Desmoid general

What is desmoid?

Another name for desmoid tumour is aggressive fibromatosis, or desmoid fibromatosis.

A desmoid tumour is a tumour caused by an overgrowth of cells from connective tissue (fibroblasts) in the deep layers of the soft tissues. This type of tumour continues to grow with fine tentacles into the surrounding area (infiltrative growth), so it can recur rapidly if not completely removed. This is a benign tumour, meaning it does not spread to other sites. Thus, a desmoid tumour cannot reach another part of the body via blood or lymph.

However, the tumour may grow aggressively locally and cause symptoms as a result.

Where and in whom do desmoid tumours occur?

This tumour is very rare, about 2-4 people per million people per year develop such a tumour. The age at which the tumour develops ranges from 15-60 years with a clear peak around the age of 30. It is more common in women.

Desmoid tumours can be subdivided by location:

• Belly wall
• Abdominal cavity (medical term: intra-abdominal)
• Areas outside the abdomen (medical term: extra-abdominal)
• Head, neck and neck
• Chest and chest cavity
• Limbs (arm or leg)

In children, most neoplasms are seen outside the abdominal cavity. Between puberty and age 40, this tumour is usually seen in the abdominal wall in women, in many cases during or shortly after pregnancy. In people older than 40, this tumour is found both inside and outside the abdominal cavity.

How do desmoid tumours arise?

In most patients, the desmoid tumour develops spontaneously due to a DNA error (mutation) in one body cell. This allows the cell to divide uninhibitedly. In these patients, the tumour is not hereditary. There is usually no obvious triggering factor for the development of a desmoid tumour. A hormonal connection is suspected, as this tumour is often found during or after pregnancy. Sometimes there is a history of trauma.

In rare cases, the tumour develops on the basis of a genetic disorder, namely familial adenomatous polyposis (Gardner syndrome). This condition is characterised by polyps in the colon.

What symptoms does this tumour cause?

You feel a hard bulb deep under the skin. The diameter of the tumour can vary greatly, going the from a few centimetres to 30 cm or more. If the tumour is around a joint, you may not be able to move this joint as well. Sometimes a desmoid tumour can be located around a nerve, which can trigger nerve pain by compressing the nerve. Also, if the tumour is in the abdominal cavity, you may experience abdominal pain, intestinal discomfort or sometimes blood loss.