# Soft Tissue Sarcoma Rapid Fire: Update Course 2015 — GCMD Library Dr. Andrea Hayes Jordan discusses soft tissue sarcomas. Her presentation covers ovarian masses, ovarian germ cell tumors and treatment, bilateral ovarian disease, ovarian preservation technique, IRS clinical grouping of soft tissue sarcomas, and rhabdomyosarcoma vs non-rhabdomyosarcomas. Type: video · 8 min · posted 2019-01-11 Canonical: https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980 ## Chapters - [0:00](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=0) Rhabdomyosarcoma Staging and Grouping - [2:06](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=126) Rhabdomyosarcoma Case Examples and Sentinel Node Biopsy - [4:31](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=271) Non-Rhabdomyosarcoma Soft Tissue Sarcomas - [6:54](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=414) Summary and Q&A ## Statements - "Half of soft tissue sarcomas are rhabdomyosarcomas and half are non-rhabdos" — Andrea (epidemiological) [0:00](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=0) - "Prognosis in rhabdomyosarcoma varies depending on site" — Andrea (clinical) [0:25](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=25) - "Abdominal, pelvic, and retroperitoneal rhabdomyosarcomas have the worst survival" — Andrea (clinical) [0:25](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=25) - "Orbital rhabdomyosarcomas have the best survival" — Andrea (clinical) [0:25](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=25) - "Favorable sites (vaginal, paratesticular rhabdos) are stage 1" — Andrea (clinical) [0:50](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=50) - "Even small tumors in the extremity or abdomen can only achieve stage 2 at best" — Andrea (clinical) [1:02](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=62) - "Surgical extent determines clinical group: group 1 if everything removed, group 2 if microscopic residual, group 3 if only biopsy performed" — Andrea (clinical) [1:14](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=74) - "Group 1 and 2 patients have an excellent prognosis while group 3 patients do more poorly" — Andrea (clinical) [1:30](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=90) - "Group assignment is a biologic determination, not a measure of surgical skill" — Andrea (opinion) [1:45](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=105) - "Attempting to resect a large unresectable tumor with high morbidity will not improve patient outcome" — Andrea (clinical) [1:52](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=112) - "5 centimeters is the cutoff for low versus high-risk patients in soft tissue sarcoma" — Andrea (clinical) [2:09](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=129) - "Sentinel lymph node biopsies are now required for all trunk and extremity rhabdomyosarcomas" — Andrea (guideline) [3:18](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=198) - "In rhabdomyosarcoma, completion node dissection is not performed after positive sentinel nodes; radiation therapy is given instead" — Andrea (guideline) [3:34](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=214) - "40 to 50% of biopsy nodes are positive in rhabdomyosarcoma" — Andrea (epidemiological) [4:39](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=279) - "Clinically negative nodes may be positive on sentinel node biopsy" — Andrea (clinical) [4:39](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=279) - "Positive lymph nodes have worse prognosis and require radiation therapy" — Andrea (clinical) [4:39](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=279) - "Histological grade is now critical in non-rhabdo soft tissue sarcomas" — Andrea (guideline) [4:54](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=294) - "Large tumors should be biopsied first and chemotherapy tried" — Andrea (guideline) [4:54](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=294) - "Sarcomas have been identified as chemotherapy-sensitive or chemotherapy-insensitive, which changes treatment" — Andrea (clinical) [5:13](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=313) - "Low-grade tumors require only resection and observation with no radiation or chemotherapy" — Andrea (guideline) [5:21](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=321) - "Low-grade tumors with positive margins receive adjuvant radiation therapy" — Andrea (guideline) [5:38](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=338) - "Unresectable tumors now receive both preoperative chemotherapy and radiation therapy (previously only chemotherapy was recommended)" — Andrea (guideline) [5:52](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=352) - "Small tumors less than 5 centimeters should be resected with attempt at negative margins" — Andrea (guideline) [6:10](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=370) - "Chemosensitive tumors include synovial and undifferentiated sarcomas" — Andrea (clinical) [6:10](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=370) - "Chemoinsensitive tumors include alveolar soft part, malignant epithelioid, and clear cell sarcomas" — Andrea (clinical) [6:28](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=388) - "All chemoinsensitive tumors are high-grade; none are low-grade" — Andrea (clinical) [6:28](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=388) - "Surgery and radiation are the primary treatments for chemoinsensitive tumors" — Andrea (guideline) [6:28](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=388) - "A 5 centimeter tumor in a child less than 3 years old is equivalent to a 3 centimeter tumor in bigger patients" — Andrea (clinical) [6:54](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=414) - "3 centimeters should be the cutoff for excision versus biopsy in toddlers rather than 5 centimeters" — Andrea (guideline) [6:54](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=414) - "Sentinel lymph node biopsy should be performed if lymph nodes are clinically negative by imaging" — Andrea (guideline) [7:15](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=435) - "Positive lymph nodes receive radiation to the lymph node basin without completion node dissection" — Andrea (guideline) [7:15](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=435) - "Low-grade non-rhabdo tumors with negative margins can be cured with surgery alone without chemotherapy" — Andrea (clinical) [7:25](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=445) - "Chemoinsensitive tumors require aggressive surgical extirpation" — Andrea (guideline) [7:37](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=457) - "Core biopsies are acceptable if 3 or 4 good non-necrotic cores are obtained" — Andrea (clinical) [8:10](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=490) - "Pathologist should check core biopsy samples before interventional radiologist finishes to ensure tissue is not all necrotic" — Andrea (guideline) [8:10](https://library.globalcastmd.com/watch/soft-tissue-sarcoma-rapid-fire-update-course-2015-980?t=490) ## Transcript OK, so these soft tissue sarcomas are quite rare and so we'll take this opportunity to give folks an update just on what's new as far as surgical therapies in these soft tissue sarcomas, and half of them are rhabdomyosarcomas, but the other half are the non-rhabdos, so we'll spend a couple of minutes on each one. So we're talking about a very, the very rare sarcomas, and the important thing to remember is that these and rhabdomyosarcoma. We'll talk about first, the prognosis varies depending on site and so the, the patients that we see the most often, of course, are the abdominal, pelvic, retroperineal ones, and those are the ones that have the worst survival over here under other and then the best survivors are the orbital r Rhabdomyosarcomas and because I know people don't deal with these every day, I'm gonna just very quickly go through how we stage them with uh favorable. Sites. So if you have a favorable site, this is basically vaginal rhabdos, persticular rhabdos. Those are stage 1 and stage 23, and 4 are unfavorable sites with various amounts of disease. But what I'd like you to remember is that, you know, we basically don't see a lot of stage 1 patients. So even if you have a small tumor in the extremity or the abdomen, the best that patient can do is a stage 2. A group, the grouping, it depends on the surgical extent. So you as a surgeon determine the clinical group. If you remove everything, it's a group 1. If you leave microscopic residual behind, it's a group two, and if you can't get it out and you just do a biopsy, it's a group 3. So the stage and the group combined are what determines how the patient's outcome is and as you can see in this Kaplan-Myer curve, the group 1 and 2 patients have an excellent prognosis, and the group 3 patients are the ones that do a little bit more poorly. I do want to emphasize here that this doesn't mean. That the, the good surgeons, quote unquote, they're ones that can get it down to group 2. This is basically a biologic determination. If there's a large tumor that a surgeon can't resect, it's gonna be a group 3, and you trying to resect that with a lot of morbidity is not going to make that patient's outcome any better. So first case, this is a 5 year old with a mass on the forearm. It's 4 centimeters in size, and as we know, 5 centimeters is the cutoff for sort of a low or a high-risk patient. So it's a 4 centimeter tumor in a 5 year old, uh, we're, we're gonna talk about this later, it's not for you to answer out loud, but just to think in your head whether you would resect it. This particular patient had their 4 centimeter tumor resected in an outside hospital. It turned out to be alveolar rhabdo. The margins were positive, uh, and of course the forearm of a 5 year old is gonna be quite small. Uh, the, on the CT scan, the axillary nodes were clinically negative, so they came to us and we did a re-incision and a sentinel lymph node biopsy, which none of the nodes were positive, and so they did not need radiation therapy to the axilla. So the, the, this is the same case and sort of the way we should be approaching it. And if you have a 5 year old with a 4 centimeter mass, even though it's less than 5 centimeters, we all know as pediatric surgeons that that's a huge mass in the forearm of a 5 year old. And to make an attempt to try to resect that is probably not gonna be worthwhile, and you should probably just do a biopsy and see what you have. But in addition, as far as updates are concerned, the one thing I want to emphasize with Rhabdo on this update is that now we are requiring sentinel lymph