Okay, good morning. This is great that you're all here. This is a monster lecture, an hour of tumors, and what I've tried to do is break it into a way that's easy to cover it by category, and I've also tried to really highlight the things that I think are high yield. On most of the questions that we see, the tumor questions are pretty straightforward. So if you know what's here, you'll get the questions right. And here we go. I might take a little break to sip some water, if I can, in a little bit, because you get a little part talking at you guys for an hour, but I'll try to make it as interesting as I can. So I have no disclosures. I'd like to first thank Peter Murray and Warren Hammert, both of whom have given this lecture before me, and helped me as I put together some of their cases in addition to my own to make this lecture palatable for you guys. So here's what we'll cover. We'll cover briefly about history and physical examination and the workup. We'll talk a little about some of the principles of biopsy, and then we'll get into tumors of bone, and then we'll finish with tumors of soft tissue, and go over some questions at the end. Usually patients present with a recent painful or painless mass. The thing that's most important to pay attention to, is there a change in character or an enlargement in the mass? Treatment delays for soft tissue sarcomas are usually things that, like infections or ganglia or hematomas, meaning people think that the patient has an infection, a ganglion or hematoma, and they miss a soft tissue sarcoma. When you look at the physical examination, size is important. Big is bad. Something greater than three to five centimeters is concerning. Ask yourself the question, is it fixed or is it mobile? Are there changes in the overlying skin or hypervascularity? Is there any compromise of nerves or vessels? And you should always examine in regional lymph nodes, and also clearly pay attention to range of motion and adjacent joint. The workup for these types of tumors is with CBC and BMP, plane radiographs, and MRI of the region with contrast. You can also use bone scintigraphy or bone scan. This detects the osteoblastic response as opposed to the tumor itself. Something that we see on test questions is that bone scintigraphy may miss early metastatic disease, and PET scan measures the metabolic activity of the tumor. You can also get a chest X-ray or chest CT to make sure that they do not have early METs, and then always get a biopsy. Chest CT rules out metastatic disease. METs to the lungs are the most common in these tumors, and they usually occur within the first year. Lymph nodes are less common, but they typically do occur with soft tissue sarcomas. Here's an example of a bone scan on the left with the disease in a femur. Here's an example of a PET scan on the right. Once again, this comes up on test questions. This measures the metabolic activity of the tumor, and it may pick up early metastatic disease. The definition of a biopsy, the technique of harvesting a representative sample of tissue without compromising definitive care. If we look at the types of biopsies for accuracy, fine needle aspiration is accurate 73% of the time. Core needle, 83 to 93%. An open biopsy with frozen section, 80%. It's difficult to assess fat and cartilage using a frozen. An open biopsy with permanent section is by far the most accurate at 96%. Open biopsy is the gold standard. You should use a linear incision, and think ahead. You should plan to incorporate the incision that you're using into a limb salvage incision, particularly if you're dealing with something that could potentially be bad, and this was written in core in 2000. The principles of open biopsy, make your incisions in line with the eventual definitive surgery. Use a longitudinal or extensile incision. It's concerning when you see something that somebody thought was a ganglion, and they use a transverse incision because it was cosmetic, and it turns out to be a solid tumor that could be bad. Stay intramuscular in a single compartment, and typically if you're taking a biopsy of a tumor that you have concerns about being malignant, you want a biopsy from the peripheral portion of the lesion. This is the area where there are viable cells rather than a center that might be necrotic. Mencken's landmark article in 1982 gets referenced on test questions periodically. Biopsy problems are three to five times more common at a referring rather than a treating institution if they're performed at a referring. So biopsy should be generally performed by a surgeon capable of definitive treatment, and you have to be able to anticipate the future treatment during your biopsy. Fine needle aspirations come up periodically on test questions, and an article in core in 2000, 66 primary bone tubers were evaluated via FNA, 73% were diagnostic. Diagnosis was slightly higher in sarcomas. The cost is cheaper than open biopsy, but it's not as accurate. This comes up on questions as well, knowing the staging systems. MSTS is pretty straightforward. This is for bone sarcomas. So stage one includes the low-grade tumors, A and B is intra versus extracompartmental. Stage two is the high-grade tumors, A and B is intra and extracompartmental. Stage three is any metastatic disease. The linking staging system is somewhat similar. Again stage one, low-grade, but you look at intracompartmental versus extracompartmental for 1A and 1B. Stage two or high-grade, the same, intra versus extra, and then stage three, any metastatic disease, intra versus extracompartmental are the same. So if you look, I put two examples for you. Here's an example, low-grade extracompartmental tumor without metastasis is stage two, and low-grade intracompartmental tumor with METs automatically is stage three. This is one that gets a little bit confusing, so you just have to memorize it. This is the AJCC staging system, only applies to soft tissue sarcomas, and they place the emphasis more on size and superficial versus deep than the grade. And like I said, you just have to memorize it, and that's why I provided it for you. Okay, moving on. So now that was the introduction, and now we're going to move on benign tumors of bone. So these are ones that should be familiar to most of you, and this should be a relatively straightforward review. Let's start with enchondroma. Enchondroma is the most common benign bone tumor in the hand. The... I'm going to go back for a second if it'll let me. The most frequent locations, proximal phalanx, middle phalanx, and the distal metacarpal. You can see an example of one here in the base of the proximal phalanx of a ring finger. What about if you see multiple enchondromas? That yellow box should be over the MCP joint for the right index and middle fingers. It somehow got moved when it was reformatted. Ollier's disease is multiple enchondromas. Mufuchi syndrome is multiple enchondromas and hemangiomas that present as calcitic stippling on radiographs. And here's an example of one at a distal ulna with changes throughout the hand, as you can see in a patient with Mufuchi syndrome. If we think about enchondromas, the malignant transformation of