Pleomorphicmany forms; cells that have different size, shape etc. dermal sarcomacancer arising from bones and/or soft tissue (PDS), also known as undifferentiated pleomorphic sarcoma of the skin, cutaneous undifferentiated pleomorphic sarcoma, or superficial malignantcancerous, may grow and spread to other areas of the body fibrous histiocytoma, is a very rare malignancy that develops on the skin, most commonly in the head and neck regions. It is a rare variant of undifferentiated pleomorphic sarcoma (UPS), which develops in the soft tissues or bones of the body, most commonly in the arms and legs.
The skin is the largest organ in our bodies that protect us from injury, loss of bodily fluids, and helps regulate body temperature. There are three layers of the skin: epidermis (top layer), dermis (middle layer) and hypodermis (bottom layer). The epidermis is the water-resistant outer layer of the skin that acts as the body’s first line of defence. It contains squamous cellsthe basic structural and functional unit of all living things (upper layer of the epidermis), basal cells (lower layer of epidermis) and melanocytes. The dermis contains the skin’s connective tissues, as well as hair follicles, sweat glands, bloodthe red bodily fluid that transports oxygen and other nutrients around the body vessels, lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid and nerves. The hypodermis, also known as subcutaneousunder the skin tissuea group of cells that work together to perform a function layer, stores fat (adipose cells), and also contains connective tissue, blood vessels and nerve cells.
PDS is often confused with atypical fibroxanthoma, as they have a similar cellular appearance when examined under the microscope. While the two appear similar, PDS tends to be more aggressive and grows deeper into nearby structures.
PDS is slightly more common in men, and tends to be diagnosed in patients over 70 years old. However, anyone can develop this disease.
Treatment
When cancers are detected, they are 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. However, because of how rare PDS is, there is currently no standard staging and grading system for this disease. Instead of staging and grading, your doctor will recommend a treatment plan based on the following factors:
- Cancer location.
- Whether or not the cancer has metastasised.
- Your age.
- General health.
- Your treatment preferences.
Your doctor may also 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 tumoura tissue mass that forms from groups of unhealthy cells 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 options for PDS 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.
- 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 PDS is, there has been limited research done into the riskthe possibility that something bad will happen factors of this disease. However, few factors have been identified:
- UV exposure causing sun damaged skin.
- Previous radiation therapy.
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Symptoms
Early symptoms of PDS may include:
- Rapidly growing plaques or nodules, often over 2cm in diameter.
- Ulceration of affected area.
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have a PDS, 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.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
