on Friday evening, where I could give a lecture on ovarian tumors in children. So I thought long and hard, but I can never refuse a joke. So I decided to rustle up something. It's going to be a short lecture. It's not going to be a detailed lecture on all the childhood tumors. I thought we would approach it in a practical way. So I'm going to share my screen now. So this is a 15 minutes lecture on practical management of ovarian tumors in children. And the agenda is going to be, I'm going to discuss a little bit about epidemiology of ovarian tumors in children, followed by germ cell and stromal tumors in children, essentially the clinical manifestations, diagnosis, tumor markers, pathology, and staging and treatment. And finally, a small clinical scenario of what you would do if you're called to OR. As far as epidemiology is concerned, epithelial tumors are very rare in premenarchal girls. And germ cell tumors are far more common. And stromal tumors are fairly common. In one of the largest studies reported on ovarian tumors in young girls, about 253 patients, below the age of 14, only 2% were epithelial tumors. 80% were germ cell tumors, and about 18% stromal tumors. Between ages of 15 and 19, more than half were germ cell tumors. And 20% was stromal, and only about 30% were epithelial tumors. So in general, when you're dealing with a young patient, young girl, and here I'm only going to talk about young girls. I'm not going to talk about the adults. Think of germ cell tumor first, and then stromal tumors next. Epithelial tumors would be right at the bottom of the list. Because it's important to realize, because the management is affected by your thought. As far as clinical manifestations, patients typically present with one or more of the following. Pain, either from torsion or rupture. Torsion is more common, because they're usually heavier tumors. Or they may come in swelling, from the mass itself. About 80% of the patients actually present with a pelvic abdominal mass. And sometimes they also may present with ascites. Now, if they have high HCG levels, they may present with precocious puberty, with abnormal vaginal bleeding. And in general, these tumors tend to be quite large. And in the serious reported, the median size was about 16 centimeters. So they're almost always easily palpable per abdomen. How do you diagnose? Now, diagnosis is very strongly suspected by the age of the patient, presence of the pelvic mass confirmed by imaging, and elevated levels of associated tumor markers. Later, I'll show you a slide of what are the markers that are important. Essentially, they are alpha-beta protein, lactic dehydrogenase, and human chorionic gonadotrophy. And the final diagnosis is always by histology, after removing the mass. You cannot have an impression and start treating the patient. For benign cystic teratomas, which are not common in the young girls, pelvic ultrasound may be quite diagnostic, but cystectomy and histological confirmation is necessary. Now, this is a busy slide, just shows the various tumor markers that may or may not be present. But I've highlighted the positive ones by the red rectangle. For dysgerminoma, the usual marker is the LDH. For embryonal carcinoma and choriocarcinoma, it's HCG. For yolk sac tumor, it's the alpha-beta protein and LDH. When it comes to sex cord stomal tumors, granulosa cell tumors usually have high levels of inhibin and AMH. And sex cord tumors present with high levels of E2. But the absence of these markers does not exclude the diagnosis. So histology is paramount in all these patients. And very quickly, the pathology, you can see that there are three layers in ovary, the surface, the stroma, and then the germ cells. And today we are just going to talk about the germ cells and the sex cord stomal tumors. They can be benign or malignant. I've highlighted the benign ones in green, which are essentially teratomas and the germ cell tumors, and the thichomas and fibromas in the sex cord stomal tumors. The rest are malignant. And I'm not going to talk about epithelial tumors at all. Now, staging. This is a big area of controversy. First of all, germ cell tumors and stromal tumors are staged in the same way as fecal staging for epithelial ovarian cancers. In summary, stage one is confined to the ovaries. Stage two is central to the other pelvic tissues. Stage three is extension beyond the pelvis or retroperitoneal nodes. And stage four is distant metastasis or liver parenchymal involvement. Now, the extent of surgical staging is very, very controversial because these tumors are actually extremely chemosensitive. So there is big controversy as whether we need to subject that patient to the morbidity of a thorough staging in these patients. The utility of omentectomy and lymphadenectomy is debatable and very often is omitted. In the pediatric intergroup study by the Pediatric Oncology Group, surgical guidelines were only adhered to in one out of 56 patients. That means they did not do a full, thorough surgical staging yet 95% of the patients were long-term survivors. And in the Italian MITO study, incomplete peritoneal staging was a risk factor for recurrence but no impact on survival because they're extremely chemosensitive. And in the CA dataset from US, lymphadenectomy had no impact on the overall survival. So coming back to the summary of the treatment, first of all, in germ cell tumors, extensive surgical staging is of natural benefit because of the sensitivity of these tumors to the chemotherapy. Again, I'm talking about younger girls. Fertility-sparing approach is a standard of care in young patients. And unilateral salchyngo-oophorectomy with preservation of the uterus is a treatment of choice even in apparent advanced cases. Say you go in and you find lymph nodes are involved, it still means you've got to be