OK, in the interest of time, let's begin the next and final sessions. I want to introduce back up John Tooting, and he tells me he's got a humdinger of a talk for you. That's the perfect introduction. Thank you, Cody. So I'm glad you're all here. Buckle your seat belts. This is a lot of information, but it's very high yield information. I think what I've done for you here, what I've tried to do for you here, is really try to distill it down in an organized way. I expect your slides that you use, not so much in the paper form, but in the online form and on your iPads or computers, to be able to go through in an organized way and do a little bit of pattern recognition and memorize the histology, memorize the x-rays, memorize the patterns and the trends. And then again, I have provided things that are high yield for previous exam questions in yellow. So if you see something that's yellow, it's been seen on one of the self-assessment exam for the last six to eight years. So I have nothing to disclose. I'd like to thank Peter Murray, Warren Hammert, and then, of course, Dr. Lifshay and Dr. Azari and the Hand Society for inviting me to speak again. We're going to talk about history and physical examination, workup, biopsy, tumors of bone, and then tumors of soft tissue. And for each of those, we'll talk about malignant and benign. So what's important about the history? Recent painful or painless mass. Change in character of a pre-existing mass, meaning it's getting bigger or it becomes painful. Treatment delays for soft tissue sarcomas are common because people think that they're infections. People think that they might be a ganglion cyst or hematomas or other injuries that might be coincident with the presentation. Remember that big is bad. Greater than 3 to 5 centimeters, start paying attention. Ask yourself, is the mass fixed or is it mobile? Overlying skin changes or hypervascularity. It looks like there's recruitment of the vascular system. Compromise of nerves or vessels. Examination of regional lymph nodes. And then limitation of range of motion and adjacent joint. These are all things that should really get your attention when you see a patient. The workup includes a CBC and BMP, plain films, MRI with contrast, sometimes bone scan. Remember that bone scan is going to detect the osteoblastic response as opposed to the tumor itself. And remember that bone scans may miss early metastatic disease. Pet scans, in contrast, measure the metabolic activity of the tumor. Also get a chest x-ray, CT chest, abdomen, pelvis before proceeding, and then consider how you're going to do the biopsy. So chest CTs are useful for ruling out metastatic disease. Metastatic disease to the lungs is most common. Usually occurs within the first year. Lymph nodes are less common, and they are typically more likely to be seen with soft tissue sarcomas. Here's an example of a bone scan that's positive for a disease in the femur and the radius. Remember, a pet scan on the right side measures metabolic activity of the tumor, and it may pick up early metastatic disease. The definition of a biopsy is the technique of harvesting a representative sample of tissue without compromising definitive care. Fine needle aspiration is about 73% accurate. Core needle biopsy is 83% to 93% accurate. Open biopsy with frozen section is 80% accurate. It's difficult to assess fat and cartilage on frozen sections. So if you're thinking about a cartilaginous tumor, you have to start paying attention, or a fatty-related soft tissue sarcoma. And open biopsy in permanent section is 96%, so that's the best. Remember, open biopsy is the gold standard. Use a linear incision. And remember to plan to incorporate the incision that you're using into definitive limb salvage if you're doing this type of procedure or surgery. And if you do that, and you get burned because you thought it was benign and ends up becoming malignant, then you won't cause problems for the patient with their next surgery. So the open biopsy principles that are important to that end are make incisions that are in line with the eventual definitive surgery. Use longitudinal incisions. Stay within a single compartment or intramuscular. And typically, you want a biopsy from the peripheral portions of the lesion where there is the most metabolic activity and not the center where there's potentially necrosis. Remember that biopsy in a paper by Henry Mencken, which is a landmark paper, remember that biopsy problems are three to five times more likely at a referring rather than a treating institution. Biopsy should typically be performed by a surgeon capable of definitive treatment. So if you think you have something that's malignant and you don't do a lot of these types of procedures, you may want to refer to somebody who does. Because you want to be able to anticipate the future treatment during the biopsy. In a study by Ward and Kilpatrick in CORE with fine needle aspirates, 66 primary bone tumors, 73% were diagnostic, higher in sarcoma. The cost is much less than an open biopsy. So this is commonly tested material using the Anakin staging system. Remember this is for bone sarcomas. That's why the bone is underlined. And it's straightforward. You think stage one, A and B are both low grade. And A and B designate intra versus extra compartmental. Two is high grade. And again, makes sense. A and B are intra and extra compartmental. And three is any metastatic disease. Here's an example of how this works. So you remember this. You understand no METs for the ones and twos. METs for the threes. So if you have a low grade extra compartmental tumor without METs, it's stage two B. Low grade intra compartmental, so it seems like it might be bad. With METs, it's automatically a three. Now this is different. And this is where they like to test you because they like to get you confused between Anakin bone tumors and then AJCC soft tissue sarcomas. And the difference here is for the ones, they're both low grade. They're small versus large, superficial and deep. For the twos, between A and B, it's low versus high grade. So that's where the change is. So you can see that for A and B in the ones, they're both low grade. But once you get to the twos, A, B and C, you have low grade for the A's and then high grade for the B's and the C's. And then three is high grade and large and deep. And then four is metastatic disease. So you just have to memorize it. All right, so let's move on to benign tumors of bone. We'll first talk about enchondroma. And remember, like what I said, I've tried to basically give you enough to be able to do some pattern recognition. Some of these are easy and some of these are a little bit harder. So enchondromas are the most common benign bone tumor in the hand. We've probably all treated them. The most frequent locations are the proximal phalanx, the middle phalanx, and the distal aspect of the metacarpal. Here's an example of a lytic lesion with a pathologic fracture in the proximal phalanx of the ring finger. There's an example of removing the tumor. So what if you see multiple enchondromas? You have to think Olea's disease or Mufuchi syndrome. And the difference is that Mufuchi's syndrome also includes hemangiomas. And these present as calcific stippling on