solitary enchondromas is extremely rare. By contrast, if patients have Ollier's disease or Mufuchi syndrome, it can be 25 or even higher in these cases. Usually these occur beyond the age of 10, between the ages of 10 and 40, often most in the fourth decade. And usually patients present with enchondromas as a pathologic fracture, and you pick them up as an incidental finding on an X-ray. On an X-ray of an enchondroma, you will see usually a small or less than five millimeter lytic lesion with intramedullary calcifications. Sometimes they're described as popcorn, punctate, stippled. There's usually no cortical involvement. There's no periosteal reaction. Sometimes you can see minimal endosteal scalloping and long bones. If you see this, it should be less than two-thirds of the cortical thickness. And they typically can have more aggressive features in the hand and still be benign. If you look at these on path, usually plain radiographs are significant for diagnosis, but you also have to think about chondrosarcoma, and on path you want to make sure that you're telling your pathologist that you've taken this lesion from the hand as well as from elsewhere. And the histology will show benign cartilage with occasional calcifications. They can be cellular, but you won't see mitotic activity. You will not see binucleated cells. And the histology will have a relatively uniform appearance, as you can see in these two pictures to the right. The treatment for symptomatic lesions is intralesional curatage with or without bone graft or cement. This is well described in multiple papers. One of the better is from 2006, JHS. You can essentially put almost anything in these lesions, and they should heal. There is some interesting controversy in the literature about the ideal treatment for end chondromas with pathologic fracture. So Ablov and Pimer in 2000 published higher complication rates reported in early treatment compared to late. In JHS in 2012, they reported similar rates of healing and complication. And you can also use something like osteocell, like I was saying, you can use calcium sulfate or bone graft to fill these lesions, and that was well described in 2013. Here's a case example, 25-year-old right hand dominant male, gradually enlarging mass of the distal right small finger, present for three to four years, works in IT. On this x-ray, you can look at the very distal tip and the distal phalanx, and you can see an intramedullary lesion that looks relatively benign. They have pain with activities, no other systemic symptoms, slightly expansile. This has been there for a while. So when you consider this benign lesion with maybe some aggressive features, painful with activities, cosmetic deformity, expansile, your options are you can monitor it, you can take a biopsy, you can do an open biopsy, you can do a partial amputation, which I would not recommend, so the answer is open biopsy with curettage and bone grafting. You can use an end to the distal phalanx through a mid-axial approach, avoid the appositional surface of the digit. You can use a K-wire or a small curette to enter the lesion. In this case, I took bone graft first from the end of the radius before proceeding with treating the lesion, and then I filled the void, and the path was consistent with an enchondroma. In two weeks, looking like it's been filled with bone, at two months, it's healing and no recurrence. Okay, so let's talk about aneurysmal bone cysts. Aneurysmal bone cysts can occur in any bone in the body. The most common location is the metaphysis of the lower extremity long bones. If it occurs in the hand, the metacarpal is the most common location. It is a solitary, expansile, and erosive lesion. It's thought to be secondary to trauma or a vascular disturbance, and it can be associated with another tumor. If it occurs in skeletal immature patients, it's usually in the second decade. It's very common after age 30. It typically occurs in skeletal immature patients. Female to male ratio is two to one, and the path will show a cystic lesion with fibrous tissue and foam giant cells. It begins the metaphysis and grows toward the physis, and it can be associated with a chromosomal translocation. There's usually a rapid course. Patients present with pain and swelling. These are all things that they'll give you in the question stem, potentially. Acute pain associated with pathologic fracture. They can have local warmth. Again, 25% are associated with previous trauma. Symptoms may increase with pregnancy, and a vertebral lesion can present with cord or nerve root compression. They expand rapidly. They distort joint surfaces. The risk of local recurrence is high. On an MRI, you may see fluid levels, and intralesional treatment with curettage and bone grafting is the treatment. You have higher healing rates with increased cellular to osteofibrillar ratio on histology. You can also use cryosurgery. The radiographic differential diagnosis for these tumors is obviously ABC or a simple bone cyst. Could be a giant cell tumor of bone. Could be a telangiectatic osteosarcoma or an angiosarcoma. So this is where the differential gets a little bit more complex. Here's an example of an expanse allelitic lesion extending to the growth plate in a distal ulna on an AP or a PA of a wrist. Again, on these, you'll get an X-ray, and you'll see a radial lesion with an expanded cortex involving the medullary canal of a metathesis. The portion will have an expanded appearance of the cortex, but it'll be contained by periosteum in a thin shell of cortical bone. It rarely penetrates the growth plate or the articular surface. If you have a CT scan, you should look for a fluid-fluid level. And a bone scan will show increased uptake in the margin of the lesion with normal background or decreased uptake in its center. So again, here's some X-rays as well as MRI of one of these where you can see expansion of the cortex. It abuts the growth plate. Has a greater diameter than the growth plate as compared to like a unicameral bone cyst, which is not broader than the emphasis. And an MRI, you can see loculation. You can see fluid-fluid levels, blood cells at the bottom, dark, and serum on the top, which is light. Here again is a plain film and an MRI and then a gross specimen of an ABC. If you see this on a CT, you'll see a fluid-fluid level that presents a water-like density layered on top of blood. Here's a case example of an ABC involving a digit. You can see a relatively large expansile lesion involving a ring finger, gross section. When you look at these on path, you'll see the purple area, which would be lining of stromal cells. And then you'll see multinucleated giant cells and fibroblasts. And then you'll see red pools of blood as well. So you see benign stromal tissue with large vascular lacunae separated by septae in which numerous giant cells are found and filled with clotted blood that resembles cranberry sauce. You have no endothelial lining to these vessels. And sometimes the ABC can be hard to distinguish from a giant cell tumor or bone because you will see these large giant cells, but you should look for hemocytarin and then you'll be pushed towards a