Ovarian Tumors with Dr. Roshni Dasgupta

When pediatric surgeons say that they are true general surgeons, man, they aren't kidding. Everything in the chest, the abdomen, and the pelvis is fair game. But it's probably safe to say that ovarian pathology is something we all can review every once in a while for clinical practice and for board exams. And so that's why today we're going to discuss ovarian tumors with an expert. So it's really important to know about these things and what how to manage them appropriately. That's Dr. Roshni Dasgupta, a pediatric surgeon at Cincinnati Children's Hospital Medical Center, and today, well, she's going to teach us everything we need to know about both the workup and the management of ovarian pathology in the pediatric patient. So stick around. This is the Stay Current podcast. When discussing neoplastic ovarian masses, there are three types based on their tissue of origin. So we can think of the three types of over introins based on their origin. So the epithelium, so epithelial ovarian tumors, which we think of as more adult type tumors, um the stromal elements um which come up with the sex cord tumors, and then the germ cells which are from the yolk sac. Thankfully, malignancy from any of these neoplastic processes are rare, overall making up only 2% of cancers in children. Um obviously a little bit more common in the age group that's older, um the 10 to 19 year old and it's all reportable really um any sort of malignant lesion under the age of five. There are genetic syndromes associated with ovarian tumors and we know that these are both clinically relevant and board exam gold. So Puts Jager is associated primarily with granulosa cell tumors and sex cord tumors, same with Oers um and Mafucci syndrome and then other ones other ones, Cdic Higashi and McCune Albright also associated with ovarian tumors. So just so you know that there are syndromic associations for the ovarian tumors and that if you have a patient with any of these syndromes, you need to have a little higher index of suspicion. Some ovarian tumors may have hormonal functionality. And so that prompts an extensive workup and that can reveal both the underlying tissue type and the neoplastic classification. You have an eight-year-old girl who comes into the emergency room like yesterday, um with a palpable lower abdominal mass. Um you also notice that she's got some pubic hair and some axillary hair on on your physical examination. What are you going to do next? In this child, we're worried about precocious puberty from the ovarian mass. And after asking about associated symptoms like pain and distension and anorexia, urinary changes or changes in bowel habits, all the normal stuff, we need to complete a full workup. This includes a renal panel, CBC, estradiol, progesterone, tumor markers, and an ultrasound to better define the mass. So on your ultrasound, um you see a mass, um and it's big. They can't really tell how big and they can't really tell anything else. Um what what sort of cross-sectional imaging do you want to get and why? This is a tricky one. When deciding between a CT and MRI, there are a few advantages and disadvantages that we should consider. CT scan, the good parts. You can sometimes get a better idea of where it is. Um you get a better idea of the extent of the tumor. Um you can also detect calcifications a little bit better than on MRI. So if there's anything that's calcified within the mass, you can see it a little bit better. Obviously, the bad part of it is that you expose a child to radiation. Um an MRI gives you that better detail. Um it can help you to it can help tell if it's actually an ovarian mass, a parovarian mass. Um often if it's small enough, when when they're very big, it's really hard to tell at all. Um but it is also excellent at detecting lymph nodes. So that's another really important thing that you said. Um, you know, that there's are all the good parts of an MRI, the bad parts are, you know, sometimes you need anesthesia because it takes longer. Sometimes it's harder to get, right? And it also sometimes also costs a lot more money. Tumor markers for this patient, well, they're exhaustive, especially because specific tumor markers are associated with specific tumor types. New tests are really going to be your tumor markers, right? Like we talked about. So the the tumor markers that are commonly thought of with ovarian tumors are exactly what you said, the AFP, the beta hCG, generally CA 125 for epithelial ovarian tumors are are what we think of. But you know, you can argue that for an 8-year-old um not being all that predictive, but then there's something called inhibin A and B that are also that are also sent and we'll talk a little bit more about which which tumors are secrete which ones, but those are really important to think about. But you are and then also your your hormones, um an LDH, CBC, renal are also important things to think about. So you're right on the right track. In this patient with virilization and pubic carrot, only eight years old, we expect an elevated inhibit B, which is associated with a granulosa cell tumor. That is a sex cord tumor, and that is where you think about those virilizing effects. So we think about the 8-year-old with um pubic hair and axillary hair, that's precocious puberty just like you're thinking and virilizing effects. So sometimes you will see that secretion of some of these tumors and sex cord tumor is one of the more common ones that will show that effect. Unfortunately, not all tumor markers are quite so precise. CA 125, for example, well, we get it all the time, but it can be elevated in benign conditions, too. Yeah, so that's why it's sometimes really hard, um because if you have a patient with an endometriosis or other sort of inflammatory conditions, it can be elevated even to the point where it it they go up to the numbers where um you you sort of crossover with malignancy. So it's a hard marker to use to kind of figure out what which one you should do. Okay, so the next type of ovarian neoplasm to consider is a germ cell tumor. And for this, I need to brush up on some embryology. If we think about the blastocyst and the primordial germ cells that happen at in the first week of gestation, um