Nasal vestibular carcinoma

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scarbrtj

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This is a case that checks disparate boxes and is rare. Thus it makes it a little confusing.

59yo male in excellent health. Non smoker. No drug use. Perhaps 40yo ago had some sort of injury and broke nose and cartilage was exposed or something like that. Never quite healed for a while, in vestibular region. About 6 mos ago had a right neck node suddenly appear. Asymptomatic. Had a simple but complete excisional biopsy of that by community ENT. Was SCC p16+. N1, level 2, maybe 3. No adverse features. Very negative margins. Extensive hunt for primary undertaken including bilateral tonsillectomy, endoscopy. PET negative. Exams negative. No primary ever found.

I saw him at that point. Essentially T0N1 unk primary. I recommended observation. Academic center recommended completion dissection and XRT to necks and oropharynx in standard unk primary style; I even think chemoRT was recommended.

He went with observation.

Following w ENT he soon had a “sore” in nasal vestibule on right come up. Treated for staph for about 2 mos. Began growing. Was biopsied. Is a p16+ SCC of nasal vestibule. I’d put its size on palpation at 2cm and I feel a little fixation. It’s non painful and not bleeding. All other exams negative. Neck well healed. I don’t have any imaging yet.

I have a clear idea about what I want to do: chemoRT w cis basically, necks to 45-50 and primary to 70. But I am wishing to know what others might do. I also like “connecting volumes” and getting the nasal vestibule to connect up with neck contours seems like I might be treating some face. Also think level 1 is in order.
71E1B71D-0346-48DF-B72C-052BBD1C0568.jpeg

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My 2-cent thinking:

1. PET-CT for staging.

2. Do endoscopy to r/o nasopharynx ca, sometimes nasopharynx ca comes out of the nostril.

3. This tumor is notorious for facial LN involvement. Check his facial LN area.

4. I'd favor chemo-RT using weekly CDDP at 40 mg/m2. Not a big fan of CDDP at q3wk (toxic).
Target volume: primary site, facial LN, both necks.
It will be tough on the pt.

The P16+ is helpful in terms of prognosis.
 
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If PET negative for nodes and another primary, pan endoscopy negative, would anybody consider brachy? Good series out there on nasal vestibule carcinoma with excellent results and cosmesis. Nasal vestibule carcinoma are treated like skin cancer, and use Wang staging. Skin cancer can be p16 positive but HPV PCR negative. It might be worth checking the PCR out of curiosity.



 
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Agree w/ Radonc90 if he has new nodes/extensive invasion. I want to know how posterior it goes and whether sinuses/hard palate etc. are involved and better understand where tumor may have originated. I'd prefer an MRI in this case to be more confident in extent of tumor/invasion and feel more confident that there isn't unappreciated PNI, etc (to supplement the PET/CT).

p16+ doesn't mean much here without it also being HPV+; a lot of tumors are p16+ (including skin cancer) without HPV infection. If the picture is the main extent of the primary disease, this can be thought of like a squamous skin cancer in that location (like carbonion said). That area receives sun exposure.

ChemoRT with broad elective coverage makes sense especially if there is nodal disease on restaging. If it's been many months and only disease is local, one could make an argument for local-only treatment at this point with the caveat that there is a risk to this, whether that be further RT or surgery or both. His node+ neck has already been observed and if continues negative, should give some additional confidence in local-only treatment.

Regarding concurrent chemo- I would withhold chemo considering this is similar to a skin cancer/ there is only 1 node involved/early T (T1-2N1). Also see: Radiation therapy for nasal vestibule squamous cell carcinoma: a 40-year experience

On treatment, obviously need to check that the area is getting enough dose.

So: Small, local only disease at this point, treat primary site +/- face/neck. No chemo. Extensive disease/new nodes, chemoRT.
 
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If this is an HPV-linked tumor, I'd like to know how it got up there... Is this the result of common sex practice where you practice? :happy::happy::happy:

Other than that:

1. Surgery could also be an option. It will probably be mutilating, given the size of the tumor, so I would not favor it. On the other hand, 70 Gy on the nose may also lead to cartilage necrosis (Any idea what chance? I'd presume 10-20%, depending on comorbidities - diabetes, cardiovascular and bearing in mind that it's already 2cm in size). So, just to avoid any issues later, I would still have the patient see a surgeon and hear what Plan B would include. You do not want him coming back to you arguing he could have had surgery upfront, if/when his nose drops off his face 6 months after 70 Gy.
2. 70 Gy to the primary site seem reasonable, but see point 4.
3. 50 Gy to the neck make sense. Would you dare to stay on the ipsilateral side if you do not have clear signs of the tumor crossing the mid-line (although I presume it probably is doing that, given its size)? I am not aware of any data. You can do that for tumors of the maxillary sinus but not for vestibular nose tumors, right? You are going to fry both parotids, if you treat bilaterally comprehensively, or you may decide to place a hard constrain on the left side. I'd treat retrospharyneal nodes too.
4. Cisplatin either weekly or every 3 weeks are both valid options given you are treating gross tumor and N+ disease, even if it's pN1. I agree that one could opt to ommit it, but: I'd give it actually and perhaps try to cut back a bit of dose to the primary instead. Perhaps 66 Gy if HPV+ and concurrent chemo-RT would suffice. That may limit the risks to the cartilage in terms of necrosis. Not a validated strategy, but perhaps something to consider.
 