potential diagnosis of an ABC. You can also have secondary ABCs. So these arise in other tumors, as I mentioned before, as a result of changes in hemodynamics. They can occur secondary to a non-oscifying fibroma, chondroblastoma, osteoblastoma, UBCs, chondromyxoid fibroma, and even fibrous dysplasia. Again here's some more examples. I put these here so you can just see lots of examples of what these might look like where you can see blood, hemocytarin, and you can see the giant cells. Here more low, these come from self-assessment exam questions. So they give you images that aren't quite as good as the ones that I just showed you from a real PATH specimen sometimes. So the treatment again is curatage and bone grafting. You do have a 20 to 40% recurrence rate. Recurrence can be managed with more aggressive curatage and excision. You can treat these with x-ray therapy in inaccessible areas such as the vertebral bodies. Marginal excision or wide excision with bone grafting is preferable. So again 60% recurrence sometimes after curatage. Excision options include on-block excision, cryosurgery, or phenol, and you can fill the bone with graft or cement. Sometimes in rare cases they even need to be treated with amputation for severely recurrent disease. Okay, we've been talking a little about giant cell tumors of bones, so as distinguished from ABC, so now we'll talk about those. Giant cell tumors typically occur in the metathesis of mature long bones. They expand to the subchondral plate and may destroy it, and they have higher rates of local recurrence. They include mononuclear stromal cells and multinucleated giant cells. It behaves as a benign aggressive tumor, but rarely it can metastasize and cause death. It occurs most frequently in the third and fourth decades. The distal radius is the most common primary in patients that have metastasis, and again this is in yellow, so this pops up on test questions. The differential diagnosis, most other possible option is osteosarcoma. So the best way to treat these is intralesional excision, trying to preserve the joint. Local recurrence rates range from 7 to 30% and potentially higher. You can reduce these rates by adjuvant cryotherapy or phenol. You can treat these with wider section for lesions, but with extension of joint involvement and for other types that have pathologic fractures or extensive cortical breakthrough. Sometimes these are reconstructed with bone graft or fusion with osteochondral allografts. Prefibular reconstruction is recommended, and usually you treat with vascularized fibular grafts and defects that are greater than 6 centimeters. In a journal of orthopedic research in 2010, it was shown that bisphosphonates can be potential inhibitors of giant cell tumor stromal cells and may have a use as adjuvant therapy as well. Here's an example of some x-rays, so you can see this expands beyond the joint, beyond where the growth plate lived, and has large lytic expansile lesion. On path, you'll see multinucleated giant cells. The background will be stromal cells and foamy histiocytes, but you won't see the hemocytin in the blood, as you saw on an ABC. The stromal cells are likely to merge to form multinucleated giant cells. Here's again another example of an expanse allelitic lesion involving the articular surface of the distal radius. In the journal of bone and joint surgery, in the late 90s, 24 patients were studied with distal radius osteoarticular allografts. There was a 25% revision rate at 8 years, 7 required arthrodesis, 1 required amputation. 3 were pain-free, 9 had pain with strenuous activities, and 4 had pain with moderate activities, so these are difficult to treat. Okay, this one should be pretty common to most of you, the osteoid osteoma. Five to 15% of these lesions occur in the hand or the wrist, the proxal phalanx and the carpus are the most common. They are lytic more likely than being sclerotic. They respond to NSAIDs, they're treated with curettage and excision, and radiofrequency ablation can be the first line in long bones, but it's a question of the hand secondary to soft tissue necrosis and the close proximity to neurovascular structures. Here's an example of an osteoid osteoma that's causing a nail deformity involving the distal phalanx of the index finger. Here is treatment for this osteoma with removal of the nail plate, extensile approach to the nail, through the nail bed to allow for repair and then removal. So what about osteochondromas? This is a benign tumor of childhood. This occurs because the peripheral cells break away from the physis, and usually the growth of these tumors stops at skeletal maturity. Here's an example of one involving a ring finger close to a PIP joint. This is the most common benign bone tumor in the body. So most common in the second decade, 11 to 20-year-old, you have benign bony exostosis with a cartilage cap. Again, this typically grows from or near the physis. The common locations are the distal femur, the proximal tibia, proximal humerus, and the distal radius. Again, the growth stops after maturity. There's less than 1% risk of malignant transformation. So if growing after maturity, then you may think that further studies are needed. It has characteristic features, bone with a cartilage cap. The cap is typically less than one centimeter thick, and if you have something greater than two centimeters, then you really need to start thinking about something bad. The base of the tumor is contiguous with the cortex of the bone. Often the medullary canal is contiguous with the lesion. They can appear as either sessile or pedunculated. Here's one involving the proximal femur and one involving the lateral aspect of the distal femur. These occur in all upper extremity tubular bones. The treatment often is observation. There's less than 1% chance of malignant transformation. But if they are painful, if they are causing neurovascular compromise, if they are causing problems with motion, then you can treat these with local excision. Here's a case example of one that I treated that involved a large sessile lesion of the proximal humerus that was causing some problems with the neurovascular structures adjacent to it. Here's a CT of what that looked like. And here's what it looked like after we took it out. Here's one involving a digit. So these also come in a slightly different flavor, which is MHE or multiple hereditary exostosis. This has autosomal dominant inheritance. This is multiple osteochondromas involving multiple bones. There are some associations, mild decrease in stature, leg length discrepancies, and angular deformities, particularly at the knee, the elbow, and the ankle. And the risk of malignant transformations of these to chondrosarcoma is less than 5%. Okay, we'll briefly talk about BPOP or Norris lesion or benign parosteal osteochondromas proliferation. When I'm feeling particularly grumpy, I'll ask my residents to say this 500 times in one fell swoop and get them tongue-tied, and then they can't talk when we're trying to do something else during the case. So it's a rare bone lesion. It affects the hands of patients in 20 to 30-year-olds. Involves the proximal, middle