radiographs. So here's an example of somebody with this problem. Malignant transformation of solitary enchondromas is extremely rare. By contrast, the risk of malignant transformation is 25 to 30% in Olea's disease and even higher in Mufuchi syndrome. Typically age 10 to 40, fourth decade, and usually pathologic fractures and incidental findings. So again, when you see these contrasts, they want you to know that for somebody who has Olea's disease or Mufuchi's and it looks like an enchondroma, they have a much higher risk of malignant transformation than just a typical enchondroma. On x-ray, you see small, less than five millimeter lytic lesions with intramedullary calcifications or popcorn punctate or stippled calcifications. There's usually no cortical involvement, no periosteal reaction. You can see endosteal scalloping. So they might try to confuse you to make you think that, oh, I see a little bit of widening of the bone, I see a little bit of endosteal scalloping, but they'll show you something that's less than 2 3rds the cortical thickness and then you'll think this is probably not malignant, this is probably an enchondroma. Here's what this looks like. Usually plane radiographs are sufficient for the diagnosis and on histology, you see benign cartilage. It can be very cellular, but you do not see any mitotic activity in these histologic specimens. You see no binucleated cells and all the cells look pretty uniform with relatively normal appearing cartilage. But remember, chondrocycoma is the differential. The treatment of symptomatic lesions and enchondromas is intralesial curatage, wisher without bone graft or cement. You can almost pack these with anything. That's really dealer's choice. There is controversy in the literature that you can be aware of regarding the ideal treatment for an enchondroma with pathologic fracture. There's a supposedly higher complication rates report in early treatment with pathologic fracture in late. That was in a study by Avlov and Pimer. I haven't seen that anecdotally. I talked to Bob about it. Bob was my partner. I miss him. He's now in Buffalo. But it was an interesting study nonetheless. There's similar rates of healing and complication in a different study by Sassoon. And then there's another study by Lynn that said the best way to treat is midaxial calcium sulfate. I typically use either cadaveric or the patient's own bone. So here's an example of a 25-year-old right-hand dominant male, gradually enlarging mass of just the right small finger, noted for three to four years. Works in information technology. You can see the small finger. You blow it up. You see, okay, that looks like an enchondroma on radiograph. Likes to bowl. And so what do you do? Well, it looks like a benign lesion. Painful with activities. There is a cosmetic deformity. It's expansile with increasing symptoms. You know that if this ended up being malignant, you'd have to treat it with probably a partial digital amputation. So, but it looks pretty benign. So the right answer is open biopsy with curatage and bone grafting, not straight to an amputation. I approach this through a midaxial incision on a non-appositional surface of the digit where I use a K-wire, curets, and I harvest, if the patient says, I don't want cadaveric bone because I don't want that in my body, I want my own bone, then I'll obviously harvest bone graft from the distal radius first with a separate set of instruments and then not contaminate the first wound. And here again, you see benign cartilage consistent with an enchondroma. Two weeks post-op, you can see the bone graft there. Two months post-op, it's healed. All right, what about ABC? So this can occur in any bone in the body. Most common location is the metaphysis of the lower extremity long bones. The metacarpal is the most common location in the hand. It's solitary, expansile, and erosive, so different appearance than the enchondroma. It's thought to be secondary to trauma or vascular disturbance, and it can be associated with other tumors, which I'll get to in a second. Common in skeletally immature patients the second decade. It's very uncommon after age 30, so most of us are out of the woods. Female to male is two to one. Pathology, cystic lesion, fibrous tissue with foam cells or giant cells, begins in the metaphysis and grows toward the physis, and there is a chromosomal translocation associated with it. Usually has a rapid course, pain and swelling, acute pain associated with pathologic fracture. They're warm, they're well-perfused, and symptoms can increase with the hormonal changes associated with pregnancy. Can also get vertebral problems with cord or root compression. They expand rapidly. They distort joint surfaces. The risk of local recurrence is extremely high. MRI and CT will potentially show fluid-fluid levels. The treatment is intralesional treatment with curettage and bone grafting or cryosurgery. So what's the radiographic differential diagnosis? This is what they'll try to show you a picture of, and then they'll ask you a bunch of these things. ABC, simple bone cysts, giant cell tumor of bone, telangiectatic osteosarcoma or angiosarcoma. Here's an example of a radiographic feature. You can see that this looks loculated. It looks almost as though it wants to have fluid-fluid levels on a plain film. It expands out. It does not cross the growth plate. So expanded cortex, medullary canal, the metaphysis, and all the things I just told you. CT scan, look for fluid-fluid levels. Bone scan will show increased uptake in the marginal lesion with normal background and decreased uptake in the center where the blood is. So here again is another example of a proximal phalanx with a aneurysmal bone cyst. Very similar radiographic features. Here you see higher-level imaging with the fluid-fluid levels. Here's an example of one that was removed. Here's fluid-fluid levels with a water-like density on top of the blood on CT scan. Here's a case example involving a phalanx with aneurysmal bone cyst. This is what they look like on growths if you slice them. So here you see on the histo purple-lining stromal cells. You see multinucleated giant cells. I wish I had a laser pointer to point them out, but if you look carefully, you can see them all over the place. There's fibroblasts, and the red stuff is pools of blood. So again, the pathology is benign stromal tissue with large vascular lacunae. You see the multinucleated giant cells. You see clotted blood. There's no endothelial lining to the vessels. And sometimes it can be hard to distinguish an ABC from a giant cell tumor of bone, but on a test question, you're gonna wanna look for blood. That'll be the tip-off that it's an ABC and not a GCT. And you can also look for hemocytin, which will be that brown staining that you'll see. And then you'll think, okay, this is not just a giant cell tumor. This is an ABC. There are also secondary ABCs, like I mentioned, that arise with other tumors. These typically occur in NOF, chondroblastoma, osteoblastoma, UBCs, chondromyxoid fibroma, and with fibrous dysplasia. Here's more histo. Here's a higher-power image. Again, you can see blood, multinucleated giant cells. Again, similar here, low-power and higher-power micrograph. What's the treatment? Curitage