Introduction to Desmoid Tumours

Executive Summary

Desmoid tumors originate from fibrous tissues and are associated with the body’s connective tissues. Desmoid are not cancers since they are benign growths that do not metastasize or spread to different body parts. These are non-cancerous tumors that usually grow slowly and do not cause any trouble to the body. Desmoid tumors begin in cells called fibroblasts. Due to the desmoid tumor’s relation with the fibroblast cells, the type of tumor growth is also called desmoid fibromatosis, aggressive fibromatosis or deep fibromatosis. These tumors can occur in both children and adults. It appears in any body part. Different types of desmoid depend on their location and include abdominal wall desmoid tumors, intra-abdominal, and extra-abdominal. Various screening techniques or diagnostic tests are used to detect whether a tumor is a desmoid tumor. A biopsy would be required to arrive at an accurate result. Specific genetic tests are also performed to understand more about the tumor.

What are Tumors?

Before talking about desmoid tumours we should understand what a tumour is. A tumour is a mass of cells formed when the DNA of healthy cells undergo mutation or damage and grow out of control. A tumour mass can be deemed benign or cancerous. A tumour is benign if it grows but does not spread or metastasize to other body parts. On the other hand, a malignant tumour is a tumour that proliferates and spreads or metastasizes to distant body sites. Malignant tumours can be dangerous.

Desmoid Tumors 

Desmoid tumors are tumors that originate from fibrous tissues. They are seen to be associated with the body’s connective tissues. Hence these tumors are related to connective tissue cancers called sarcomas. But desmoid tumors are not cancers since they are benign growths that do not metastasize or spread to different body parts. Since connective tissues are found throughout the body, desmoid tumor can occur in any part or body location.

Desmoid tumors are non-cancerous tumors that usually grow slowly and do not cause any trouble to the body ​1​. But certain desmoid tumors can increase and interfere with the body’s normal mechanisms and activities. Usually, these tumor are not life-threatening, but some cases of desmoid can be a threat to survival when these tumor tend to be in close contact with vital tissues and organs like the lungs, kidneys, blood vessels, nerves, intestines etc. 

Sometimes doctors and healthcare professionals may find it difficult to remove the tumor. Also, these tumors are notorious for recurring back in the body even after surgery. In some cases, the desmoid tumor shrinks on its own without any treatment and becomes inactive, almost like a scar. This condition is called spontaneous regression, and in such cases, no further treatment would be required.

Desmoid tumors begin in cells called fibroblasts ​2​. Fibroblasts are common cells found in the body’s connective tissues. These cells play a vital role in wound healing. Due to the desmoid tumor’s relation with the fibroblast cells, the type of tumor growth is also called desmoid fibromatosis, aggressive fibromatosis or deep fibromatosis ​3​. These tumors can occur in both children and adults.

Desmoid tumors can occur in any part of the body. However, these tumors are mostly seen in the abdomen, legs or arms. Based on their location, doctors describe these tumors as ​4​:

  • Abdominal wall desmoid tumors: the type of desmoid tumors that occur within the abdominal wall that surrounds the different organs in the abdomen.
  • Intra-abdominal desmoid tumors: intra-abdominal desmoid tumors occur in the mesentery, which is the tissue that provides padding to the intestines. Blood vessels branch out from these tissues. Tumors that arise in the intra-abdominal region usually doesn’t cause any symptoms until they compress any internal organ structures or could be felt as a mass or lump, or bump. 
  • Extra-abdominal desmoid tumors: such desmoid tumor occur in the regions outside the abdomen. The tumors typically develop in the chest wall, upper legs and upper arms. These tumors could be found in the neck and head region in some cases. In rare cases, desmoid tumor are seen to develop in the urinary tract system, such as in the scrotum or bladder.  

A desmoid tumor can occur in anyone. In some people, the condition occurs only once, whereas, in some others, the disease recurs back even after treatment and cure. The doctor may use various screening techniques or diagnostic tests to detect whether a tumor is a desmoid tumor or not. A biopsy would be required to arrive at an accurate result. Since desmoid tumors are a rare disease condition, it would be best to examine them by an experienced pathologist (a doctor who analyses and evaluates the tissue sample under a microscope to make a diagnosis) who is familiar with the tumor condition.

The pathologist will keenly analyze the extracted tissue under a microscope, determine its shape and size, and use special stains to revise or confirm the diagnosis of desmoid tumor. In some cases, to understand more about the tumor, specific genetic tests may be performed. If, upon diagnosis, a desmoid tumor is detected, make sure that your treatment and care is carried out by a health care team that specializes in sarcomas or tumor conditions like desmoid tumors.

References

  1. 1.
    Kasper B, Ströbel P, Hohenberger P. Desmoid Tumors: Clinical Features and Treatment Options for Advanced Disease. The Oncologist. Published online April 8, 2011:682-693. doi:10.1634/theoncologist.2010-0281
  2. 2.
    Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. European Journal of Surgical Oncology (EJSO). Published online December 2001:701-706. doi:10.1053/ejso.2001.1169
  3. 3.
    Garcia-Ortega DY, Martín-Tellez KS, Cuellar-Hubbe M, et al. Desmoid-Type Fibromatosis. Cancers. Published online July 9, 2020:1851. doi:10.3390/cancers12071851
  4. 4.
    Ganeshan D, Amini B, Nikolaidis P, Assing M, Vikram R. Current Update on Desmoid Fibromatosis. Journal of Computer Assisted Tomography. Published online 2019:29-38. doi:10.1097/rct.0000000000000790