phalanges, and the MCPs. Surgical management warranted to rule out malignancy in these cases. The differential diagnosis includes osteochondroma. There's no continuity of the medullary canal when you see these lesions. There's no periosteal action, but the local recurrence is about 50%. Here's an example of a Norris lesion in the second web space between the index and the middle finger metacarpals. It looks a little concerning. If you look at these on path, gross, you'll see a nodular surface with glistening cartilage. It may have a distinct blue tint. I think that's difficult to see. The systology will show a very cellular cartilage and a proliferation of bizarre fibroblasts and disorganized bone with spindle-shaped fibroblasts. The peripheral lesion has occasional cartilage with large chondrocytes without columnation as is seen in the osteochondromas. So it won't look like normal cartilage. Here's a bonus case example. This is a 32-year-old right-hand dominant Amish male who fell off a horse. His past milk history is important for type 1 diabetes, diabetic retinopathy, end-stage renal disease on dialysis, hypertension, CHF, and a history of PE. Remember when the question writers write these tests, they probably are developing their question in bone tumors from a real patient example. And if you see some of these things in a question stem and you think it might be a tumor question, you might say to yourself, hmm, diabetes, end-stage renal disease, on dialysis, why did they put that stuff there? You see they have a distal radius fracture. Maybe something kind of funny looking in the carpal bones. So CT scan, you see something still funny looking with this lytic process with periarticular erosions. You might think something like infection which can mimic just about anything. And you can see the distal radius fracture, multiple lytic lesions in the carpus. And the radiology report would say something like multiple erosions mainly involving the carpus but also the metaphyseal heads which represents, which in the setting of dialysis may represent amyloidosis. However, this is a nonspecific finding. It could also be crystalline arthropathy such as CPPD and inflammatory arthropathy and then the infectious etiology. So you get basically everything. And the most likely diagnosis in this case is renal osteodystrophy. And the pathogenesis of this problem is a renal dysfunction leads to phosphate retention. And then you have secondary hyperparathyroidism. And then you get this osteitis fibrosis cystica secondary to high bone turnover with hyperparathyroidism. So I put that in there just because not all things are tumors and this might present as such but just be aware that this can happen. All right, so now we're on to the malignant tumors of bone. These are rare tumors. They are mesenchymal cell origin or from the mesoderm. The incident is one in 5,000 and 15% occur in the upper extremity. And the prognosis is based on size, grade, primary versus recurrent and then the cell type. Chondrosarcoma is the most common malignant bone tumor in the hand. This question has occurred again and again and again over self-assessment exams. So that one you guys will most certainly not get wrong. It's a slow-growing painless mass. 26% of them have been present for 10 years. They typically present in the fifth or sixth decades. They can occur from malignant degeneration of an enchondroma such as Aliase disease as we just talked about. And the hand lesions, interestingly, usually do not metastasize. And sometimes the low-grade tumors are difficult to distinguish from enchondromas on pathology. The epidemiology of chondrosarcomas is that they're common in the pelvis, they're common in the femur, they're common in the shoulder, ribs, and sternum. They're second to osteosarcoma in frequency and malignancy. The peak incidence is in the fifth to sixth decades. They affect the metathesis. And patients with Aliase and Mifushi syndrome can often present in the third or fourth decades or 20 to 30-year-olds, so a little bit earlier. There are various types of chondrosarcomas. There are primary, they come in low-grade, high-grade, de-differentiated, clear cell and mesenchymal. And then there are secondary, so these arise in pre-existing benign cartilage lesions such as in enchondromas, osteochondromas, fibrous dysplasia, periosteochondromas, synovial chondromatosis, and chondroblastoma. So this is just some nomenclature. When you look at these on an X-ray, you'll see diffuse, expanse, isolated lesions, poorly marginated. You'll have a large soft tissue mass, plus or minus surrounding flocculitis, surrounding flocculant calcifications. You'll have destruction of the underlying bone with cortical thickening and endosteal scalping. You will also see simple calcifications. And usually a CT scan and MRI give a much better picture of the tumor and the surrounding anatomy and soft tissue involvement. And in these cases, you should get bone scan for metastatic disease. Here's a case example of a VA patient of mine that had a chondrosarcoma involving the middle finger. So you see a large expansile mass involving the hand and the middle finger, involving most of the proximal phalanx, treated with a ray resection. Gross exam. And on histology, you'll see binucleated cells with nuclear crowding, mitosis, and pleomorphism. So all the cells will look different, and that's how this is different than the end chondroma. So you'll see cartilage, but then you'll see cells in all sorts of different ranges of maturity and mitosis. Here's some more examples. So you can see the invasiveness into the bone in comparison to the chondromas. There's an example of normal hyaline cartilage on the left, and there's an example of chondrosarcoma on the right. So just look at the hypercellularity of the chondrosarcoma. The treatment of chondrosarcomas, surgery is the only effective treatment. They try to fool you with this and have you treat patients with chondrosarcomas with radiation or chemo and don't do that, don't fall for that trap. So there's limited role for chemo and radiation. The overall recurrence is 10 to 15%, and there's really been no change in the treatment of this disease over the last 30 years. The prognosis for the five-year survival for grade ones is 88, grade twos, 75, grade three, and 40%. So as I was telling you, grade is important for this. De-differentiated chondrosarcomas do very poorly. And usually, clear cell periosteal have better prognosis and have rare metastasis. Here's a case example of a chondrosarcoma, 52-year-old, right-hand dominant male, one-year growing mass to the right forearm, past medical and surgery history none, palpable mass in the right distal form and radius, mild tenderness with deep palpation, motor weakness to grip, normal sensation, and median ulnar and radial distributions. So if you look at this, if this person were to show up to your clinic, you'd say this guy was actually a farrier from northern Wisconsin and one of the nicest, salty-earth guys. Son was a national champion PBR bull rider. And I'll never forget that. I'll share the story, because we're going fast and we're halfway through and you guys are