and bone grafting has a 20 to 40% recurrence rate, so you have to counsel the patients that these are likely to come back. Recurrence can be managed with more aggressive curitage incision, like complete excision and bone allograft. X-ray therapy can be useful, but it's difficult in inaccessible areas. And so usually marginal excision or wide excision with bone graft is preferable. After curitage alone, there's a 60% recurrence. Revision options include on-block excision, cryosurgery, or phenol, filled with bone graft or cement, or amputation. Okay, so what about giant cell tumor of bone? These occur in the metaphysis of mature long bones, expands to subchondral plate, and may destroy the subchondral plate. High rates of local recurrence with giant cell tumors. It behaves as a benign, aggressive tumor, but rarely they can metastasize and cause death, and that has been tested. It's most frequently in the third and fourth decades. The distal radius is the most common primary site in patients with metastasis, and the differential diagnosis is osteosarcoma. So interlesional excision while preserving the joint is obviously desirable. The local recurrence rates are seven to 30%. You can help with adjuvant therapy. A wider section for lesions with extensive joint involvement is sometimes necessary, or when you have pathologic fractures that have extensive cortical breakthrough, and then you have to obviously reconstruct the bone. In 2010, Lau in a journal of orthopedic research showed that bisphosphonates can be potential inhibitors of giant cell tumor stromal cells and can be used as an adjuvant therapy. I haven't seen that on a test question, but it could pop up, I suppose. Giant cell tumor radiographs, here's an example of a giant cell tumor. Contrast this with what you saw with the ABC, where you see this crossed what would have been the growth plate or where there might be a growth plate, and it's expanding into the joint. What's the pathology? Again, no blood here. You see multinucleated giant cells and stromal cells that are likely to merge to form these giant cells. Here's a case example of a giant cell tumor involving the distal radius, which I told you is a common location. Mencken, again, another paper where he studied 24 patients with distal radius osteoarticular allograft, 25% revision in 8.1 years, seven for arthrodesis, one for amputation for tumor recurrence. Three were pain-free, nine had painless strenuous activities, and four had pain with moderate exercise. So these are not typically very easy to treat. Okay, I think we're all familiar with osteoid osteomas. Five to 15% of these lesions occur in the hand and the wrist commonly in the proximal phalanx and the carpus. Lytic lesions are much more common than sclerotic lesions. They respond to anti-inflammatories. These are treated with simple curatase and excision. You can use radiofrequency ablation in long bones, but it's questionable in the hand, secondary to soft tissue necrosis that's associated with it. Here's an example of an osteoid osteoma that is creating a nail deformity. You can see that it's expansile slightly. There's a lytic area that's pushing up on where the germinal matrix is growing the nail. And here's how you remove it. Remove the nail plate, make a longitudinal incision, and take out the osteoid osteoma, and then patients love you. Osteochondroma. So this is a benign tumor of childhood. The etiology is peripheral cells break away from the physis. Growth halts at skeletal maturity. There's an example of one on the phalanx. It's the most common benign tumor of the body. It's most common in the second decade, 11 to 20 years old. It has a benign bony exostosis with a cartilage cap. And what's important about that is that these typically stop growing at maturity. If they're still growing, or if the patient's already mature and you see something like this, you have to think to yourself, this could be something bad. And there's less than 1% of malignant transformation. The characteristic features are bone with a cartilage cap. Typically the cap is less than one millimeter thick. And if it's greater than two centimeters, and you can see that on either imaging or when you're anatomically looking at it, you have to do a workup for malignancy. Typically you can see that on imaging before you get to the point of taking it out. The base of the tumor is contiguous with the cortex of the bone. Often the medullary canal is contiguous with the lesion itself. And they can come in a couple different flavors. They can be pedunculated or they can be relatively sessile. So there's one that's a little bit more sessile, and here's one that's a little bit more pedunculated. So they occur in all upper extremity tubular bones. The treatment is observation. Again, low chance of malignant transformation. If they're painful or if they're causing snapping of tendons, or if they're causing a compression of nerves or vessels, or if there's some kind of angulation or obstruction of motion, that's the indication to treat them. So here's an example of one of my patients who had a proximal humerus sessile osteochondroma that was causing pain and problems with range of motion. So there's a CT scan, and there's after it's been removed. Here's one of a digit. Okay, so what about MHE? So this is osteochondromas that are different. These are multiple hereditary exostoses. It has autosomal dominant inheritance, which has been tested more than once. These are multiple osteochondromas involving multiple bones. Patients often have mild decrease in stature, leg length discrepancies, angular deformities, and the risk of malignant transformation here is less than 5%, so slightly higher. And as you see these contrasts where you think osteochondroma, but they tell you all these things, you have to say, okay, the test question answer is gonna be higher chance of malignant transformation. You know, two or three steps removed from understanding about osteochondromas in general. So what about BPOP or Nora's lesion? This is a mouthful, benign parosteal osteochondromas proliferation. Bizarre parosteal osteochondromas proliferation. I say this to my kids when I'm looking at this, preparing for you guys, and they just start laughing. Rare BPOP is much easier. Rare bone lesion, it affects the hands of patients in their 20s to 30s. Surgical management is definitely warranted to rule out malignancies, because these can be harlequins, if you will. So the differential diagnosis is osteochondroma, no periosteal reaction, though, and local recurrences in 50%, so these can come back. Here's an example of what one looks like radiographically in between the index finger and the middle finger, metacarpal heads adjacent to the collateral ligaments. On gross, you see a nodular surface with glistening cartilage, but it may have this strange, distinct blue tint. The histology is very cellular cartilage, a proliferation of bizarre fibroblasts, disorganized bone, kind of like everything. You get this mishmash of everything. All right, here's a bonus case example. This is a 32-year-old right-hand dominant Amish male that presented to my clinic after falling off a horse. You can see that he has a distal radius fracture. He also has not a lot of healthcare, type 1 