Pancreatic rare tumours


What is pancreatic cancer?

What is cancer?

Cells are the building blocks that form different structures in the body. Genes contain the instructions in cells for making new proteins, which give every cell its specific function. These regulatory elements control how cells behave. Abnormal changes (mutations) in genes can turn normal cells into cancer cells through complex mechanisms.

Cell division is a tightly controlled mechanism in which a cell will create two daughter cells. These new cells replace injured or dying cells as needed. This duplication is controlled by several proteins, which ensure the correct progression through the cell cycle (process that the cell follows to duplicate). These proteins can be accelerators to progress through the cell cycle, or can act as brakes to provide time for the cell to correct duplication of the DNA and avoid introducing mutations. Normal cells stay where they belong and do not spread to other parts of the body; they die as they grow old, or become damaged.

A cancer cell can duplicate extensively and without control for two reasons: the accelerator protein (proto-oncogene) has a mutation which means the acceleration cannot be stopped, or the brake protein (tumour suppressor) has a mutation that does not allow them to work properly. Sometimes, both events can happen in different proteins making them difficult to treat.

Cancer cells duplicate extensively until they produce a massive amount of cells. The initial proliferative cells form a solid mass, called a primary tumour. Sometimes, cancer cells do not stay in place, as they should; they will invade other tissues giving place to metastasis. Researchers are still learning what causes genes to mutate and cause cancer.

This is a very broad explanation of what cancer is, and it applies more to adult cancers. Most paediatric cancers develop because of sporadic epigenetic alterations. A very simple classification for paediatric cancers can be the following:

  • Intracranial solid tumors
  • Extracranial solid tumors
  • Leukaemias

Cancer may occur anywhere in the body. Cancers are named depending on the type of cell that triggers the uncontrolled proliferation.

The basics of pancreatic cancer

When the primary tumour is located in the pancreas, it is called pancreatic cancer. It can cause abdomen pain, weight loss, diabetes, jaundice, etc., depending on the affected part of the pancreas. The most common cancer of the pancreas is the pancreatic ductal adenocarcinoma (PDAC) and often, the term “pancreas cancer” relates to this type of tumour.

However, there are other pancreatic cancers with diverse characteristics, patterns and behaviours. Although very rare, the commonest pancreatic tumours in children are solid pseudo-papillary neoplasm and pancreatoblastoma.1 Here we will discuss different neoplasms of non-ductal origin, which comprise the 5% of the pancreatic tumours.2

1Mylonas, K. S. et al. Solid pseudopapillary and malignant pancreatic tumors in childhood: A systematic review and evidence quality assessment. Pediatr. Blood Cancer 65, e27114 (2018).
2Mostafa, M. E., Erbarut-seven, I., Pehlivanoglu, B. & Adsay, V. Pathologic classification of “ pancreatic cancers ”: current concepts and challenges.
Last modified
30 December 2019