doing great. But I remember talking to the son in the clinic one day before surgery, and he was wearing running shoes and a baseball hat and a shirt and jeans. And I asked him what the worst injury he ever had was. And he says, well, this one time, the horse kicked me in the face and it knocked out all my teeth. And all these teeth just pulled out this whole rack of teeth. And I was like, oh, what's the next one? He said, well, this one time, I fell off the horse and I broke my femur. That hurt quite a bit, but I was riding a week later. I said, okay, come on, what's the absolute worst thing you've ever seen? And he goes, well, this one time, I fell off the horse and he jumped on my chest and I was in the ICU for two weeks. But I was able to get back and ride my horse a week later. And then I went online, I looked him up and I was like, holy smokes, this guy had won all sorts of stuff. And his earnings were like, I don't know, two or $300,000 a year for taking that kind of a whooping. So one of the, like I said, one of the nicest guys. So back to the case, that was just a little breather there. So here's what it looks like in the distal radius. So this looks bad, right? This is, you guys should be pretty concerned about this if you see this on an MRI. The open biopsy pathology showed a histologic type chondrosarcoma with intra-tubecular invasion and rare focal necrosis, but appears to be confined to the bone. So grade one, so that's good. So the plan was for wide excision followed by complex reconstruction with a vascularized fibula and primary wrist fusion. So here's an intraoperative shot of bridging, removing essentially the whole proximal row of the distal radius and then putting in a vascularized fibula to the capitate from the radius and then bridging with a long plate. And this is three and a half months post-op. This is 10 months post-op. So he actually got back to being a farrier, which is pretty cool. So osteosarcomas, the instance in the hand is about 0.18%. It's the most common location. The upper extremity is the proximal humerus. Hand lesions are typically seen in older adults, maybe potentially to radiation. So be careful as we use x-ray machines. Be really conscious of how often you're sticking your hand when you're treating your patients. The most common metastatic site is the lung. Patients present with pain and swelling and often symptoms are present for three to 12 months before the diagnosis. On imaging, you'll see something super bad, sunburst pattern, periosteal elevation. You may see a Codman's triangle, which we teach all of our residents to look for. They can present as either blastic or lytic. You'll see osteoid producing the stroma on a histologic specimen. Again, they can come as osteoblastic, chondroblastic, telangiectatic, or fibroblastic. Here you should see malignant osteoid and the cells all look different. So cells making tons of bone and cells in various stages of maturity. The treatment for these is neoadjuvant chemotherapy and then wide excision and amputation. There's no rule for external beam radiation. And when you give patients with chemotherapy, you're looking for the tumor necrosis response of greater than 90% because these patients will typically do better. Here's an example of an osteosarcoma involving the proximal humerus with allograft reconstruction. And then plate fixation. What about Ewing's sarcoma? This is the round blue cell tumor that we all remember. Presentation is similar to infection, but patients will present with pain, swelling, erythema, and fever. And this is one that comes with a T11 to 22 translocation. They love to test that. So this is a large lytic destructive lesion associated with a soft tissue mass. There's periosteal elevation, most common in the humerus in 10 to 15 years of age. There's an example of that lesion in a distal humerus. So again, there's improved survival with adjuvant chemotherapy followed by wide excision with adequate margins. Okay, what about metastatic disease? Rare in the upper extremity. If it does occur, the distal phalanx is most frequently involved in the hand. 33% of hand METs are from previously undiagnosed cancer. And hand METs are a very poor prognostic sign for patient survival. And the lung primary accounts for 40% of all hand metastasis. And they love to test that on questions as well. On imaging, you'll see something that is lytic, expansile, destructive, such as what you see in this proximal phalanx of a digit. The differential diagnosis is osteomyelitis, felon, gout, RA, or as we all now know, craps. Treatment is palliative. The average life expectancy is less than six months. And this is written in a landmark article in 1987 in the Journal of Hand Surgery. Okay, so that's bone tumors. And now we're on to benign soft tissue tumors. This is one we all know, the ganglion cysts. It's the most common soft tissue tumor of the hand. So all comers, benign, malignant, everything. This is the most common soft tissue tumor of the hand. Mucin-filled cysts attach to underlying joint capsular tendon sheath. There's no reports of malignant degeneration, but malignant soft tissue tumors may be misdiagnosed as ganglion cysts, as I mentioned earlier. We see that occasionally. Typically, they're located over the dorsal SL ligament in 60, 70%. You can also see them volatilely along the radial artery. These can be treated, the dorsal ones in particular, can be treated with aspiration plus or minus steroid. They have about 30% long-term success in the literature. And if you take these out, you should tell your patients there's about a 10% chance of them possibly coming back. And then you should hopefully beat those odds. When they do present on the volaris, they're usually located originating from the STT joint or the radioscapoid joint. Sometimes they can originate from the flexor carpi radialis sheath. And then there's always the mucous cysts, which are similar, but usually related to arthritis in the DIP joint. Sometimes you can have them associated with a carbametacarbobas. Sometimes they're from the PIP joint. Sometimes they're over the first dorsal compartment after patients have had first dorsal compartment corticosteroid injections. Here's a case example of a large volar radial ganglion. They often extend a long ways down to the carpus. And if you use tourniquet in these cases, or if you use a wide-awake local anesthetic, whatever you choose, you just have to make sure that you're careful to respect the radial artery. Otherwise, you'll have to repair it or ligate it. So epidermal inclusion cysts, this is the most common cutaneous cyst. It's also called an epidermal cyst. The size can be variable, but usually no bigger than two to three centimeters. They originate from the follicular infundibulum, usually secondary from previous trauma. The contents are always gross. The med students are always like, that's nasty. Cheesy mixture of degraded lipid and keratin. And they can erode bone in the phalanges. So you have to be aware of that for epidermal inclusion cysts, because they might give you some history and then show you something that looks like an erosion from the outside of the bone, making you