node biopsies for all of these patients. So if you have a patient with the trunk or extremities, rhabdomyosarcoma, you have to do a sentinel lymph node biopsy. Um, and once you do the sentinel lymph node biopsy, the, the difference in rhabdo as far as the other diseases that we're used to, such as melanoma, is that you do not do a completion node dissection, you just get radiation therapy. So back to this patient, so we do the sentinel lymph node biopsy, none of the nodes are positive, so no radiation therapy. Now, the, if since it's alveolar rhabdo, they will get chemo, they, they should get chemotherapy after you did the biopsy, and now this is, this is usually gonna be down to a 1 centimeter mass, it's very easy to resect with negative margins. The next case is a 2 year old where has an excisional biopsy, uh, again, the margins were positive, the surgeon knew, didn't know what it was and sort of knew that they would be positive and went back to do a re-excision, and that's the time of re-excision that you need to do your sentinel lymph node mapping. And in this case, the, the sentinel lymph nodes were positive and that patient received axillary radiation therapy and chemotherapy. So the lymphatic mapping is something I know we've all learned, but as far as Rhabdo, it's been one of those things that's been optional and now it's being required. So, 40 to 50% of the biopsy nodes are positive, the clinically negative nodes may also be positive, so that's why we're doing the sentinel node biopsy, and we know that positive nodes have a worse prognosis and require radiation therapy. So we're gonna switch to non-rhabdose soft tissue sarcoma, so the, the changes in non-rhabdos, and what I want you to remember is the histological grade is now critical. We know that large tumors, you should biopsy first and try chemotherapy, but the histologic grade is what you should be paying attention to and not just the diagnosis. And, and the other difference is that we've identified sarcomas that are chemotherapy, insensitive and sensitive, and that changes the treatment. So, I'm just gonna briefly want you to focus on this tumor grade part of this slide, which is the current recommended treatment for soft tissue sarcomas. If you have a low-grade tumor, all you're gonna do is resect that patient and they're only gonna get observed. There's no need for radiation or chemotherapy. If you resect a low-grade tumor and you have positive margins, they will get adjuvant radiation therapy. Uh, so this is something. That um we've shown, and I'm not gonna share all the data because of time reasons, but that is now the new recommended treatment. The other thing I want to point out here is if you have an unresectable tumor that's huge, that you want to treat that patient with preoperative radiation therapy, uh, and preoperative chemotherapy, but first, uh, we were first only recommending preoperative chemo, now we're recommending both chemo and radiation. So small tumors less than 5 centimeters, uh, go ahead and resect those, try to get a negative margin, and to know that the chemosensitivity tumor, the chemo sensitive tumors are the ones that you, if you can't resect, you'd be enthusiastic about giving chemotherapy first, that's the synovial and undifferentiated. The chemo insensitive, uh, tumors, sorry, that's a type I should say insensitive, are the alveolar soft part, the malignant epithelioid clear cell, anything else that's sort of other. Those are the ones that you as a surgeon, the radiation therapist really will be the primary treater of those uh disorders, and all of those are high grade, none of those are low-grade tumors. And then of course the high risk patients to get the uh preoperative radiation and chemotherapy. So when endowed biopsy, remember that the uh the Italians did a very nice study showing that a 5 centimeter tumor in a small child less than 3 years old is about the same as a 3 centimeter tumor in a in a in a. Bigger patients, so you really wanna think about more 3 centimeters as your cutoff for excision as opposed to 5 centimeters if you're dealing with a toddler. Always do a sentinel lymph node biopsy if clinically the lymph nodes are negative by imaging, and remember that they're gonna radiate the lymph node base and you do not have to do a completion node dissection. Find out what the grade of the tumor is if you're resecting a non-rhabdo tumor because those are the ones you can cure with surgery alone, uh, and if you have negative margins, those patients don't need chemotherapy. The chemo insensitive tumors are the ones that you wanna be very aggressive with surgically, uh, to try to extirpate, extirpate those as much as possible. Thank Perfect. And Andrea, thank you very much. And uh and, and there's a quick question. I think we're about a half an hour behind, so we're gonna move ahead. Does anyone have a quick comment or question? Core biopsy versus open experience. You hear that, Andrea? Oh, he asked about a core biopsy. Core biopsies are acceptable. Uh, you'll, you'll need to have your pathologist check the core before the interventional radiologist is finished to make sure that it's not all necrotic, but as long as you get 3 or 4 good cores that are not necrotic, those are acceptable. Very good. Any other? --- Not medical advice · citation policy: https://library.globalcastmd.com/ai