conservative in these patients because the lymph nodes can be taken care of by chemotherapy. Sampling of the normal-looking contralateral ovary is not recommended as postoperative adhesions are common and may impact fertility. And most of these patients present with unilateral tumors. Bilateral tumors are uncommon in germ cell tumors. And here's where the treatment varies between the adult oncologists treating germ cell tumors and the pediatric oncologists treating stem tumors. We were always, the gynecologists have always been taught all germ cell tumors other than stage 1A dysgerminoma and grade 1, grade 2 immature teratoma will have chemotherapy. But the pediatric oncology group have shown that all stage 1 regardless of type or grade can be observed. So that is the second column in the pediatric recommendation by the pediatric oncology group. But the adult oncology group in United States and elsewhere all around the world, they only treat stage 1 dysgerminoma and immature teratoma grade 1 with observation. All the rest get chemotherapy. We don't know whether this is the right approach. In fact, there's a very large international study that's occurring now where they have randomized it to observation versus chemotherapy in stage 1 tumors. We'll have to wait for the results of this trial before we decide which is the best way. Now with stromal tumors, the most common stromal tumor in young girls is juvenile granulosa cell tumor. They usually present in early stage and unilateral salpingo-oophorectomy is recommended. And an endometrial biopsy is recommended when the tumor is a granulosa cell tumor because the production of hormones. No adjuvant chemotherapy unless advanced disease and long-term surveillance is important as the recurrences can occur several years later, 15, 20 years later. So as to the case presented earlier, I would suggest that she would get a unilateral salpingo-oophorectomy and just observe. There is no need for any biopsy of the momentum lymph nodes and no chemotherapies needed. So I'm just going to present a clinical scenario. A 16-year-old girl has an emergency laparotomy for a suspected ovarian torsion. And on opening the abdomen, the operating gynecology resident finds a 10-centimeter solid-looking right ovarian mass. She calls for assistance from a friendly gynecological oncologist, which happens to be you. No preoperative imaging or tumor markers have been done. So what would you do? Maybe Binod can answer that shortly, quickly. What would you do? You're called by your resident. Hello? Binod? Hello, Binod? Sanjaya? Sanjaya, what would you do, Sanjaya? Yeah, Sanjaya, what would you do? A right ovarian mass, no tumor markers, no imaging. First, I'll do the detailed clinical workup. No, nothing. She was operated as a semi-emergency for pain, suspected torsion. You open up, and she found this right ovarian mass. She's a 16-year-old girl. So we know we get this kind of patient often with the G1 oncologist. A general gynecologist do a surgery and refer to you and ask you, now that you have granulosis, cell tumor, everything else looks great. What's our next step? I'll ask for a consultation with the senior one, if possible. Okay, and if you are the senior one, what would you do? The patient is on the table. In that case, I'll do, as sir presented, earlier, I'll do a unilateral sacrum rectomy. Yes. And look for any deposits around. Yes. If there are any omental deposits or anything, any enlarged lymph nodes or anything. Right. If there was ascites, or I do a Mediterranean WASP for cytology as well. Yeah, and then wait for the histology to decide what you want to do. Okay, very good. Okay, this is what I would, I just had a suggested plan of action. First, examine the tumor very carefully. Now, if it is mainly cystic, a cystectomy would be all right. She's 16 years old. Now, remember, whenever in doubt, be conservative, because you can't put back anything. It's very easy to remove things, but not put back. So if it's a mainly cystic tumor, I think it's justified to do a cystectomy, but if it's completely solid, you would do a unilateral sacrum oophorectomy. And the most important thing in the operating theater is a frozen section, because that would tell you what you need to do further. And if it is benign, that's end of your operation, you just observe. If it is malignant, then you do an exploration as you suggested, look for metastatic disease. And then once the histology comes back, you can stage the disease and tumor markers would be done if it hadn't been done earlier. And then you have all the information. Once you've got a stage and a histology, you can decide what you want to do further. All right? So that is in a nutshell, what I would do in a patient who I'm just called upon suddenly with no other information. So be conservative. And when in doubt, just remove the ovary and do nothing else. Wait until the histology is ready. All right? I'll stop there. Any comments or any questions? I think that was really good, Professor Ilan Charon. I have a few cases presented at our tumor board with gynecologists did the cystectomy during the cesarean or other times they didn't send her frozen. Now the patient is here in my office. The discussion is always, should we take her back to do the salpingo-oophorectomy now that she had a cystectomy? And what's your experience on that? I think with a germ cell tumor, I think it should be a unilateral salpingo-oophorectomy. There's no place for cystectomy in observation. And nowadays with minimally invasive surgery is so prevalent, you can always take her back to the theater and do a unilateral salpingo-oophorectomy. I would be very careful just to manage her with cystectomy alone, knowing that she has a germ cell tumor.

ovarian tumors

children

practical management

germ cell tumors

stromal tumors