think that it's an incondromal, but you'll have to be smart and figure out that it's an epidermal inclusion cyst. What about desmoids? These are infiltrated tumors. They present challenging surgical problems. I don't like it when these show up to clinic, because they can be really difficult to treat. They're histologically benign, but they behave aggressively. Thankfully, less than 5% of desmoids occur in the hand in the form. Wide excision is preferred in the early stages, but the recurrence rate is super high. Their recurrence rate is higher in children and adolescents. Intralesional excision essentially means absolutely going to recur. There's a questionable role for therapy, but again, this is difficult to administer in the upper extremity. They may be estrogen receptor positive, and they may respond to tamoxifen. And so sometimes you can shrink these preoperatively, and you can slow the growth or treat inoperable situations with this type of therapy. Here's an example of a desmoid involving the distal upper extremity close to the wrist. What about the neurolemoma, or schwannoma, or benign peripheral nerve sheath tumor? This is the most common benign nerve tumor at the upper extremity. The cell of origin is the Schwann cell. The neurologic examination is normal, but patients will have a TINL sign over the nerve at the side of the lesion. There's two components, the Antony A and the Antony B component. And surgery is to shell out the tumor, but you must tell your patients that there's a 4% neurologic complication with injury. Here's an example of a schwannoma in the form. Here's a case example. This was one that was a little bit difficult to pick up because it had been persisting for a while, and then ultimately we ordered an MRI, and you can see the slight enhancement on post-contrast CT. You can see it's pretty deep beneath the carpal, the contents of the carpal tunnel, adjacent to the metacarpal and the intrinsics. Remember, tell patients that they have a 4% chance of nerve symptoms when you take these out afterwards, if you can shell them out, even if you can shell them out nicely, and they still may have some issues. What about neurofibroma? These arrive in the nerve fascicles. They are slow-growing, painless lesions. Usually occur in patients with neurofibromatosis, but it can be found in any nerve. If you do see a rapid increase in size, this should raise the suspicion for possible ligament transformation. They typically occur in the 20 to 30-year-olds. Surgical removal means sacrifice of the nerve, and sometimes observation is a reasonable alternative, and sometimes you need to be prepared for nerve grafting. Here's an example of a patient with a neurofibroma involving the ulnar nerve and a patient with neurofibromatosis. Here's an example of what they look like on MRI. Here's the reconstruction. Here's another one. I think that might have been in the wrong place, that slide, sorry. So, giant cell tumor of the tendon sheath. These also we see commonly. Slowly progressive, painless, firm, multilobular. You want to assess adjacent joints for infiltration. You will see multinucleated giant cells and xanthoma cells. They may have increase in cellularity or mitotic activity, and the treatment is marginal excision. Remember, the recurrence rate can be high. They just have these leg tendrils, these fibrils that will go kind of all over the place, and they look yellow, and you have to pull them away from the fat and identify that it's extending beyond what you think might be the well-encapsulated part of this, and so the recurrence rate is pretty high. So, this is a benign reactive lesion, which is similar to PVNS. Usually, they present as tena-synovial giant cell tumors, so this is not giant cell tumor of bone. This is a circumstriped tumor that does not always arise from the tendon sheath, but it may arise from the synovium as well. You will see a population of oval cells set in a minor condensed eosinophilic fibrostroma, and you'll see scattered multinucleated giant cells. Here's an example of a large giant cell tumor of the tendon sheath involving the flexor tendons of a ring finger. Here's another one. Here's another one. So, this is an example of one that was eroding bone, which can happen as well in giant cell tumor of tendon sheaths that have been there for a while. They can erode the adjacent bone. Okay, glomus tumors. These come up on test questions pretty frequently as well. The glomus tumor is a neoplasm of smooth muscle cells of the glomus. So, don't be afraid that if you see a question stem that makes you think it's a glomus tumor and you see glomus in the answer, don't think that they're trying to fool you. It might be there because that's the answer. They usually occur in the subulnar area of the digit. You have radiating pain that's very sensitive to temperature. You have point tenderness over the lesion and they can become disabling. Sometimes these are imaged with MRI with contrast and you can really get nice pictures with a good magnet and a patient that's not moving. They are treated with marginal excision, but the recurrence rate can be as high as 10% because you missed the lesion. So, here's an example of a glomus tumor involving the nail bed. We'll take out the glomus tumor after you and then repair the nail bed. What about pyogenic granuloma? These are pretty common. Disorder of angiogenesis, cause remains unknown, but often trauma. So, endothelial cells and mast cells are seen in pathology. Usually they're not painful, but they can bleed like snot. Treatment with silver nitrate and cauterization is the first line. For small tumors, larger ones you can treat with marginal excision and a cuff of normal tissue to help prevent recurrence, which is relatively uncommon. So, here's one that this patient of mine was walking around with this one on the tip of his finger for a long time. I don't know exactly know how he was able to do that and still type, but he did. And here's another example of it. It's like a little extra buddy just hanging out on his finger. So, what about lipomas? Painless, soft, rubbery mass. Lipomas of the palm can be substantial in size. The histology is similar to local fat. The MRI can be diagnostic. So, lipomas are of similar image intensity as surrounding fat on both T1 and T2 images. Here's a large lipoma involving the palm. All right, we're on to the last section, malignant soft tissues of the upper extremity. What about epithelioid sarcoma? This is the most common soft tissue sarcoma of the hand. They have an innocuous appearance. They're slow growing, which leads to misdiagnosis. And the average time for diagnosis of epithelioid sarcoma is sometimes as long as 18 months. They present a painless sperm nodule on the digits in the palm of the forearm. Sometimes patients can have draining skin or ulceration over the mass if they're present for a really long time. They typically occur in young adults, which is bad. And they spread to lymph nodes, which is also bad. And sometimes radiation or brachytherapy is used for local control. If you look at the histology, they will usually tell you in the question stem that they are positive EMA. Here's