We are all aware of the fact that most lumps under the skin are benign. Usually they are fatty lumps called lipomas. Small lumps can be sebaceous cysts, and there are many other types, mostly harmless. So, what does it matter if an occasional malignant one slips through and takes a while to be diagnosed?
Well, it actually matters a lot; in fact, it’s probably a matter of life or death for the patient with a sarcoma. The fact is that for a sarcoma, the bigger the lump, the worse the outlook. The most aggressive types carry some risk of spreading to other parts of the body, even when they’re less than 5 cm (2") in size. This means that every delay waiting for a test, falsely reassuring the patient that it is "just a haematoma", "lymph gland", "lipoma" etc, means that it is simply getting larger all the while, becoming increasingly likely to spread to other parts of the body, e.g. the lungs, and hence, be incurable.
Where’s the proof that early diagnosis makes a difference?
Unfortunately, unlike with common cancers such as breast cancer, the evidence that removing sarcomas at an early stage improves outcome is somewhat hard to find, because it isn’t possible to do a clinical trial. However, every study that has ever looked at all the factors influencing outcome, has concluded that for each individual sarcoma subtype - and they do, of course, vary - tumor size and grade (the degree of aggressiveness) are the most important. Therefore, it seems inconceivable that earlier diagnosis and removal when the disease is smaller would not improve the outlook.
What harm is done if I take it out myself?
Sadly, one of the most common ways in which a doctor hears about the diagnosis of sarcoma is when the pathologist rings up to say that the "benign" lump he/she took out under local anaesthetic is actually a sarcoma, and it hasn’t been completely removed. In fact, if you take out a sarcoma and you can actually see it as you go along, then you’ve left some behind. They appear to "shell out" very easily but the so-called "pseudocapsule" is just that, a false capsule or margin; tumour cells extend beyond it. To remove a sarcoma properly, you have to plan the operation based on appropriate imaging and take it out with a good margin of normal tissue all around it. A poor initial operation contaminates the tissues and will sometimes put a scar in the wrong place, which compromises a definitive operation, and makes it more likely that the disease will come back locally.
Among the many horror stories I could relate are the following glaring examples: the first was a patient with sarcoma who was initially treated with antibiotics and then attacked with a scalpel in the mistaken belief that the lump was an abscess – it wasn’t, indeed it was rock hard; the second was a patient with a bleeding tendency who was repeatedly told that the enlarging lump in his thigh was a blood clot. By the time the diagnosis was made, the tumour had spread to the lungs. Clearly, in such cases, inappropriate intervention and delay in diagnosis proved very damaging. In the majority of cases where the tumour is small, a second operation can be performed to remove residual tumour cells without this having a major impact on survival. However, where the second operation reveals residual tumour, there is an increased risk of distant disease spread.
So how can I know if a lump is worrying without taking it out?
There are some very simply rules. These are the characteristics of a subcutaneous mass that should alert you. Remember that sarcomas can occur anywhere, but the most common site is the leg. A lump that has 2 or more of the following characteristics is potentially malignant and should be treated as such:
- Deep within the tissues (technically, deep to deep fascia, which means you can’t easily move it around)
- 5 cm or more in diameter
- Is growing
Some doctors would add "especially if it is painful," but this isn’t a very reliable sign, and lack of pain is not necessarily reassuring. So, if you find a lump with any of these characteristics, it should be imaged, (e.g. Magnetic Resonance Imaging, MRI scan) and you should send the patient/have yourself referred to a team that is experienced in the management of sarcomas.
Why refer to a multidisciplinary team in a cancer centre?
To diagnose and treat a sarcoma properly, you need to have specialists with the necessary experience in diagnostic radiology, histopathology, surgery, radiation oncology and medical oncology. A scan (usually MRI) is required to identify the size and extent of the disease. A biopsy needs to be done appropriately, and an experienced pathologist is then required to make the diagnosis - these are rare diseases. Surgery needs to be done in a planned way that will ensure complete removal of the tumour, hopefully preserving normal function. Depending on the size, grade, and completeness of resection, radiotherapy may be required. Sometimes this is employed pre-operatively. For some diseases, and in some clinical situations, it is appropriate to use chemotherapy either before or after the operation (or both).
Sarcomas cannot be treated appropriately in the community. If there is doubt as to the nature of a lump, it should be referred to a specialist centre. Follow the simple rules: firm, deep lumps that are growing are rarely benign. Early diagnosis is the key to improving outcomes for patients with sarcoma.
Suzie Siegel, who wrote an article for ESUN, Options and Follow-up Care for Women with Uterine Sarcoma, sent the following note to Ian Judson in response to the above editorial.
"I wish Dr. Judson would comment on gynecologic sarcomas. In the United States, women often see a gynecologist or gynecologic oncologist. The latter often thinks he is the specialist that a woman with sarcoma should see. If he refers her for a second opinion, he may send her to another gyn oncologist, not to a sarcoma center. Women with gyn sarcomas fall through the cracks of the medical system. Very few gyn oncologists study gyn sarcomas. Meanwhile, doctors in sarcoma centers include us in clinical trials and research but rarely, if ever, mention our situation."
Dr. Judson replied:
You raise a very important point. Until a few years ago we saw very few gynaecological sarcomas on my unit but now they represent a significant part of our work. They are frequently only diagnosed after hysterectomy and there is no doubt that the fact that they are so often mistaken for benign fibroids is the main reason for their late diagnosis. There remains a great deal of ignorance about their appropriate management in terms of adjuvant radiotherapy, subsequent surveillance and the potential adverse effect of hormone replacement therapy in a proportion of cases. I agree that patients with gynaecological sarcomas deserve to be managed by sarcoma multidisciplinary teams just as much as any other type of sarcoma, since the same principles apply, i.e. patients with rare tumours benefit from being treated by an experienced team. Thank you for raising awareness of this issue.
With best wishes,