diabetes, diabetic retinopathy, end-stage renal disease on dialysis, hypertension. I mean, for 32, you're like, oh my goodness. So if you see something like this and you see all these types of things and the question stem, you say, hmm, I remember something maybe about metabolic bone disease. And you look at the wrist films, you're like, I see these lytic lesions in the scaphoid, in the capitate, and his bone doesn't look that great. Radius fracture is pretty well reduced. Here's your CT scan, because you wanna know a little bit more about what's going on, and you see this mothy appearance, and you're thinking, is this an infection? With this guy's medical history of diabetes, what could this be? Remember, infection can mimic everything. But then you think to yourself, well, he's got this renal disease. There's more images. So this guy has renal osteodystrophy. And this can be tested and can mimic tumors. So the pathogenesis is renal dysfunction that leads to phosphate retention. And then you end up getting the secondary hyperparathyroidism and then the technical term for this is osteitis fibrosis cystica. And it's due to high bone turnover due to hyperparathyroidism. So renal osteodystrophy has been tested as well. So they like to go completely crazy and go to that metabolic bone disease. Okay, so that's all the benign bone tumors. Now we're gonna move on to the malignant ones. So these are rare tumors. They are mesenchymal cell origin. The incidence is one of 5,000 tumors. 15% occur in the upper extremity. So it's rare, and then it's even rarer that they occur in the upper extremity. Prognosis is typically based on size, grade, primary versus recurrent, and then the cell type. So let's talk about chondrosarcomas. It's the most common malignant bone tumor in the hand. They test that. It's usually a slow-growing, painless mass, 26% greater than 10-year presence. Usually presents in the fifth or sixth decades. Like I mentioned earlier, it can occur from malignant degeneration in olease disease or Mefuchi's. Hand lesions do not normally metastasize. And the low-grade tumors are difficult to distinguish from end chondromas on pathology. So if you're sending something that you think is an end chondroma, it's important to mention to the pathologist that it came from the hand, which you obviously will do. So common in the pelvis, proximal femur, shoulder, ribs, sternum. Second to osteosarcoma in frequency of malignancy. The peak incidence is fifth to sixth decades. Affects the metathesis. And remember, the patients with olease and Mefuchi syndrome. It's a little repetitive, but I wanted to hit it twice. So what about chondrosarcomas? Their primary tumors are low-grade, high-grade. They can be differentiated. They can also come as clear cell or mesenchymal. So there's a bunch of different types. The secondary are the ones that typically arise in pre-existing benign cartilage lesions. These are the ones that are the end chondromas, the osteochondromas, fibrous dysplasia, periosteochondromas, synovocondromatosis, and chondroblastoma, et cetera. So what do you see on radiographs? Here you'll see diffuse expanse allelitic lesion that's poorly marginated. You'll see large soft tissue masses, plus or minus flocculant calcifications. You see destruction of the underlying bone, cortical thickening with endosteal scalping. And the CT scan and MRI are really critical here to give you a much better picture of what the tumor looks like in the surrounding anatomy. Here's an example of a chondrosarcoma involving the hand with a patient at the VA. This is not the same patient, but this is how that one was treated. Both were treated with ray resection. Here's another one, chondrosarcoma. Here's the gross, and here's the histo. So this is the important part. You see, in contrast to the endochondromas, here you'll see binucleated cells, you'll see nuclear crowding, you'll see cellular atypia, you'll see mitosis, or pleomorphism, pleomorphism is another word for atypia. So cells all look different. So if you see this, even though you see lots of cartilage, you see cells that don't look the same. You have to think chondrosarcoma. And they'll contrast that. It'll get you to choose between endochondroma and chondrosarcoma. Okay, so here's again the same very, same features, periosteal chondrosarcoma. Normal hyaline cartilage on the left for comparison, and then chondrosarcoma on the right. Treatment is really the only effective, surgery is the only effective treatment. There's a limited role for chemotherapy or radiation, so don't choose chemotherapy or radiation for the treatment of these. The overall occurrence rate is 10 to 15%. There's really been no change in how we treat these over the last 30 years. Prognosis is 100% based on the grade. Okay, so the ones that look bad do worse, okay. So here's a case example of a chondrosarcoma. A 52-year-old right-hand dominant male, growing mass in the forearm. It's palpable mass within the radial distal forearm and in the radius, mild tenderness, weakness with motor, normal sensation. So you look at that, and you're like, ooh, that does not look very good. So here's your MRI. That looks even worse. So open biopsy was the gold standard. You have a histologic-type chondrosarcoma, grade one, so that's actually some good news. This was treated with wide excision followed by complex reconstruction. I used a vascular fibular autograft and a primary wrist fusion. This guy was an awesome guy. This guy was a farrier, and his son was a world champion PBR bull rider who said this story. So I'm gonna take a brief break because I know you guys are like, oh my God, there's so much to cover. But I'll tell you this story just to bring you back. I met this son in the clinic, and he's like, I talked to him, and I'm not a very good Southern accent guy, but I said, so what's the worst injury you ever had? His son, who was pretty upset that his dad just got diagnosed with cancer, he's like, well, this one time, I fell off the horse, and I stuck my tibia into the ground. I was like, okay, that's pretty bad. And I was like, okay, what's another one? He's like, well, this one time, the horse stepped on my chest, and I was in the ICU for a week. He's like, but I got back on on day eight and rode again. I was like, okay. I was like, all right, come on, you gotta give me one more. He's like, well, this is the one where I just about lost it. He pulls up his belt buckle, and I see this. I'm like, yeah, well, this is, I came back and I won this belt buckle after this time that the horse kicked me in the face and knocked out all my teeth and broke my jaw. And he just drops out his entire rack of teeth. And I was like, note to self, don't be a professional bull rider. So I went online and I looked at what his earnings were, and he had made some money, but definitely not worth it. But his dad ended up doing really well, and he was a real pleasure to treat. So, all right. So anyway, here's three and a half months post-op. There's the fibula and the plate. And he's now shooing horses again, but it took him a little while. Okay, so what about osteosarcoma? The incidence in the hand is 0.18. It's the most common location in the upper extremity is the proximal humerus. So hand lesions are typically