a 32-year-old female with a five-year history of recurring infections of the left thumb. Multiple debridements, positive cultures, but no biopsy ever obtained. So remember, culture what you biopsy and biopsy what you culture if you're looking at bad stuff so that this doesn't just go on and on and on and on and on. So this is an epithelioid sarcoma involving the thumb. Here's an example of the MRI. So what about MFH? MFH are pleomorphic high-grade sarcomas. Most common soft tissue sarcoma overall, and 19% of these occur in the upper extremity. Six to eight decade of life. So older patients, they again present as painless, rapid growth and trauma. It's often undiagnosed. They can also arise as bone tumors, curiously. These typically metastasize to the lung. And they're treated with wide excision adjuvant radiation. And there's a 65% five-year survival rate. And there's a 10 to 30% recurrence rate with wide margins and radiation therapy. And if you can do a radical excision, the recurrence rate approaches 0%. And on histology, you'll see spindle-shaped fibroblasts with a storiform pattern, as you can see in this histo slide here. Here's an 85-year-old minister with a one-year history of draining left forearm mass. This looks, again, very concerning for a soft tissue sarcoma. So here's a wide excision. So what about synovial sarcoma? This is associated with tendons, bursa, and the joints. The wrist is the most common area if it occurs in the upper extremity, but you can see them in the elbows, such as this picture here. Usually, it's a slow-growing, painless mass that's been present for years. It's also seen in young adults. You will see calcifications such as this on radiographs. And they're treated with wide resection and amputation. There is a role for adjuvant chemo and therapy. 82% five-year survival, but a 32% recurrence. And these can both metastasize. These can metastasize to both lung and lymph nodes. So 16-month-old child found to have a forearm mass by the family, no obvious pain, but does appear to limit use at times. No obvious neurovascular deficit. All motor function is intact. All passive range of motion is well-tolerated. So you look at this and you say, this is a soft tissue mass with no calcifications. The radius looks a little bit remodeled. Could be anything. Rhabdo, myosarcoma could be possible. Extraskeletal ewings, hemangioma, hematoma, whole bunch of stuff. So there's the mass right there on the X-ray. And then an ultrasound is performed. So you have a longitudinal ultrasound on the left, transverse incision on the right. It's solid. It has blood flow. So it's not really a hematoma. It's not really an abscess or some goofy monster ganglion. It's probably bad, but it's nonspecific at this point until you have this enhancing intramuscular mass that looks a lot like a lot of masses. So it's not an abscess. It's not a hematoma. Has a little bit of necrosis. It's not a hemangioma. Could be a nerve sheath tumor. It's probably the only chance that it's something benign. And it's a high likelihood of something being something malignant, but the differential diagnosis is broad. So here's an MR angiogram that's helpful for preoperative planning because it's pretty large in a small limb. And this helps you figure out the ability to resect a tumor while trying to preserve blood supply to the hand. That's why we ordered it. Open biopsy showed a spindle cell sarcoma with genetic analysis. It's malignant soft tissue sarcoma and the plan is for wider section. And it's a spindle cell sarcoma with genetic analysis that's pending. So what's your plan? Obviously you're gonna take this out, right? So this is what the histo looks like. There you look at all the spindle cells. What about clear cell sarcoma? Malignant melanoma of soft parts. It's a distinct soft tissue sarcoma that lacks involvement of the epidermis. You see round cells with clear cytoplasm bordered by a fibrous network. It's associated with tendons or aponeurotic junctions. It's slow growing, occasionally painful, and often undiagnosed. And most patients will develop a recurrence and a metastasis. This is another one of my patients. So this actually initially thought she had a spider bite. And then had a biopsy on top of a spider bite. And then had a wide excision. And then reconstruction with a radial forearm flap. What about liposarcoma? So 10.6% of liposarcomas are in the shoulder, arm, and forearm. They occur in the fourth and fifth decades of life. Usually a painful, large mass of sizable proportions such as this one here. Most common is well differentiated or myxoid. Surgical removal is often incomplete. They're difficult to treat and recurrence is common requiring these metastasized to the lung. Big tumor in the shoulder. Requiring reconstruction with a flap. What about rhabdomyosarcoma? This is the most soft tissue sarcoma of childhood. Rapid growth, substantial size, painless, sometimes associated with trauma. It's a skeletal muscle tumor. So it's deep seated. And it has undifferentiated mesenchymal cells. Lung and lymph nodes, again, are the most common metastatic sites. This is treated with wide excision, adjuvant chemotherapy, sometime methotrexate radiation, 80% five year survival. Angiosarcoma, less than 1% of soft tissue sarcomas. Poor prognosis, they occur as skin or subcutaneous lesions. You'll see endothelial cells arranged in vascular channels. You'll have chronic lymphedema, recognized as the etiologic factor. You'll have a patient that'll have impaired immunologic status that may potentially develop an angiosarcoma. And usually the other cause is absence of a lymphatic egress from the limbs. So they'll give you this as somebody who's had previous history of breast cancer with lymph node dissection or radical lymph node excision. So angiosarcoma following chronic lymphedema can occur in women post-radical mastectomy. Lymphangiosarcoma or Sturt-Trevis syndrome is what it's often called. It occurs 0.5% in post-mastectomy patients with lymphedema. Okay, so I know I went through the soft tissue sarcomas last. They're less common, but there are some commonalities. And so now I'm gonna go through a review of the soft tissue sarcomas so we hit it again. And then we'll review some commonalities and we'll hopefully help you get the test question right. And you guys are doing great. So synovial cell sarcomas arise near the joints but rarely within joints. Most common sarcoma in young adults 15 to 40. X18 translocation in 90%. The treatment is wide surgical resection with radiation. Rhabdomyosarcoma, most common sarcoma in children. Four subtypes with five year survival. Embryonal, alveolar, botrioid, and pleomorphic. The genetics is a 213 translocation and the treatment is, again, wide surgical resection with radiation. Liposarcoma is the most commonly in older individuals such as that one I showed you on the shoulder. There's multiple histologic subtypes, well differentiated such as an atypical lipoma. Myxoid, which is the most common. And then there's also round cell, pleomorphic, and de-differentiated. The genetics on a myxoid is a 1216 translocation. And the treatment really depends on the subtype. Well