it week three, these cells form the wall of the yolksac yolksac and they migrate along the meentery of the hind gut. And so germ cell tumors, as you know, can be gonadal or extra gonadal, right? So sacrococigeal teratomas or you can have teratomas in your chest. All those are types of germ cell tumors. But we're going to focus on the ones that migrate to the gonads. Depending on where the germ cells migrate, it determines their classification. So, for example, if they migrate to the gonads, we call them gonadal, but if they migrate away from the gonads and become sacrococigeal teratomas or mediastinal lesions, those are extra gonadal. Within the gonads, um the if they differentiate the way they're they're supposed to, um then you can see that they can go on to embryonal carcinomas and then they undergo either embryonic or extra embryonic differentiation. So in that extra embryonic differentiation, those are coriocarcinomas and yolksac tumors. And then on the embryonic side of it, they're teratomas and mixed germ cell tumors. Now, that's if differentiation, if it all goes as planned, and we know that doesn't always happen. So if they don't differentiate properly, we classify them as seminomas in boys and dysgerminomas in girls. So um germ cells are really common um in terms of the malignant neoplasms that we see in children. Um you see them in both prepubertal young girls and in women or girls that have undergone puberty as well. and often they present with really large tumors that can obstruct or. Um about 20% of them are malignant and just like Laura said, AFP is the primary marker for disease and dysgerminomas are the most common malignant histology. Dysgerminomas comprise 30% of the total types of germ cell tumors and they can be bilateral. Additionally, they have a pretty unique laboratory pattern. They often have an elevated LDH, but in a pure dysgerminoma, you can actually have a normal AFP and beta hCG, so it can be a little confusing. Um, and but you can also have um some hypercalcemia as well in these patients. Okay, so let's dive into the specifics of how to treat patients with these lesions. So, let's say we have a 13-year-old patient presents with a palpable mass, elevated AFP to 24,000. This differential is broad with an AFP that high, including hepatoblastoma, a teratoma, a germ line tumor. Again, I wasn't going to say anything, but we're in the ovarian tumor podcast, so it's probably that one. If you want to know more about hepatoblastoma, then check out our podcast with Dr. Tiao. But with that differential and those signings, we definitely need an ultrasound. Let's see what her ultrasound shows. Complex cystic solid mass, um and your AFP is 24,000. There's a bit of fluid in the pelvis on your ultrasound, get measures about 12 centimeters. 12 centimeters? It's pretty big. Definitely needs criteria for an operation, but what are we going to do when we get there? First things first, we need to sample the perineal fluid washings, and then we need to resect the lesion without rupture. Finally, we need to do an intraoperative lymph node exam. Now, it's kind of a tricky delineation between germ cell and epithelial ovarian tumor staging. Okay, you're absolutely right. So you just want to examine lymph nodes. You only want to take out lymph nodes if they are enlarged or suspicious. Okay? That does not require any um sort of standard required lymph node dissection for germ cell tumors. So when you stage a germ cell tumor, you are looking to do peritneal washings, you need to look at the entire peritneal cavity. You only do an omental resection if there are suspicious nodules. You do a unilateral cell oophorectomy and a salpingectomy only if the fallopian tube is involved. If it is not involved, you want to preserve it. Okay? Um if it is possible to preserve any portion of the ovary, you should think about that. Just depends on what it looks like. Um because ovarian preservation is really important to think about as we go through any of these things. And then you want to examine that contralateral ovary. Okay? So that is what germ cell tumor staging is. And that's very it's different from epithelial cell tumor staging. Okay, so I just want to make sure that we're getting this right. So for germ cell tumors, we have to do an intraoperative assessment of the lymph nodes, but we only remove those that are abnormal. And an omental resection, it's not really required. But for all ovarian tumors, peritoneal washings are a must. So when we talk about just staging for germ cell tumors, this is kind of what Chris just talked about, um that you want to make sure that this is what you do and this is um what you do for any tumor that you think is a germ cell. Okay? Um when we think about staging, um is COG staging for germ cell tumors, really stage 1, 2, 3, 4. Um stage one is really limited to the ovary, your washings are negative. Um uh your stage two, you have either rupture. Um you have your rupture of your tumor in terms of into that through the capsule, your peritoneal washings are still negative. Um and then stage three is if you've got lymph node involvement and stage four is if you have distant metastasis. Operating either laparoscopically or an open operation, that's really up to the surgeon's discussion. But whatever you choose, remember, you can't rupture the tumor. When you treat these patients, um generally stage one tumors, um you can just do surgery on. So you if you don't rupture them, you don't cause any of issues, they can be observed and all you have to do is operate on them um and then watch them. However, if you try to do this laparoscopically and rupture it and you spill tumor, then you're you are sort of required to give the patient chemotherapy. So it's really important that you do the do an operation that's safe. And if it if it is within the scope and you're able to do it within the scope, it's totally fine, but it's something you got to make sure that you are able to do without any risk because you can you can significantly change their treatment and their overall outcome um by rupturing the tumor. For patients who require chemotherapy, the standard approach is called ped. It's a platinum, etoposide and bleomycin and it's really effective. Very effective and it's been