seen in older adults. The question is, like what we're doing with X-rays and pinning fractures, be careful about how much radiation your hands are seeing. The most common metastatic site is the lung. Pain and swelling symptoms typically present three to 12 months before diagnosis. On imaging, you'll see this classic sunburst pattern. So this is what they'll show you. You'll see this, you'll be like, okay. And you'll think, oh yeah, I remember Codman's triangles from way back. And these can be osteoblastic or osteolytic. The osteoid is produced in the stroma. And when you look at them histologically, you can make a decision, are these osteoblastic? Are they more bone? Are they more cartilage like chondroblastic? Are they more vessel? Are they telangiectatic? Or are they more fibroblasts? So here you should see, this is yet a new pattern recognition slide where you see malignant osteoid, cells that are all different, and tons of pleomorphism. The treatment for these is neoadjuvant chemotherapy. So very different than the chondrosarcomas, and you have to remember that because they like to compare do you know how to treat an osteosarcoma versus a chondrosarcoma. The treatment is wide excision, amputation, surgical treatment of choice, it's either one of those. There's no role for radiation. And the tumor necrosis response of greater than 90% is what you want to see after chemotherapy. And then patients typically will do a little bit better. So here's an example of one of the proximal humerus, the most common location in the upper extremity. Long plate, allograft. So Ewing sarcoma. So this is the round blue cell tumor that we all know about. The presentation is similar to an infection because they present with pain, swelling, erythema, lymphadenopathy. You have the classic T11 to 22 translocation. These are large lytic destructive lesions. They're associated with a soft tissue mass. You have periosteal elevation. They're most common in the humerus, usually in younger people, 10 to 15 years of age. So here you see this. You say, oh, this does not look good. It's in the humerus in a young person with periosteal elevation. You do have improved survival with adjuvant chemotherapy, especially if there's a tumor response, but you have to treat these with wide excision and adequate margins. What about metastatic disease? Remember, these are the great imitators, like infection. These are rare in the upper extremity, but if they do occur, you're most likely to see them in the distal phalanx. 33% hand mets are previously undiagnosed cancer. So if you see something that looks like metastatic disease in a patient at the VA, for example, you have to think, uh-oh, there's something else going on. Is it prostate cancer? Could be. So hand metastases are a prognostic sign. Lung primary accounts for 40% of all hand mets. On radiographic examination, you'll see lytic, expansile, and destructive lesions. You have to think the differential could be any of these. It could be infection, could be of the bone, could be a felon with erosion into the bone. You could have gout. You could have RA. Or you could have, as Ryan Calfee called it yesterday, craps, I suppose. But if I saw this, I would say, this is not craps, this is something bad. Or something I need to make sure is not bad. So what's the treatment? It's typically palliative, because these patients don't do very well. Their typical life expectancy is less than six months. Okay. I'm going to drink tea for a quick second. We're halfway through. You guys are doing awesome. We're going to talk about benign soft tissue tumors. I'm trying not to pound the mic with my voice. So okay. But I assume you guys are turning down the gain or whatever. Okay. So what about ganglion cysts? We all treat these. So this is the most common soft tissue tumor in the hand. You have mucin-filled cysts that are attached to underlying joint capsule or a tendon sheath. There's really no reports of malignant degeneration. But you can have malignant soft tissue tumors that are misdiagnosed as ganglion cysts. So remember, typically, unless you're absolutely certain, you want to culture what you biopsy, biopsy with culture. And if you take tissue out of a patient, you want to just at least think about it. I don't send all ganglion cysts for PATH. I don't think that's necessary. But if I have any suspicion or if I have patients that are big worriers, then I obviously do. So typically, if you see these, they'll be located in the dorsal wrist, overlying the scapulonate ligament. And when you take these out, make sure you're careful with the scapulonate ligament so you don't inadvertently damage it. You can aspirate these and give steroid, but that typically only has 30% long-term success. But I think it's a reasonable first approach. And surgical excision can have recurrence rates as high as 10%. If they're on the volar wrist, they're usually originating from the radial scapula joint or the STT joint. And they're always intimately associated with the radial artery, at least stuck to it. And you have to find the radial artery or cut it, which is bad. So mucous cysts occur out in the fingertips. They often cause nail bed deformities. And then you can also have cysts from carpometacarpal bosses, retinacular cysts, first-dorsal compartment cysts, all that kind of stuff. Here's an example of a large volar radial ganglion. Again, stuck to the radial artery that you do not want to cut. What about the epidermal inclusion cysts? These are fun. We probably all treat these. These are the most common cutaneous cyst. They're sebaceous or epidermoid cysts. They can occur all over the place, but we commonly see them in the hand, like here, because we take care of people that puncture themselves. It basically originates from another word that my kids love, follicular infundibulum. It's usually from trauma. When you cut them open, the contents are gross. Cheesy mixture of degraded lipid and keratin. Looks like cottage cheese. Interestingly, these can erode bone in the phalanges in some cases if they've been there for a while. But you'll see erosion from the outside, and you won't typically. It'll look more benign than something from the inside out. They'll be from the outside in. What about desmoids? This is an infiltrative tumor that's very difficult to treat. I hate seeing these when I've seen them. I've seen a few of them in the hand, and they're just tough. They're histologically benign, but they behave aggressively. Less than 5% of them, thankfully, occur in the hand and the forearm. In the early stages, you can treat them with wide excision, but they often recur. That's why they're so difficult. There has been a questionable role of radiation therapy. They can also respond to tamoxifen because they can be estrogen receptor positive. You can shrink them sometimes with chemo like this, but usually they just grow slowly in the inoperable cases. Here's an example of one in the wrist. What about neurolemoma? This is the schwannoma, or the benign peripheral nerve sheath tumor. It's the most common benign nerve tumor in the upper extremity. Many of you have seen these. The cell of origin is the schwann cell. Patients will often have a TINL sign at the location of the lesion in the nerve. These have two