differentiated is marginal resection alone. Others, wide resection with radiation. There's many, many others. So fibrosarcoma is previously known as the MFH. Angiosarcoma, leiomyosarcoma, lymphangiosarcoma, dermatofibrosarcoma protuberans. There's over 50 histological subtypes. These are just some of the most common. So what's the treatment for bone and soft tissue sarcomas in general? Limb salvage is extremely important for obvious reasons in the upper extremity. Barriers help to combine the tumor and allow you to have limb salvage, such as the extensor retinaculum and the transocarpal ligament. Treatment is surgery with wide excision with a three centimeter margin. And in treating these cases, really as you saw in the patient that I showed you a little while ago that required the radial forearm flap, positive margins are really unacceptable. So you have to take a lot. And the reason is positive margins equal recurrence. Recurrence equals METS, and METS equals death. So 29% instance of METS at primary sarcoma presentation. So you have to watch out for that. 53% with recurrent sarcoma presentation. So the incidence of metastatic disease goes up when they recur. That should make sense. And radiation diminishes the recurrence in most soft tissue sarcomas by 50%, and that's why you are seeing that radiation is an adjunct to wide excision in all of those cases I presented to you. And a hand soft tissue sarcoma that has a tumor that's a size greater than five centimeter has a worse prognosis, and that has come up on a test question as well. So five centimeters is the number that you're looking for. So what's the treatment for bone and soft tissue sarcomas? Patients can get external beam radiation for pre-op to stage down the tumor, and then you can give a lower dose. But by doing that, you do have a four times more likely incidence of wound complications. And usually when you give the radiation post-op, it's higher dose than pre-op. You can also use brachytherapy, about 40 gray. Usually you use iridium-192 or iodine-125. The nice thing about this is you have less overall radiation and it can be often more soft tissue sparing. So treatment for bone and soft tissue sarcomas is also chemotherapy. It's often combined with XRT, and it's usually you're using this in the bone sarcomas than in the soft tissue sarcomas. And the radiation is usually more compelling in the soft tissue sarcomas, and you guys should see that from the way we were treating those tumors. With limb salvage techniques for treatment of these tumors, with radiation and chemo, you have a 75% to 80% survival rate for most of these. The ultimate survival, again, is dependent on the tumor grade, the size, the depth, and then whether or not they have metastasis or the stage. What about D? This is what I added for this year as it was seen on a test question. A dermatofibromal sarcoma protuberans, or DFSP. This is a low-grade malignancy. It's a painful, firm nodule, overlying skin changes. The form is common. Usually it remains undiagnosed for years. You'll see slender spindle cells with a story-of-form pattern. It's misdiagnosed as a fibrosarcoma. The risk of METS is low, the recurrence is high, and you have higher cure rates, again, with something close to three centimeter margins. Malignant peripheral nerve sheath tumor. 50% of malignant peripheral nerve sheath tumor occur in fibromatosis, third to six decades of life. This is an enlarging, painful mass, neurologic symptoms. They spread along the nerve sheath. There's a high recurrence rate, five-year survival is approximately 44%, and the most common site of metastatic location is the lung. Here's an example of a large malignant peripheral nerve sheath tumor in the upper extremity. Okay, so this is the high-yield commonalities. So what has similar histology? So fibrosarcoma, desmoids, and MFH. Enchondroma and low-grade chondrosarcoma. And giant cell tumor, the tendon sheath, and PVNS. So if you see these things and you're thinking one or the other, then just remember that there's some other things that might have similar histology, and then you can look at the histo and get it right. What things have similar MRI appearance? Neurolemoma, neurofibromas, and the one that I just gave you the malignant peripheral nerve sheath tumors. Soft tissue sarcomas that are found in the subcutaneous tissue. So DFSP, epithelial sarcoma, and MFH, or pleomorphic high-grade sarcoma, and angiosarcoma. So these are the ones that are more superficial. Soft tissue tumors, sarcomas that are involving the aponeurotic tendon and bursa. Clear cell sarcomas, fibrosarcomas, and synovial cell sarcomas. Soft tissue sarcomas that metastasize to the lymph nodes. Synovial, epithelioid, clear cell, and rhabdo. METs to the lung. Most common location for METs from bone and soft tissue sarcomas is the lung. The most common primary tumor for METs is to the hand. And the destination of METs from a giant cell tumor of bone from the distal radius is the lung. And these all come up on test questions, so I'm basically giving you the question stem of the question answer. There are some BASF conditions. So these are more malformations than they are tumors, but I included them just to be complete. The tumors typically have endothelial hyperplasia, and the malformations have dysmorphogenesis with normal endothelial cell turnover. So the tumors, hemangiomas, 85%. The others are glomas, angiosarcoma, and Kaposi sarcoma. The malformations include the venous lymphatic, the arteriovenous, the capillary, and the combined. And I had a recent scare in my own life where my son had a malformation potentially concerning for a tumor, but ultimately it was treated with sclerosis, which is the modern therapy for a low venous malformation. So it's relatively not invasive. So hemangiomas, these are benign vascular proliferations. Four to one, female to male. They can be superficial or deep, and they typically have a single cutaneous lesion. They appear during the first four weeks of life. They rapidly grow for 12 months. They have slow involution. And interestingly, 70% of these regress by age seven and 90% by age nine. There is this Kasselbach-Merritt syndrome, which is a potentially fatal coagulopathy with platelet trapping, and it's associated with hemangioma thrombocytopenia syndrome. Here's an example of hemangioma involving a hand and a digit, one involving a shoulder. Really bad one. This is not my case, involving a hand. Treatment of bulges is removal by marginal excision. They may require debulking in children for large symptomatic lesions. Cryosurgeries can often be used. Our plastic surgery guys use laser ablation as alternative treatments and embolization. So remember, vascular malformations are not tumors. They typically present at birth, but may not be clinically evident. They grow commensurate with the child and are affected by hormonal and other factors, female to male, as one-to-one. They're classified based on channel type, slow flow capillary venous lymphatic, and high flow AVM and AVP.

bone tumors

enchondroma

aneurysmal bone cyst

giant cell tumors

osteosarcoma

soft tissue tumors

ganglion cysts

lipomas

synovial sarcomas