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Patient FAQs for Sarcoma and Other Rare Cancer

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I'm a pathologist with interest in both skin and bone/soft tissue sarcoma pathology. I have a particular interest in sarcomas and a wide variety of rare tumors and cancers of the skin and soft tissue. I volunteer in many patient support groups on Facebook (List of Facebook Patient Support Groups for Sarcoma and Other Rare Cancer). My role in those groups is as a patient advocate who has expertise in the diagnosis of sarcoma and other rare skin and soft tissue tumors. As a pathologist, I diagnose patients and work closely with treating physicians such as surgeons and oncologists, but I do not treat patients personally. Some people ask me why I spend my free time working with patient support groups: This is my form of medical volunteering. I donate my time to these groups and try to give general information to patients about their rare tumors and attempt to help them better navigate the confusing medical system of cancer care. In exchange, the patients teach me SO much about their disease, including their symptoms, side effects of treatments, and their personal experiences in the course of diagnosis and treatment. I think this kind of information helps make me a better and more empathetic doctor. It also gives me a strong desire to work harder on researching these diseases, teaching other physicians about them, raising public awareness, and being an overall champion of these "orphaned" types of sarcoma and rare cancers. Although I can't give official medical advice here or in Facebook groups (since you are not my own patient and I am not your doctor), I do my best to answer general questions and provide education about these tumors so that patients have better understanding of their type of disease and the associated medical terminology so they can communicate better with their own physicians about their medical care. DISCLAIMER: This is an actively-growing compilation of my answers and comments to frequently asked questions on these topics. This is not medical advice: I am not your doctor and you are not my patient. These are just general comments that represent my personal views and experiences on these topics. I may be wrong about some of these things. Some of my views may (and hopefully will) change over time. These comments are for educational purposes only and should NEVER be used in place of your own doctor's advice. If you have any medical concerns, please talk to your doctor. Jerad M Gardner, MD

Special thanks to my medical student Julie M Youngs for organizing and editing this page for me.

General Comments:

Q: My doctor found a lung nodule on my most recent scan. Does this mean my cancer is spreading?

A: Small lung nodules (and nodules in other places) are very common and a constant source of frustration for cancer doctors and patients alike. We look closer at people who have cancer and do scans. When we scan, we often find incidental tumors, lung granulomas (a reactive nodule occurring when the body fights off bits of fungus we inhale from the environment), etc. that were there all along and are not dangerous. But it can be near impossible to tell them apart from disease spread (cancer metastasis) early on. So we watch and wait to see if they grow or we biopsy them if we can.

Q: I found a bump on my leg a few months ago and now it is getting bigger. I'm worried I have a sarcoma!

A: Although lumps and bumps can definitely be sarcoma or other cancers, a benign diagnosis is far more likely. You absolutely need to see your doctor about it so they can perform a biopsy. Sarcomas and other cancers often mimic benign cysts or lipomas. And as sarcomas are much, much more rare, many times they are dismissed as merely a cyst or lipoma for some time. The only way to be certain of the diagnosis is to have a pathologist examine the tissue microscopically. But in the meantime, take deep breaths and don't get too upset before you even know what the diagnosis is.

Sometimes patients are worried that a delay in getting the mass removed will result in the tumor spreading or acting more aggressively should it turn out to be a sarcoma. No one knows for sure, but in my personal opinion, I think a few weeks waiting for the doctor is unlikely to make any major difference for sarcoma, or most types of cancer for that matter. I don't say this to encourage patients to delay seeking medical attention, but rather do that those who are waiting to be seen by a doctor will not feel more anxiety than they already do. Faster diagnosis and treatment is always ideal, so if you have concern about any lump, bump, mole, or other skin spot, schedule an appointment with your doctor (ideally, a dermatologist) right away.


Q: What do "primary" and "secondary" angiosarcoma mean?

A: The terminology is confusing. Primary angiosarcoma means the tumor is "de novo" ("from the beginning"). It just randomly occurred and we don't know what caused it. Secondary angiosarcoma means the tumor was caused by something, usually radiation therapy for breast cancer or some other type of cancer in the past. Both primary and secondary AS often do present in or near the skin or underlying soft tissue. When angiosarcoma occurs in an internal organ like the heart of liver it is usually primary angiosarcoma, because these sites are not as exposed to radiation during radiation therapy. Both primary and secondary angiosarcoma on the skin surface can have similar appearances: bruise like patches, thick red/purple plaques, ulcerated nodules, or a combination of these. To my knowledge, they do not have distinct appearance from one another clinically. Biologically, however, there seem to be significant differences in the genetics involved in primary vs. secondary angiosarcoma. When either type of angiosarcoma spreads to a different place in your body, that is called metastasis (if it spreads to a distant location, it may also be referred to as "stage IV" disease...pronounced "stage four").

