Follicular dendritic cell sarcomacancer arising from bones and/or soft tissue (FDCS) is a rare and slow-growing malignancy that commonly develops in the lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid. More specifically, it develops in dendritic cellsthe basic structural and functional unit of all living things, which play a vital role in immune function.
There are two types of FDCS, which are categorised by where they originate. The most common type is nodal FDCS, which develops in the dendritic cells in the lymph nodes. This variant is most commonly diagnosed in cervical and axillary (arm pit region) lymph nodes. The less common type is called extranodal FDCS, which develops outside of the lymph nodes. This variant can develop in areas such as the head and neck, digestive systema group of organs and tissues responsible for breaking down food, absorbing nutrients, and eliminating waste, spleen, liver, lungs, skin, and mediastinumthe space between the lungs that holds many important structures, including the heart, trachea and oesophagus.
FDCS is most commonly diagnosed in people over 50; however, anyone of any age and gender can develop this disease.
Treatment
If FDCS is detected, it will be staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumoura tissue mass that forms from groups of unhealthy cells) indicates the size and depth of the tumour.
- N (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissuea group of cells that work together to perform a function.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localisedaffecting only one area of body cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including age, stage of disease and overall health. The types of treatments generally don’t vary between adults and children.
Treatment options for FDCS may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence to remove as much of the tumour as possible.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Targeted therapymedication that targets specific molecular features of cancer cells.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
Because of how rare FDCS is, there has been minimal research into the riskthe possibility that something bad will happen factors of this disease. However, some conditions have demonstrated a potential link to the development to FDCS, including:
- Castleman’s disease.
- Epstein Barr virus.
- Paraneoplastic pemphigus.
- Myasthenia gravis.
Not everyone with these conditions will develop the FDCS and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Symptoms
In the early stages of FDCS, symptoms are often absent or confused for other conditions. As the cancer progresses, symptoms may appear but will vary depending on location. Some of these symptoms may include:
- Painless swelling, inflammation and/or lump in lymph node (nodal FDCS) or elsewhere (extranodal FDCS).
- Fever.
- Unexplained weight loss.
- Abdominal pain that worsens as abdominal massa growth of cells that come together to make a lump, may or may not be cancer grows.
- Cough and/or sore throat.
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
- Night sweats.
Not everyone with the symptoms above will have cancer but see your GP if you are concerned.
Diagnosis
FDCS is often misdiagnosed as other conditions. If your doctor suspects you have FDCS, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- PET (positron emission tomography) scana type of medical imaging that uses radioactive tracers to create detailed images of the body.
- X-raya type of medical imaging that uses x-ray beams to create detailed images of the body .
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
