July is sarcoma awareness month so there is no better time like the present to educate on this cancer subtype. As per our tradition we will level the ‘working ground’ by demystifying what type of cancer this is and who it is most likely to get affect.( I can’t bring myself to say ‘playing ground’ because we don’t play when we deal with cancer so I made up a phrase).
To start with understanding where it arises is key. Most, if not all, the cancers we have addressed so far have arisen from cells that line our organs whether inside or outside called epithelial cells. On a slightly more technical level we call these carcinomas. Our cells do not exist isolated they are supported by connective tissue and this is made up of cells and structures that can give rise to cancer called sarcoma. You will one day learn about leukemia and lymphomas.
There are two main types of sarcoma namely soft tissue and bone (see picture). Soft tissue sarcomas literally develop mostly in soft parts of the body like muscle, skin, vessels and nerves. Bone sarcomas develop from bone and cartilage. To break down the understanding further I thought to divide the information into three articles general soft tissue sarcomas, Kaposi‘s Sarcoma and Bone sarcoma. So this week we shall address the general soft tissue sarcomas.
Though soft tissue sarcomas are rare (about 1% of all malignancies) they still have an impact as they can be really nasty tumours with bad outcomes. It’s either a case of a bump that has grown slowly over a long period of time, seemingly none threatening, or sometimes a mass that grows remarkably fast and aggressively before the patient has a chance to think things through or the steps to take. They can grow very large in ‘inconvenient’ but conspicuous places and also tend to fit in with genetic predispositions in terms of risk. Some common genetic conditions are retinoblastoma, neurofibromatosis type I, Li-Fraumeni syndrome and so on. I particularly don’t like sarcomas because they can though extremely rarely, manipulate something good and beneficial like anticancer treatment in form of radiotherapy and chemotherapy and increase their own risk of developing. The explanation is that as chemotherapy and radiotherapy work they target cells that have abnormal genetic material aka cancer cells and get rid of them. However, sometimes normal cells are also injured and their own genetic material takes a knock increasing the risk of a sarcoma in these cells. Overall and in context the benefit of going through treatment outweighs the risk of developing a cancer and that is why as we have discussed in previous articles the decision for delivering cancer treatment is not one that is arrived at lightly. Such cancers develop after many years following treatment.
Other risk factors are environmental /occupational such as exposure to vinyl chloride, arsenic and thorium dioxide. Vinyl chloride is always a hot topic as it is used in the manufacture of PVC. However, it is said this compound is safe in the end products like PVC water pipes. The risk is probably more relevant to workers in the factories. Thorium dioxide is less of an issue today as it was used in a contrast medium in the 1950s. HIV infection is also linked tying in with immunosuppression as a pathway of development.
Unfortunately there are no specific preventative and screening interventions for sarcoma. Therefore early diagnosis relies on awareness and prompt action. The signs and symptoms of soft tissue sarcoma vary as it depends where the mass starts to grow. If it is in the nerve sheath or in a space where growth is restricted, the symptoms are manifested early on. In the abdomen the mass may grow to significant proportions before it is noticed. Patients may experience some universal symptoms of cancer such as unexplained unintended weight loss and fatigability.
A diagnosis can only be made by carrying out some specific tests. Though sarcoma is a serious disease it is a rare one and the first medical personnel you see want to rule out more common. A biopsy is definitely needed to determine exactly which type of soft tissue sarcoma it is and this ultimately affects the treatment plan
The cornerstone of management for most subtypes is surgery. The aim is to remove the whole tumour with good margins but if this is not possible upfront, the treating doctors may suggest some chemotherapy or radiotherapy before surgery. In some cases after surgery the indication for chemotherapy and radiotherapy remains.
Survivorship for sarcomas carries a different dimension. Patients may have large defects after surgery. The good news is reconstructive surgery is possible and these defects can be filled relatively well. Physiotherapy may be necessary to regain function if necessary. Though more common with bone cancer, patients may sometimes lose a part of their body completely like a limb or eye. An occupational therapist should probably be involved in the management of such a patient. To clarify an occupational therapist focuses on evaluating and improving a patients function as well as how to avoid future injuries. Physiotherapists concentrate on evaluating and diagnosing actual causes of movement deficiencies. They are both very knowledgeable on the musculoskeletal system.
It is well worth to mention the peculiar variants of soft tissue sarcomas such as gastrointestinal stromal tumour (GIST) which is the focus of a targeted treatment called Imatinib or Gleevec. Another special drug being used more and more is Trabectidin (Yondelis) for the treatment of a subtype called liposarcoma, which is known to be very resistant to treatment.
Literally everyday scientists, both non-clinician and clinician, are working round the clock towards finding new discoveries in the prevention and treatment of cancer, but I also believe you as an individual and us as a nation can work towards the same. How do we do this? By living an anticancer lifestyle. Exercise more and eat well. Learn how to recognise stress and eliminate it. Learn how to relax and enjoy yourself without bad vices or overindulging. Even though the past few weeks have been freezing cold, on the whole we have good whether which would allow us to take up more outdoor activities and active lifestyles.
I leave you today with one of my favourite mantras ‘Be the change you want to see’.