Dermatofibrosarcoma Protuberans (DFSP)

Q: What is the risk of metastasis in fibrosarcomatous DFSP (FS-DFSP) vs conventional DFSP?

A: Long story short: it's controversial. Some literature says there is no significant risk of metastasis beyond that of typical DFSP provided the tumor is excised with free wide margins. Other literature suggests the rate of metastasis is somewhat higher (around 15%). That is still relatively low compared to most other cancers, but a lot more than the 2-5% risk of mets that is quoted for typical DFSP in the literature. My belief is that both of those statistics are likely much higher than reality. I've seen probably 75+ cases of DFSP in my career so far, and I've only seen 1 case with mets that I know of. On the largest of the DFSP support groups on Facebook, there are over 1000 members with DFSP (as of July 2016), and many of them have FS-DFSP, yet there are only a few cases of alleged metastases in that entire group. As I have not seen the pathology of those cases myself, I cannot be certain about the accuracy of those diagnoses.

Q: What is the treatment for DFSP?

A: Usually DFSP is treated with surgery only, although in large or difficult cases radiation or some special chemotherapy drugs (like gleevec) may be used. DFSP can sometimes grow back after surgery, but the good news is that it very rarely spreads to other parts of the body and therefore survival is close to 100%. So it is not like other cancers. Make sure you are seeing doctors who have some experience with treating DFSP, also you might want to have your pathology reviewed by a soft tissue sarcoma pathology expert to be sure the diagnosis is correct before proceeding with any treatment. You can ask your doctor about this. He/she should have an idea of whether your pathologist has experience with DFSP or not.

Q: Does DFSP metastasize to lymph nodes?

A: DFSP, like most sarcomas, does not typically spread to lymph nodes. Of course there are exceptions to pretty much every rule in medicine, but I've never personally seen DFSP spread to lymph nodes. I've seen maybe 75+ cases of DFSP so far in my training and career as a sarcoma pathologist, so I have more experience with this tumor than many doctors do. I think the main issue with the enlarged "lymph node" discovered on physical exam (a lump that can be felt) is to be sure that it truly is a lymph node and not a nodule of tumor or something else. This is also an issue but to a lesser degree on imaging studies like CT or MRI; most radiologists are pretty good at telling lymph nodes from other things. Bottom line is I would not get too worried to hear about an enlarged lymph node discovered on a scan if I were a DFSP patient. Certainly discuss with your doctor. It it were me as the patient, I would likely prefer to watch it closely but would also be open to the idea of biopsying it if either I or my treating doctors felt worry. Sometimes a negative biopsy result is the best cure for persistent patient anxiety.

Q: Can I have DFSP in multiple places?

A: it is very very rare to get DFSP in two different sites (though exceptions exist). Anytime someone is diagnosed with cancer, the patient and their docs look at the patient much more carefully and start noticing every little spot. That's a good thing, don't get me wrong. But it also means that most if not all of these extra spots will be unrelated to your DFSP. BUT as you all well know, sometimes little bumps end up being cancer. So you very well may find other forms of true skin cancer or a variety of other benign skin growths if/when you have these biopsied. I might suggest that you go see a dermatologist as well as your surgeon. A dermatologist may help at figuring out which of your bumps need to be biopsied.

Q: Will exposure to air during surgery cause my tumor to spread?

A: This is a common concern about cancer of all types. I am not aware of any data to suggest that exposure to air causes an increased risk of tumor spread or progression. I personally believe that surgery (even incomplete) is not likely to promote deeper invasion or dissemination of DFSP in the majority of typical cases. Surely there may be some exceptions, but the take home message is that in general the benefit of surgical removal of a cancer (including DFSP) outweighs the risks. I don't want DFSP patients fearing that biopsy or surgery (wide local or Mohs) will make their DFSP worse. Obviously, it is best to have the proper type of biopsy or surgery performed up front from a doctor with experience in treating DFSP. I think that one of the biggest issues with DFSP is lack of early detection and lack of awareness of this disease among most medical providers. 

Q: Will DFSP show up on a PET scan?