components that they like to test, the antony A and the antony B regions. Surgery is to shell out the tumor, but remember, you have to tell patients ahead of time that they may have some neurologic complication afterwards. If you don't tell them ahead of time, they're upset. Even sometimes, if you do tell them, they're upset. This is what they look like. Here's a case example of one. Normal appearing plain films, of course, but then you have an MRI that shows slight enhancement like on the previous MRI a slide ago of post-contrast T2 images. You can see it adjacent there to the metacarpal, deep to the flexor tendons and the median nerve and the arch. Here's one that was taken out in the arm. You can see it within the ulnar nerve after you take it out. I actually think that was the medial antebrachial cutaneous nerve, sorry, not the ulnar nerve. There was one that I did in the ulnar nerve that I didn't include. What about neurofibroma? These arise in the nerve fascicles, so different than the schwannomas. These are slow-growing, painless lesions usually found in patients with neurofibromatosis, but they can actually be found in any nerve. It doesn't have to be with somebody with neurofibromatosis. If you see a rapid increase in size, like I told you on the very early slides, this should raise suspicion of something that could be having a malignant transformation. Typically they're in the 20 to 30-year-old age group. Almost always, when you take care of these, surgical removal means different than the schwannoma. It means sacrificing the nerve. A lot of times you try to observe these. Here's an example of somebody with neurofibromatosis with neurofibromas in the hand and the ulnar nerve. Here's an example of MRI imaging of neurofibroma. You can see that it involves the whole nerve. Here's another case example of taking out a neurofibroma and grafting the ulnar nerve. This actually, interestingly, was strange. It was a very cutaneous nerve that actually was a neurofibroma, and I thought it was going to end up being a mucous cyst, but because it looked funny when I took out the cyst, I ended up sending it to PATH. So giant cell tumor of the tendon sheath. We've all seen these. These are slowly progressive, painless, firm, multilobular. Assess the joints for infiltration, because these, as you know, have these tendrils that can go almost anywhere. You'll see multinucleated giant cells and xanthoma cells on the PATH. May have increased cellularity or mitotic activity, so they could look scary. And the local recurrence rate, even though they're benign, is as high as 40 to 50 percent. So typically, these look similar to PBNS, but PBNS occurs in the joint, and you'll see these large multinucleated giant cells. Here's an example of a giant cell tumor of the tendon sheath along the flexor surface of the ring finger and along the extensor surface of the small finger. The key to these is when you take them out is to really just try to make sure you keep them as well encapsulated as you can, and really take your time, and then hopefully they won't come back. Here's another one that was, I don't know how, somebody waits again to come in with this. It's like, oh, that's been there a long time. And they often push the nerves away. Occasionally they can invade the bone. So here's an example of one that was along the flexor surface of the middle phalanx, and you can see that it actually was eroding the middle phalanx just proximal to the DIP joint. This one was a little bit more difficult to manage. Okay, what about the glomus tumor? These are fun. Glomus tumor is a neoplasm of smooth muscle cells in the glomus. It involves the subungal area of the digit. Patients have horrific pain that's sensitive to temperature, and I've seen patients that like literally are disabled from this. Occasionally I'll get MRI with gadolinium to see if I can make sure that I'm clear on the diagnosis, but usually the history gives it away. And these are treated with marginal surgical excision recurrence rate as high as 10%. And I've had patients say, just cut off my finger if you can't fix this problem because it hurts so much. So here's one that involves the undersurface of the sterile matrix. And you take them out, and then patients are like, that's amazing. You're the best. So what about pyogenic granulomas? These are also fun. Again, this is a patient that I don't quite frankly understand how they were walking around with this pyogenic granuloma on the tip of their finger for a while, especially since it was their middle finger, I think. But anyway, these are usually not painful, but they can bleed like crazy. And I always, not always, but I often will take care of this in the clinic in the procedure room with removal and silver nitrate and sometimes a finger tourniquet. This particular one I had the resident do it just so that they could be surprised and just bleeding everywhere. I'm like, well, why don't you put the finger tourniquet on? So anyway, these usually go away once you treat them properly. The patient was unbelievably happy when we removed this, and I literally don't know how he walked around with it for as long as he did. Here's an example again. These can sometimes become infected, but if you're careful, they're easy to manage. What about the lipomas? These are painless soft tissue rubbery masses. Lipomas in the palm can be substantial in size. They can be histologically similar to local fat, and the MRI can be diagnostic. These are similar image intensity of surrounding fat on both T1 and T2 images. Here's an example of a very large one involving the hand. This was not my case. I think this was Peter Murray's case. Okay, we're on to the last section. You guys are doing great. To malignant soft tissue tumors. The first one we'll talk about is epithelioid sarcoma. So this is the most common soft tissue sarcoma in the hand. And these usually have an innocuous appearance, slow growing, and they often are misdiagnosed. The average time to diagnosis of these is 18 months, and that's been tested. They usually present as painless, firm, nodule-to-digits in the palm or the forearm. Sometimes patients allow them to start ulcerating, again, which I don't understand. But young adults, average age is 33, and they commonly spread to lymph nodes. And usually you have to treat these with radiation brachytherapy initially for local control. They can be positive EMA, which pops up on test questions, and that stands for epithelial membrane antigen, which should be easy to remember, because epithelioid sarcoma and epithelial are similar words. So here, again, you see a ton of pleomorphism with a whole lot of cellular atypia. Here's a 32-year-old female with a five-year history of recurring infections, quote unquote, of the left thumb, multiple debridements, positive cultures, but no biopsy was ever obtained, surprisingly. So this is, again, I mentioned this once before, but remember to culture what you biopsy and biopsy what you culture. So here you have erosion from underlying tumor. You get an MRI, you say, ooh, that doesn't look that great, and you take it out and try not to shorten their thumb or try to figure out how you're going to do a toe-to-hand later. So what about MFH? This is pleomorphic sarcoma high grade. It's the most common sarcoma in general, not the most common sarcoma in the upper extremity. 