跟讀練習: Accurately Diagnosing the Soft Tissue Sarcoma - 通過影片學習英語口語

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You know, as our lead surgeon and one of the lead surgeons in the world,
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I think it's always, you know,
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very important that our surgical colleagues are dramatically involved in the treatment of sarcoma
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because usually you're the gatekeeper in the entrance of a lot of this.
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And so, you know, I think it would be good to ask you what your typical workup is for a patient
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is suspected of having a sarcoma.
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Sure, thanks, Brian.
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You know, it's generally a patient will present to a primary care doctor
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or an orthopedic surgeon or a general surgeon with a mass that is growing,
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something that is changing, or maybe abdominal distension and discomfort.
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But like we all learn in medical school,
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a good history in physical is the first thing we do.
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If this mass has been there for a long time,
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it's less likely to be a malignant process or an aggressive malignant process.
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If it's rapidly changing, the acuity is obviously there and something needs to be done about it.
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But it's very important to stress the history and physical.
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I always joke with my trainees that the most important test we order is the history and physical.
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With that being said, we generally do recommend plain film imaging,
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radiographs, orthogonal views in the extremity particularly,
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because that can shed a lot of information in the area of cost containment.
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If it's completely radiolucent in an extremity film,
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it is very likely to be lipomatous,
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well-differentiated liposarcoma at the worst,
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versus if it has some calcifications on it,
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the acuity is probably there and that it's a synovial sarcoma or potentially a higher grade liposarcoma,
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things of that sort.
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So radiographs are important and they're inexpensive.
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Beyond that, we do think that advanced imaging is important before any biopsy is done.
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We generally recommend an MRI in the extremity,
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sometimes in the pelvis, but often a CT is adequate.
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And if we really suspect that it's a sarcoma,
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we prefer getting a chest CT first before any intervention is done.
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But if you're seeing the patient in the clinic
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and you're going to do a biopsy in the clinic because it's amenable to that,
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you can hold off on some of that advanced imaging.
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But we certainly recommend getting advanced imaging of the primary before any intervention is done.
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As we all will talk about,
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the most common site for these metastases is the chest,
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and so we do think chest imaging is of paramount importance.
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No, I agree, and I think it's always important to remember that,
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you know, we hear lots of interesting things and you know,
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car doors do not cause hematomas unless you're on blood thinners.
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And so, you know, it's very important to always do that history and physical,
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because these masses sometimes just get found by car doors.
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Otherwise, you know, surgeons go and explore in the middle of a sarcoma,
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and if you don't do these right,
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I mean, you can get inferior outcomes.
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And so, I mean, as we're moving through from the history and physical to imaging,
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I think the next most important thing is really an understanding of diagnosis
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and grading of these tumors and really what that means.
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And I think maybe Dr. Jones can give us a little insight about how they approach that.
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Thank you.
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So as Dr. Patel mentioned,
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these are heterogeneous and a complex group of diseases.
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So it's very important to obtain a clear and accurate histological diagnosis prior to starting treatment.
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Each histological subtype can have different clinical behavior due to different underlying biology and can also have a different treatment approach.
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So it's important to have an experienced pathologist make the diagnosis prior to starting treatment.
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The World Health Organization classification system is generally regarded as the standardized publication for classifying these tumors.
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and it's very important that newer techniques such as molecular techniques are incorporated into the diagnosis as well.
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In terms of grading, the most common grading system that's used is the French,
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so-called French grading system, incorporating three grades,
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high, intermediate and low, based on tumor differentiation, necrosis and mitotic count.
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and the grading system has implications in terms of prognosis.
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I think that's really, really important.
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And, you know, in the year 2015 with our ability to sequence everybody
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and our ability to look at all sorts of molecular markers,
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you know, I think we're into a very complex world and a very rare group of tumors.
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And so, you know, Jonathan,
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what is your goals of molecular testing and how do you incorporate that into your practice?
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Good question.
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And thank you for the invitation to participate in the panel.
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And I agree with, certainly with Shreyas and Robin,
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regarding the complexity of sarcomas.