used for decades and it was very well salvaged and you can see that that six year over overall survival is excellent even in stage four patients. Moving on to teratomas. So there's two types, mature and immature. Both consist of all three primordial tissue types including ectoderm, mesoderm, endoderm, and that means we can see some pretty interesting things inside, like brains and bones and teeth. The cystictomas are the most common and they can be bilateral in about 10% of patients. You see calcifications in them um in a lot of times and that's why that CT scan is is often nice to see. Mature teratomas are probably the most common, but immature are really important to identify. They have neuroepithelial components which are graded on a scale of zero to three. The higher the grade, the more likely to have yolksac fosi and those are concerning for malignant behavior. Most immature teratomas, if you resect them, actually don't need any sort of chemotherapy. So that's the same thing with mature teratomas and immature teratomas, you just have to watch them really carefully. In patients with a mature teratoma, we know those need resection. So in those with a cystic component, it's safe to try and decompress the cyst first. So it'll probably improve access to the teratoma and increase your chances of ovarian preservation. Yeah, so you can definitely do that and if you can find that plane, you can shell up the the mass and leave the ovary alone. You do not need to close the ovarian capsule. People will say you do, you do not. There's no data to show that you need to. It'll just form all by itself. Um, and the other things you can do is you can glue a bag to um a little plastic bowel bag to the ovarian mass itself and you can put a needle through it. You really don't want to spill around anything because you can get something called to growing teratoma syndrome, which can be a real problem, um where you just get teratoma throughout the abdomen and it continues to grow and it's not responsive to chemotherapy. Um so you can try to ovarian spare it, you can limit your incision size both laparoscopically and open by doing these techniques. When considering epithelial ovarian tumors, we have another example of why kids just aren't little adults. So while relatively rare in adults, epithelial tumors account for about 20% of pediatric tumors. They are mucinous and serus and the serus lesions are more commonly bilateral, not the mucinous. The tumor marker of choice is CA 125, but like we talked about, it's just not perfect. Can be elevated in malignant epithelial tumors, but remember they can also be um elevated in the benign conditions like endometriosis and PID. Um, so it's just important to remember that it's not a perfect tumor marker. The staging system for epithelial tumors, well, it's different from germ line tumors, mainly in nodal dissection, that's really required for staging of these, along with an omentectomy. If you have a patient we know or has an elevated and CA 125, she's 17, we think she has an epithelial ovarian tumor, you're going to do what in terms of your operation. So we definitely need our standard pelvic washings. Then we examine the abdomen looking for anything that's suspicious. But here's where the difference comes in between germ cell and epithelial tumors. Um you're going to do an omentectomy, you're going to remove your primary tumor. Um generally, again, you want to spare the tube if you can. You need to actually do a lymph node dissection and where do you think the boundaries of your lymph node dissection should be? Pelvic structures, those drain to pelvic lymph nodes, so peritic from the iliacs to the renal artery, that seems like a pretty reasonable place to start. Right, so think about where it's a pelvic organ, right? So it's it's going to drain to the iliacs and below the renals. So you want to do a dissection up to that because we know that about 30% of nodes that look normal that you're feeling in epithelial ovarian cancer can be positive and that will will upstage you. In adults, surgery can get kind of radical, including a hysterectomy and a bilateral salpingectomy. However, in children, fertility sparing is an important consideration because in kids, well, they do pretty well. There are something called borderline epithelial tumors. Basically, they're more common in kids, they have a better prognosis than invasive ovarian cancer. They are they are again have the serus and mucinous types and they can recur. So it's really important that when we see a borderline tumor, um one of the things that you can try and do is do a ovarian sparing surgery and then if you go and you get pathology back and it's a borderline tumor, you can do one of two things. You can go back and take the ovary out or you can watch them serially, but you have to commit to really following these patients very closely um over many years because these tumors can come back. Okay, that was a lot of ovarian tumors. Don't worry. Dr. Dasgupta is awesome. And so she put together a list of all the types of ovarian tumors and their relevant tumor markers. I'm sure she's accepting thank you notes, but until then, we've just put the table below the media player. So check it out. Um these are a sort of list of the types of tumors and the tumor markers that are positive for. So this is a good thing to sort of memorize. You guys can us send this to everybody. Um but I think what you need to understand is there's no perfect tumor marker. Um studies that have come out of the MWPC in the last couple of years have really told us that it's the panel of tumor markers that are are sort of helpful, but not not not one perfect tumor marker and not one perfect way to predict malignancy in an ovarian mass. Plus, at Cincinnati Children's Hospital, there's a new protocol detailing how best to investigate ovarian masses, including imaging recommendations, oncology involvement guidelines, and a laboratory workup. And yes, it's linked below. Thanks for joining our episode on ovarian pathology with Dr. Dasgupta. Remember to download the Stay Current app. Check us out on YouTube, Facebook, Twitter, leave us a comment wherever you listen to the podcast. But until next time, I'm Britney and remember, knowledge should be free.