19% of these occur in the upper extremity. It's the sixth to eighth decade of life, so slightly older population than the epithelioid sarcomas. They can arise as bone tumors. Occasionally they metastasize to the lung. And here you'll see something looks a little bit different than the epithelioid sarcoma. You'll see these spindle-shaped fibroblasts in a story of form pattern like you can see here on this histologic specimen. Here's an 85-year-old minister with a one-year history of a draining left forearm mass. And here you can see a large soft tissue tumor involving multiple compartments. So that involves a big reconstruction. So what about synovial cell sarcoma? So this is associated with tendons, bursa, and joints. The wrist is the most common area in the upper extremity if it occurs there. They like to test on synovial sarcoma. That's why you see so much yellow on this slide. They've been present for years. You can see these type of calcifications on radiographs. The treatment is wide resection or amputation. There is a role for adjuvant chemotherapy and radiation. The five-year survival is 82%, but there is a 32% recurrence rate. And you have to remember that these go to lung and lymph nodes. So here's a case example of a spindle cell sarcoma, 16-month-old child found to have forearm mass by family. No other issues really. So you look at this and you say, okay, I can see that there on x-ray there's a soft tissue mass, particularly on that lateral image. And you say, hmm, the radius looks a little bit strange. Doesn't look kind of classic. It could be really anything. Could it be rhabdo? Maybe. Could it be an extra-skeletal Ewing's potentially? Could it be a vascular malformation? Maybe even possibly more likely. Could it be hematoma? Probably not likely. You have an ultrasound and it's solid and it has blood flow in it. So it's not a hematoma. It's not an abscess or some strange ganglion. That almost certainly is not something good now that you're starting to get more imaging. And here you have T1 and T2 and then post-contrast images where you see enhancing intramuscular mass that looks like a lot of masses. Again, it's not an abscess or a hematoma. There is some necrosis. So you think this needs a biopsy and it's probably going to be something bad. In this case, wanted to know a little bit more about the vessels. So here's an MR angiogram to understand how it was being recruited, how vessels were being recruited. Open biopsy and then it was a malignant soft tissue sarcoma. And so you had to treat it with wide resection. Here's the path. Again, looks bad. You can see a bad-looking tumor, very active cellularly that's invading muscle in the soft tissue. And again, here you see all sorts of mitoses and cellular atypia. What about clear cell sarcoma? So this is a malignant melanoma of soft parts. It's a distinct soft tissue sarcoma that lacks the involvement of the epidermis. And you can see round cells with a clear-appearing cytoplasm, as you can see in this histologic slide. It's associated with tendons and or aponeurotic junctions. It's usually slow-growing and most patients can develop occurrences in metastasis. So this is a bad one. This is one of my patients that thought they had a spider bite. And I had a biopsy, came back as a clear cell sarcoma, ended up getting a wide resection and then a radial forearm flap with skin graft. And she lived for about 14 months or so. Bad metastatic disease. Nice lady. So what about liposarcoma? So liposarcomas, 10.6 of liposarcomas occur in the shoulder, arm, and forearm. So you may see these and you may think that they might be a lipoma, but just beware. They typically occur in the fourth or fifth decades of life. They have a painful, large mass of sizable proportions. The most common is a well-differentiated or a myxoid. Surgical removal is often incomplete because they're expansile, they're not as well-encapsulated as the typical lipomas that we treat. And the recurrence is common. These met to the lungs, metastasize to the lungs. Here's a large one in the back of the arm. Bigger section and then a flap. I did this with one of our partners. What about rhabdomyosarcoma? Most common soft tissue sarcomas of childhood. These rapidly grow. They can be very substantial, but these are typically painful. The question is whether or not these can be associated with pre-existing trauma. It's a skeletal muscle tumor. Lung and lymph nodes are common metastatic sites for these. These are often treated with wide excision, adjuvant chemotherapy, methotrexate, and radiation. The five-year survival is 80%, so not quite so bad. Angiosarcoma. This one gets tested a ton. I don't know why. Less than 1% of soft tissue sarcomas, so I don't know why they test something that's so uncommon, but it has a poor prognosis. They occur as skin or subcutaneous lesions. Endothelial cells are arranged in vascular channels. You see chronic lymphedema as an etiologic factor, potentially. Patients that they might give you in the question stem, somebody who's had a history of a radical lymph node dissection for breast cancer, and then they'll give you something else that would push you towards angiosarcoma. Remember that these are typically called lymph angiosarcomas or Stuart-Trevis syndrome. It can happen in 0.5% of post-mastectomy patients with lymphedema. This is the highest yield part of this. What I try to do is group some commonalities so that when you go back and you review right before your test, you can kind of put some linkages together after all those basically definitions and descriptions. So synovial sarcoma. These arise near the joints, but rarely within the joints. The most common sarcoma in young adults, so these typically are in 15 to 40 years old. The genetics is X18 translocation, seen in greater than 90%, and these are treated with resection and radiation. What about rhabdomyosarcoma, the most common sarcoma in children? There's four subtypes, embryonal, alveolar, botryoid, and pleomorphic. The genetics here is a 213 translocation, and you just have to memorize these. I don't know how else to remember it myself. Treatment is wide surgical resection with radiation. What about liposarcoma? So that's different still. These typically occur in older individuals. So there's multiple histologic subtypes. There's well-differentiated, which is like an atypical lipoma. There's mixoid, there's round cell, pleomorphic, and de-differentiated. The translocation here is 1216, and it's commonly the one that's described for the mixoids. And the treatment here really depends on the subtype. The ones that are well-differentiated can be treated almost like a regular well-encapsulated lipoma, but the other ones need to be treated with wide resection, clear margins, and radiation. So rhabdomyosarcoma, the most common sarcoma in children. Again, that's a repeat slide. I don't know why that's there again. Maybe I hit backwards. I'm hitting backwards by accident. I'm so sorry. I'm like, why am I doing that again? I don't think there'd be two slides. So there's many, many more. Fibrosarcoma, angiosarcoma, leiomyosarcoma, lymphangiosarcoma, dermatofibrosarcoma, protuberans. There's like a bunch. So just know that there's a bunch. This doesn't really get tested. So what about the treatment for bone and soft tissue sarcomas? Limb salvage is very important in the upper extremity. There are barriers that help confine the tumor, permit limb salvage, extensor retinaculum, transverse carpal ligament, things like that. But surgery is wide excision with a three centimeter margin. Positive margins are really unacceptable because positive margins mean recurrence, and recurrence means metastatic disease, and metastatic disease means death. So 29% incidence of METs at primary sarcoma presentation. 