPNP) is mostly seen in young women in the second and third decades of life; it is quite uncommon in children. We aimed to review our institutional experience with SPNP in children.

Hospital charts of children <18 years of age diagnosed to have SPNP were reviewed for demographic characteristics, presenting symptoms, diagnostic interventions, physical examination findings, radiological data, extent of disease, diagnostic and management strategies and final outcome.

Nineteen cases were diagnosed as SPNP between 1992 and 2017 (female: male, 16:3; median age 13 years, range 8.5-16.5). The most common symptom was abdominal pain. Physical examination was normal in 12/19 cases. Three cases were diagnosed incidentally. The most common tumour localization was the head of the pancreas. Median tumour diameter was 5 cm (1.4-15). One patient had abdominal disseminated disease, and others had localized disease. Surgical interventions were enucleations in nine, distal pancreatectomies in four and total resection in two patients; three underwent no surgery following diagnosis. Only one patient received adjuvant chemotherapy and radiotherapy. One patient died, one was lost to follow-up at 164 months and 17 were under follow-up with no events at a median of 60 months (20-308 months).

In childen, SPNP demonstrates different clinical features. Complete resection is curative in most patients. In children, the optimal surgical strategy for SPNP is still debatable. Instead of radical resections, limited pancreatic resections, such as enucleations, with negative surgical margins should be attempted. For unresectable or recurrent tumours, cisplatin and 5-FU-based chemotherapy might be considered.