As time has progressed and there has been a greater understanding of the pathophysiology of lymphedema, it has become clear that lymphedema in and of itself can set the stage for the development of secondary cancers.
There are two broad groups of these cancers. First, lymphatic cancers and secondly skin cancers.
This article is based on a discussion from our forums and from research articles obtained from sources such as Pub Med and other related medical journals.
My recent post on cutaneous lymphomas caused quite a flurry of emails of list with requests that I share a bit more about my experience and how you would recognize a malignancy secondary to lymphedema.
Before I share my own experience, I feel the need to lay a foundation to help clear things.
I need also to repeat that I am not a medical professional and only share from my own personal struggle with LE, the experiences of others and from the research that I have done. If there are medical professionals in the group that have additional information, we would all greatly appreciate you feeling free to join in this discussion.
I want to say also, that there are several factors that appear to contribute to one's susceptibility towards a malignancy with LE.
1) The length of time you have had the condition is a factor. The cases I have studied or known about involve patients who have had LE for a decade and more so decades.
2) The condition of the LE limb is also a factor. Remember, and this is important, that the size of a limb with lymphedema does NOT translate into the stage it is in. We so often see these horrendously huge legs and assume that this is lymphedema at its worse.
Reference Page: Stages of Lymphedema
The staging is determined not by size, but by tissue condition. Late stage lymphedema is when the limb has turned hard and fibrotic. It is at this very hard tissue stage the LE'ers become more susceptible to severe complications.
Reference Page: Complications of Lymphedema
It is also a stage where the lymphedema may even become untreatable.
Reference Page: Lymphedema Fibrosis
3 ) The treatment received on the limb seems to also contribute greatly to the possible development of the more serious complications, which include malignancies.
The cases I know of involve lymphedema that received incorrect or no treatment. Interestingly I also know of several LE'ers who had debulking surgeries who have subsequently come down with lymphoma. Whether there is a real tie in has yet to be determined by research, but that is an interesting point.
This is another reason why I sound like such a broken record about getting treatment promptly and in doing all you can do to prevent the LE from worsening.
This is an interesting discussion in itself. There are numerous reasons why lymphedema patients may be undiagnosed or misdiagnosed with a secondary cancer.
Not long ago I had a well known and respected LE doc tell me that they thought perhaps I didn't really have lymphoma. They said this out of the blue without any review of the biopsies or the history of my malignancy.
Their excuse was a simple one that is the cornerstone as to why these cases are not diagnosed.
1) Biopsies in an area of lymphedema can give false positives. This is because of the inflammation involved with lymphedema. This inflammation may appear as a cancer when it isn't. Fortunately, the initial surgeons involved in my biopsies understood this and we did double biopsies of the tumor with the specimens being reviewed by several pathologists, each one coming up with the same diagnoses. While biopsies can provide a false positive, the reverse is also true (I suspect more then is realized). They may simply dismiss a skin condition or a biopsy as simple inflammation.
2) Just as biopsies are difficult in lymphedema so too are the results that may be given by radiological exams. A PET for example will pick up inflammation as a glowing spot, not being able to distinguish between that and a real malignancy. This makes diagnosis by radiology a very difficult process. Cancers like lymphomas are notoriously hard to diagnose anyway. They often don't present visibly until the disease has progressed into the later stages. Often, there is no “warning” with lymphomas like there may be with other cancers.
3) The lack of knowledge in the medical community is another factor contributing to the poor diagnostic rate of secondary malignancies. Most doctors, if they know anything of this subject will immediately bring up lymphangiosarcoma (Stewart Treves Syndrome)
This dreaded cancer has long known to be associated with lymphedema. It is fast moving, usually fatal and presents with immediate dermal manifestations. So, it is easy see when it starts
What is just NOW begin recognized are the other cancers forms I mentioned in my earlier post. Kaposi's sarcoma, t-cell lymphoma and b-cell lymphoma. It is interesting to note too, that Kaposi's sarcoma used to be (and still is) thought of as a cancer of HIV/AIDS. From the research done there, they discovered that “whoa” this is something lymphedema patients get.
This is just a brief foundation…next I'll share a bit about my experience and how I finally was diagnosed.
Get serious about treatment…be compliant!!!!!!!!!!!!
Many of the emails ask me to share how I was diagnosed. To save carpal tunnel
This page gives a narrative of my experience up to the lung procedure I had done in January, I haven't updated it since then.
I really want to emphasis that this wasn't something to scare you, just to inform. I didn't realize the reaction that would occur to that post and msybe would have had second thoughts about posting it had I known.
Please, don't think that because you have a skin plaque, or a nodule, papillomatosis or an area of strange discoloration that it automatically means you have cancer. Most of these are common to long standing LE and are just benign. My diagnosis rested on in dept biopsies of specific tumors and a small needle of the right inguinal node.
They have biopsied these nodules (papillomatosis) - and of these all are benign. That ugly skin plaque we with late stage lymphedema has shown also to be benign.
The key is get treatment, get it early, be compliant and do all you can do to keep the LE from geting worse.
Sao Paulo Med J. 2010 Ja
Gomes CA, Magalhães CB, Soares Junior C, Peixoto Rde O.
Department of Surgery, Hospital Universitário, Universidade Federal de Juiz de Fora. Abstract
CONTEXT: Squamous cell carcinoma arising from chronic lymphedema has only been reported in the literature 11 times (12 cases). Some aspects of its pathogenesis remain unclear and, for the first time, attention has been drawn to epidemiological data.
CASE REPORT: A 90-year-old white female with chronic unilateral lower-limb lymphedema, secondary to trauma 20 years earlier, presented with a three-month history of a vegetating cutaneous lesion. There had not been any previous local ulceration. The tumor was completely excised and the histopathological analysis showed that it was an infiltrating squamous cell carcinoma. A literature review in the Medline (Medical Literature Analysis and Retrieval System Online) and Lilacs (Literatura Latino-Americana e do Caribe em Ciências da Saúde) databases using the MeSH (Medical Subject Heading) terms “Carcinoma, Squamous Cell” AND “Lymphedema” identified 112 references and found 12 similar case reports.
We recently had a discussion on this topic in yet another online lymphedema support group and one member wrote in saying “Am I Doomed?”
I want to share one response from a primary lymphedema patient Della:
I did not mean to frighten you. It frightens me to think about further complications from LE. I feel, though, that the best thing is to learn all that we can.
Aside from that and trying to take care of myself, as an old song says “I will live until I die”—enjoying life to the best of my ability and hoping I add something to the lives of others.
After all, my one grandmother lived to 102 and the other to 89 (neither withLE).. My dad, with LE, lived to age 85 and my mom is 81. My hope is that new treatments will be developed for the benefit of my three children who have LE.
My best to you