A: Despite it's widespread use, PET use is not always agreed upon even for cancers that usually show up on PET. PET is best at detecting cells that are growing quickly and dividing quickly (these have a high metabolism and use up lots of sugar). Most of the time DFSP grows and divides relatively slowly compared to many other cancers like breast, or colon, or lung cancer. I think its use for DFSP is probably pretty limited. Again, I'm not a radiologist, but this is my personal opinion. And I am not saying anyone's doctor is wrong for doing any of these scans. This is a rare tumor without a widely accepted, standardized approach to follow- up, so in these scenarios each doctor uses their medical reasoning and a variety of other factors to decide what is best to do for the patient. Medicine really is both an art (as in a subjective specialized skill, not as in a Monet or DaVinci painting) and a science. That is frustrating sometimes both for patients and for us as doctors.

Q: Will I need an amputation for DFSP?

A: I would get a 3rd or even 4th opinion before having one of my limbs amputated for DFSP if I were a patient. I have never personally seen a case that required amputation. I'm sure it might happen for very advanced cases, but it would be very unusual. DFSP is unusual in that even for as invasive as it is, it usually does not extend into the underlying muscle very much. It tends to stick to the skin and underlying fat.

Q: Why is DFSP often confused with a cyst?

A: It is a frustrating problem. And I do work hard to train my students and residents to biopsy things more and to never blow people off if they have a lump or mass. The problem is that for every patient who comes in and has a "cyst" that turns out to be a DFSP, there will be 10,000 other patients who come in for a cyst that when biopsied turns out to be just a cyst! You all are likely the only DFSP patient your PCP/GP will EVER see in their whole career! I see sarcomas daily and sometimes several sarcomas in a single day, but I only see 5-10 cases of DFSP per year. And sarcomas are my specialty!

What I've learned in dermpath and sarcoma path... EVERYTHING is clinically a "cyst" (until it turns out not to be). I have a box of cases that were clinically cyst but histologically all sorts of weird tumors or other things. One day I'll make a slide show of "it's not a cyst." In our survey study of over 200 DFSP patients, the majority of patients were misdiagnosed clinically as a cyst, often delaying treatment for several years.

Q: What is the follow-up regimen for DFSP?

A: I'm often asked this question, and I don't know the answer, honestly. I hope our research studies will shed light on this. DFSP recurrences can be subtle and too small to see well on MRI with all of the scar tissue around.

If I were the patient, I think I would want these things. This is just my unofficial opinion about myself as a hypothetical patient. This is not medical advice. I don't want you to go and refuse to hear what your own doctor says just because "well Jerad said this..."  Ok? :-)

1. Regular self skin checks and visits to the dermatologist or the surgical oncologist if I found any new bumps or changes. A new bump that worries me = get a biopsy (in my book, but hey, I'm a pathologist!)

2. See dermatologist, surgical oncologist, or other treating doctors on a regular interval per their recommendation (usually every 3 or 6 months at first, eventually becoming yearly is the typical scenario for most sarcomas

3. I'm personally not sure that regular MRI or CT gives a huge amount of benefit, because a small subtle recurrence will be hard to detect and a big one will be easy enough to see just by examining the skin. But I would consider having MRI on intervals if my doctor wanted them

4. Many of you have asked about regular chest X-ray or CT. For high grade sarcomas that often metastasize, it is common to do a chest CT at the time of diagnosis during work up and then to repeat this at intervals to check for metastasis. Although DFSP can metastasize, this seems to be very rare. I've seen it only once.  So is it worth getting all those CT scans for such a low chance? I don't know. That is a question of risk vs. benefit that you will have to carefully discuss with your doctor. If it were me, I might do it once at diagnosis but I would personally probably be okay with not repeating it regularly. Some patients may need the reassurance of a negative scan to feel okay. I am pretty familiar with DFSP and think about it every day pretty much, so I am probably not the typical new DFSP patient! It's also hard for someone who doesn't have cancer to know what they will do or feel or think if they ever get cancer. The emotional aspect is unpredictable.

I hope these thoughts help you in your discussions with your own doctors.

Desmoid Tumor

Q: How do I explain my tumor to others?

A: You all have a tough situation to explain to laypeople (and most doctors for that matter). I think it is fair to tell people "I have a rare soft tissue cancer. It never spreads to other areas of the body like most other cancers, but because it invades everything around it, it often grows back repeatedly requiring multiple major surgeries. I probably won't die from it, but it has caused many serious side effects that will affect me for the rest of my life." I admit that in my view, desmoid is not technically cancer, but what exactly defines cancer for rare, weird tumors like desmoid is a matter of some debate even among cancer experts.

Epithelioid Hemangioendothelioma

Epithelioid Sarcoma

Fibromatosis (Desmoid Tumor)

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