Pancreatic rare tumours

Treatment for these types of cancer depends on the stage and location of it, as well as on the overall health and personal circumstances. When surgery is not an option or is not enough, treatment may include radiation, chemotherapy or a combination of these, all depending on each individual case.

PanNENs can be treated by surgery when possible. Usually, the tumour is removed through a laparotomy (minimally invasive surgery) but in some cases, it might be necessary to perform a subtotal pancreatectomy (cut and extract a piece of the pancreas) of the affected part of the pancreas.

The majority of insulinomas, the most common type of PanNEN, are benign with only a 6% incidence of malignancy, and long term survival for nonmalignant tumours after surgical resection is around 80-90% in adults and is estimated to be the same in children.^7,8

In the case of pancreatoblastoma, surgical resection is the main option too and may be accompanied by chemotherapy.⁹ If complete resection of the tumour seems unfeasible through laparotomy, an open biopsy must be performed for diagnosis.

The prognosis for pancreatoblastoma after complete resection is in general good and no chemotherapy is needed. It may metastasize, but even in this cases, with metastasectomy (removal of the metastatic mass) and after chemotherapy, patients seem to have a good prognosis.⁹ The mean rate of overall survival is around the 60-80% when complete resection is performed and interestingly, the prognosis does not seem to correlate with the tumour site and size.¹⁰

Finally, treatment is well-defined and effective for SPN. Complete surgery of the area occupied by the tumour is performed, through laparoscopy when possible.¹¹ Because the tumour has low malignant potential, no extra treatment is needed. In the rare case of this tumour wide-spreading around the body or the near area of the primary tumour, complete surgery is complemented with radiotherapy.¹²

SPNs are generally considered benign lesions although metastases can be found in some of the cases. Children have a lower percentage of malignancy than adults and the 5-year survival rate has been reported to be of 95-98% after complete resection. The prognosis for this type of tumour is generally considered very well with appropriate treatment.¹²

⁷Padidela, R. et al. Insulinoma in childhood: clinical, radiological, molecular and histological aspects of nine patients. Eur. J. Endocrinol. 170, 741–7 (2014).

⁸Peranteau, W. H. et al. The surgical management of insulinomas in children. J. Pediatr. Surg. 48, 2517–24 (2013).

⁹Bien, E. et al. Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). Eur. J. Cancer 47, 2347–52 (2011).

¹⁰Perez, E. A. et al. Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients. J. Pediatr. Surg. 44, 197–203 (2009).

¹¹Namgoong, J.-M. et al. Laparoscopic distal pancreatectomy to treat solid pseudopapillary tumors in children: transition from open to laparoscopic approaches in suitable cases. Pediatr. Surg. Int. 30, 259–66 (2014).

¹²Morita, K. et al. Solid pseudopapillary tumor of the pancreas in children: surgical intervention strategies based on pathological findings. Pediatr. Surg. Int. 30, 253–257 (2014).