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In fact, there's over 200 different types,
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if you look at the pathology textbooks.
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And I think that molecular testing is underutilized in the world of sarcoma and that it can help in diagnosis.
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For instance, there are a number of histologies
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that have specific genetic abnormalities such as gene amplification and MDM2 and some of the liposarcomas,
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de-differentiated and well-differentiated.
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And this testing can not only help make the diagnosis,
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but there could also be therapeutic relevance.
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For instance, in the era of targeted therapies,
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we are now able to pair specific molecular events such as
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a mutation in a gene called KIT in GI stromal tumor with a therapy such as imatinib
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or sunitinib or regorafenib and be able to really precisely treat patients based on the molecular rearrangements or abnormalities.
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And this is particularly pertinent to sarcoma since we have
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so many histologic subtypes that have a very high frequency of these molecular abnormalities.
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So, Brian, if I could add a few comments with this too.
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I think has been well stated.
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It's a huge number of different tumors.
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And just like you would never classify carcinomas as one disease,
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you wouldn't lump breast carcinoma and prostate carcinoma into the same thing.
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We do that in sarcomas where we have these different diseases.
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We lump together sarcomas because they're so rare and they can have similar presentations but markedly different genetics and different behavior.
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And one of the key parts is to make sure,
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as Robin was saying, that an expert in sarcoma pathologies reviewing the material,
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especially
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when it's obtained at a community hospital where pathologists may only
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see a few cases of these in the course of their career.
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The hierarchical testing that they do is based not just on the specimen they have,
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but also based on the presentation of the patient.
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So it's also very important that the medical
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and surgical teams are in communication with the pathologist about the location of the tumor,
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the behavior of the tumor, and those other aspects.
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So a multidisciplinary approach is really important.
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And their testing often looks at the shape of the cells first and where it is located,
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and then that helps drive other testing like immunistic chemistry and other molecular testing.
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I think we're still very early in the question of the wide-scale genomic testing that's performed either for research
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or for clinical commercial purposes right now.
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In our experience, and I think a lot of the other centers are doing this as well,
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where we've sequenced large panels of genes,
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we've seen a disappointingly few number of mutations that we didn't already expect in sarcomas.
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I think it's still a very important area for research,
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but personally I'm not yet ready to order huge screening panels for genetic mutations,
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but instead stay focused, as John was just talking about based on the appearance of the tumor,
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suspected appearance of the tumor,
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and the possible mutation types.
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Dr. Yeah, I'd like to,
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if I may, an illustrative example is in the pediatric realm of a very practical fusion product,
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you know, the fusion-associated tumors,
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rhabdomyosarcoma, where historically it's been either in pediatric realm, conventionally alveolar versus embrinal.
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And now it's actually fusion-driven,
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and that's the gold standard.
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And if there's some Pax-Foxo associated tumor that may have some embryonal features,
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they're still going to call it alveolar.
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And it's really that and the clinical trials moving forward through the Children's Inchology Group,
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et cetera, are all doing this.
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And I think Synovial and Ewings are following suit with that.
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So it's really boots on the ground.
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This is happening today, not just some academic ivory tower concept.
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And I'd like to just highlight the importance of a multidisciplinary team in making the diagnosis.
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Andy said, very often it's important to have the clinical features
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and the radiological features together with the pathology to make the diagnosis.
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And that's really, really important to highlight.
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I think it's important to highlight the fact that the one thing that these are all mistaken for are melanomas,
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and melanomas are, you know, they're mistaken for sarcomas.
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And if you really make that error,
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you've altered radically a treatment path for a patient.
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And this is why having a highly experienced sarcoma pathologist confirming what they're seeing is really that important.
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And what's very interesting is it's very hard,
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I believe, to have the discussion we're having today
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because we're talking about at least 80 different diseases that probably break up in six different categories of each one.
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And we go from probably one of the best understood tumors on the planet,
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which is the GI stromal tumor,
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where we understand what mutations go with what drug,
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to the undifferentiated pleomorphic sarcoma,
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where we're still doing lots of clinical trials,
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which we'll get into later.

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