53% with recurrent sarcoma presentation. Radiation does diminish the recurrence in most soft tissue sarcomas by 50%. And hand and soft tissue sarcoma tumor size greater than five centimeters has the worst prognosis, and they've tested that. So know that five centimeters is a magic number. So typically you're treating these with external beam radiation. The downside to this is that you have potential wound complication rates four times the wound complication rates than if you don't, but patients do better. You can also do brachytherapy as well. You have less radiation, and usually brachytherapy is more soft tissue sparing. So chemotherapy is often combined with XRT in the treatment of these. There's more compelling results of chemotherapy in bone sarcomas than in the soft tissue sarcomas, and there's more compelling results with radiation in the soft tissue sarcomas than in the bone tumors. And if you just think about that conceptually, it makes sense. It's probably easier to irradiate a soft tissue sarcoma than a bone tumor, and it's probably easier to treat bone tumors with chemo, but that's at least how I remember it. So with limb salvage techniques, radiation, chemotherapy, both 70% to 80% five-year survival expected for many bone and soft tissue sarcomas, but really the ultimate survival is dependent on tumor grade, the size, the depth, and the stage, as I mentioned. So what about, there's a couple oddballs here that I'm going to include. Dermatofibromasarcoma protuberans, or DFSP. This is a low-grade malignancy, painless firm nodule, overlying skin changes, form is the most common location. So the reason I mention this is because you just want to be aware that it can happen in the upper extremity. The risk of metastasis is low, but local recurrence is high, and that's because it usually doesn't get clear margins. So you need to have greater than 2.5 centimeter margins, not just take out the mass itself. What about malignant peripheral nerve sheath tumors? So typically these are occurring in patients with neurofibromatosis, third to six decades, they spread along the nerve sheath. There is a high recurrence rate, the five-year survival is 44%. So in patients that have neurofibromatosis and then have this, it's not a good thing, and these metastasize to the lung. Here's a case of a malignant peripheral nerve sheath tumor, I think that was Warren Hammert's case. Okay, so what are the high-yield commonalities? So what has similar histology? Fibrosarcoma, desmoid, and MFH. So those three, so those will often be potential answers to a question, and you want to be prepared to be able to tell the difference between the three of them based on something else in the history, whether it be a genetic thing or histologic thing. Enchondroma and low-grade chondrosarcoma, I think we drove that home during the lecture to be able to differentiate between the two of those. And then giant cell tumor of the tendon sheath and PBNS. One occurs within the joint and one occurs outside the joint, but the path looks very similar in histo. So what has similar MRI appearance? Neurolemoma, neurofibroma, and malignant peripheral nerve sheath tumor. But again, like I mentioned, they're going to give you something different in the history like the patient may have neurofibromatosis. Soft-tissue sarcomas that are found in the sub-Q tissues. This is another set of commonalities where you have DFSP, epithelial sarcoma, MFH, and then angiosarcoma, but they're all different and they'll give you something, but just know that these all occur in the subcutaneous tissue. Tissues that are, soft-tissue sarcomas that are involving the aponeurotic tendon and bursa. Clear cell sarcomas like the example that I showed you and the one that had the rate of form fibrous sarcoma and synovial cell sarcoma. What are the soft-tissue sarcomas that go to the lymph nodes? So synovial, epithelioid, clear cell, and rhabdomyosarcoma. And I hit this a bunch of times during the lecture, but what are the ones that metastasize to the lung? This is the most common location for metastatic disease for bone and soft-tissue sarcomas. This is the most common primary tumor for METs to the hand and the destination of METs from the giant cell tumors of bone from the distal radius. The vascular conditions tumors, these typically will have endothelial hyperplasia, but then there's also malformations. They're not tumors. These have dysmorphogenesis with normal endothelial cell turnover. So these tumors are going to be the ones that are the hemangiomas. There are others. In theory, it is one of these vascular conditions, angiosarcoma and Kaposi sarcoma in patients that have HIV. Malformations. These typically are a whole bunch of different subtypes, and the classification is mostly driven by the radiologists because they're the ones who are mostly treating these malformations. These are venous, lymphatic, arteriovenous, capillary, and combined. And they're really getting pretty good at treating those, so I don't typically treat a lot of these vascular malformations. Surgically, they end up becoming a really difficult thing to treat because they often recur. Hemangioma is a benign vascular proliferation. It's four to one female to male. They can be superficial, capillary, or deep or cavernous. The deep ones often have high flow characteristics that you can see on ultrasound. And typically, there's a single cutaneous lesion. They often appear during the first four weeks of life. They're rapid growth during the first 12 months. They have slow involution, 70% regress by age seven years, and 90% by age nine. There is something to be aware of called Kasbach-Merritt syndrome. It's a potential fatal coagulopathy. You have platelet trapping, and you end up getting hemangiothrombocytopenia syndrome. Here's an example of some hemangiomas in some young patients. Really bad. Treatment in adults is usually by marginal excision. They can require debulking in children for large symptomatic lesions. There is a lot of advancement in cryosurgery and laser ablation that's working really, really well. And you can also, when you have discrete vessels, do embolization. These are not tumors. They typically present at birth, but they may not be clinically evident. The growth is typically commensurate with a child and affected by hormonal and other factors, male to females, one to one. These are typically classified, as I mentioned, on channel type, slow flow versus high flow, capillary venous lymphatic. And you guys survived. Thanks so much.

bone tumors

soft tissue tumors

enchondromas

osteosarcomas

lipomas

ganglion cysts

diagnosis

surgical excision

chemotherapy