 نحن نعود إلى أخر محاولة This is a 62-year-old female who presented with blood in the urine Initially she was treated for a bladder infection but the bleeding persisted so she was evaluated further and was found to have kidney cancer we'll look at images in a minute but her past history she has reflux symptoms, diabetes and she is in excellent health otherwise she has no symptoms related to cancer but the laboratory investigation shows this is a blood test called Lactate dehydrogenase when it's elevated it indicates a high tumor burden the upper limit of normal in our lab is 618 so this is more than twice higher than the upper limit of normal this is a function of the filtration of the kidney it's called Estimated GFR Glominal Filtration Rate as we heard this morning the normal is 60 and above this is clearly reduced filtration rate so she has compromised kidney function but the rest of her labs are within normal limits and there is no evidence of metastatic disease to bone or brain by these imaging studies and here is her scan and you can see a very large mass arising from the right kidney and what would you do, Dr. Karam? the mass looks unusual in its configuration and you can see part of the right kidney it looks like it's de-vascularized almost the lower part and it's replacing the actual kidney itself and there's also some enlarged nodes behind the vena cava and between the vena cava and the aorta and I can't tell what's going on with the adrenal yet but it is a bit unusual and this is someone I would probably recommend a biopsy before we proceed with any further therapies Dr. Matee do you recommend there is no metastatic disease? yes, so I think that biopsies is an intriguing option and I think reasonable for the reasons Dr. Karam mentions the thing is you think about doing surgery and you're going to remove all visible disease so why not? the problem is when it's in the lymph nodes and if it's clear cell it's not really going to be curative if it's something else or if it has sarcomatoid then even less so so I mean I don't want to get overly technical in terms of all those different things but I actually think biopsy is pretty reasonable okay well this is her katskin the chest and she has here enlarged lymph nodes but she does have metastatic disease so there are enlarged lymph nodes in the chest and again here is we see the mass in the right kidney now with this information large mass in the right kidney enlarged lymph nodes near the right kidney and these lymph nodes in the chest how would your management change? Dr. Matee I'd feel even better about doing a biopsy okay Dr. Wood do you agree? or would you go straight to surgery? I actually don't agree I think that again the vast majority of this patient's disease is in her abdomen and is surgically resectable I'll buy you know it would be a big surgery she has an excellent performance status there's really no contra indications to doing surgery and so I would go with a right radical nephrectomy and resect the nodal disease that's present in the retroperitoneum and allow her to recover and reassess and then decide on systemic therapy based on the histology of the primary tumor okay we'll hear it from the medical oncologist Dr. Pile would you do a biopsy as recommended by Dr. Matee and Dr. Karam and then how would that change your approach? I don't think it's right or wrong but I would probably go without the wood to do the surgery I don't I mean I would always say whether this is a urethelocarsinoma or other more rare type of kidney cancer but you know primarily the tumor isn't a kidney and this patient is relatively symptomatic I would take the kidney out Dr. Harrison you agree so first? yeah I agree I mean there's always the concern it's some unusual histology but I agree with that I would recommend you to correct me and resection of all the lymph nodes that are visible at the time of surgery the pathology consistent with what you suspected a rare kidney cancer that's called translocation carcinoma of the kidney we refer to it as XP11 .2 and you know we know she has metastatic disease that's the M1 the nodes the maker M1 and the T3A the positive for metastasis tumor involvement with extranoidal extension now what do you recommend Dr. Pillay you suspected she may have rare tumor would your systemic therapy now be influenced by the pathology Nizar could you just explain the importance of or what the significance of extranoidal extension is for the patient yeah extranoidal extension of lymph nodes is worse carries worse than if the nodes are still confined so even though she had a resection of all the lymph nodes that carries a worse prognosis Dr. Pillay the pathology now indicating this rare tumor type does that influence your selection of systemic therapy this is a challenging case because this is a really very rare kidney cancer and we don't know what's the best option for the patient of course a surgery is probably the best option but that's it hasn't really solved the issue here there are some I believe some case reported about them to in Hebrews in this in this kidney cancer type but I would because we don't know how to treat it I'm a little bit positive about this patient she's 62 usually we do see this in younger in younger patient and more more visceral metastasis than lymph node metastasis but it is what it is so I was still probably treated with the drug that we have for advanced kidney cancer chemotherapy sometimes the disease is growing very rapidly and it has involvement in the leave of the lungs I would treat it with standard drugs Doctor Harrison is there a role for RL2 here in this lady with this tumor type I think it's unknown but I doubt I wouldn't use IL2 so as the panelist mentioned this is a rare kidney tumor but now it's estimated that 1% of adults with renal sarcarcinoma have this type so when you do what we call FISH on these tumors you diagnose the translocation in 1% so it's still rare 1% is rare IL2 is not recommended for patients with non-clear cell types but only for the clear cell so I agree I think how those IL2 is not an option here and the prognosis of these tumors if they're older and Doctor Pillay made the point that these are usually seen in pediatric ages adolescents and young adults and rarely in older people they do present with bulky disease and lymph node metastasis and they are treated as we treat other types preferably on a clinical trial and we have a clinical trial at MD Anderson for non-clear cell as well as the Duke group and we have a study for these patients they're calling it the ASPEN trial and it's looking at treating those patients into two groups one group with Evrolimus an employer inhibitor and one group with synitinib and we have the same trial we call it ESPM trial and it's Evrolimus versus synitinib again for these patients with these rare tumors but we always go to研究 but we will know what we want to work on is that we can come up with new videos and watch the content and will add new videos لا أعلم إذا أعلمنا if we know the answer to that at this point. Well, I mean that's the challenge is that this is such a rare histology that, you know, we can't look at the last 500 patients that had translocation tumor because we don't have 500 patients. And, you know, I think in the absence of, you know, effective systemic therapy that, you know, radical tumor debulking represents this patient's best chance at having some sort of prolongation of life. I think if you look, if you extrapolate from the pediatric literature, translocation in young adults or pediatrics, children, the approach has been, before all these target therapies came to existence, was to do multiple resections. The resection is obviously nephrectomy and then removing lymph nodes and continuing to go on and doing successive resections. And these patients live many years and the prognosis is better when you're able to perform surgery. So in this, in her case, it is, you know, she's lucky. I believe that she presented with resectable disease at the time of initial diagnosis that she's able to get the bulk of her disease out.