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- Surgical consult at academic center today
- MRI Friday

will update
 
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My 2-cent thinking:

1. PET-CT for staging.

2. Do endoscopy to r/o nasopharynx ca, sometimes nasopharynx ca comes out of the nostril.

3. This tumor is notorious for facial LN involvement. Check his facial LN area.

4. I'd favor chemo-RT using weekly CDDP at 40 mg/m2. Not a big fan of CDDP at q3wk (toxic).
Target volume: primary site, facial LN, both necks.
It will be tough on the pt.

The P16+ is helpful in terms of prognosis.
agree with the above. Have not made up my mind about q3weekly cisplatin. It definitely seems better tolerated now than 10 years ago. Would add that MSKCC treating neck to 30 Gy with chemo in HPV+ pts. Would consider going at 150 cGy (which was used on rtog) / 200 cGy (gross disease with margin) Dont think that would be so toxic
 
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Would add that MSKCC treating neck to 30 Gy with chemo in HPV+ pts.
p16+ Is not the same as HPV+ (although they are correlated- especially so for nonsmoker/oropharyngeal). Essentially all cancers can be p16+ but that’s not a reason to trim dose. This disease is more like a primary skin cancer than hpv+ orophayngeal.
 
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The p16 positivity is a red herring. This is a skin cancer, folks! The options if negative nodes and no other primary identified are

1) EBRT to 66-70gy (Option 1/2 can be with or without elective coverage depending on risk tolerance. Keep in mind most patients are salvageable)
2) EBRT to 66-70 with chemo, could consider immunotherapy (50 pct response in metastatic setting in skin cancer). Some may cite recent data showing chemo is actually detrimental to skin cancer treatment with XRT.
3) interstitial brachytherapy or Intracavitary mould technique (linked above)
4) surgery, likely disfiguring hack job

If +nodes: chemoxrt vs xrt alone, can comsider IO again

BTW this is an excellent board case to teach learners.
 
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p16+ Is not the same as HPV+ (although they are correlated- especially so for nonsmoker/oropharyngeal). Essentially all cancers can be p16+ but that’s not a reason to trim dose. This disease is more like a primary skin cancer than hpv+ orophayngeal.
we know that p16 is favorable in oropharyngeal, and anal and cervical cancer, and vulvar.

Other cancers like serous endometrial p16 may accumulate, but not due to hpv infection and it doesnt carry the same favorable prognosis. Extra-oropharyngeal head neck cancers, I dont think p16 carries favorable prognosis.
 
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we know that p16 is favorable in oropharyngeal, and anal and cervical cancer, and vulvar.

Other cancers like serous endometrial p16 may accumulate, but not due to hpv infection and it doesnt carry the same favorable prognosis. Extra-oropharyngeal head neck cancers, I dont think p16 carries favorable prognosis.

some evidence in EBER+ p16+ nasopharynx
 
I know this is crazy talk but are we 100% sure that this isn't two different situations (unknown primary mucosal site and also a skin cancer)?

 
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I would certainly consider surgery... especially if you are friendly with (and trust) surgeons at a high volume center. Reconstruction may be feasible if timed appropriately with adjuvant RT, but may not be so easy if the patient develops necrosis down the road after definitive CRT. This route is only feasible if you can trust your surgeon to forgo an operation if the patient is likely to end up disfigured (which clearly may not be the case).
 
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Agree that P16+ is a red herring. Can get HPV pCR to confirm. What's the time interval between H&N Unknown Primary and new nasal vestibular SCC?

Agree with MRI and consideration of PET/CT, along with endoscopy, to rule out Nasophx/Sinus primary.

Assuming that the picture is all we see (no involved nodes) and he has at least say 6-12 months between his HNSCC UnkPrimary and this, would favor 60-70Gy, RT alone, to just the local area + margin.

No compelling data for concurrent chemotherapy in skin cancer. I don't like Cis. Some like Cetux, I don't.

Some sprinkle in some Cemiplimab in this scenario, which is probably not good either (@thecarbonionangle can you link whatever you have on Cemiplimab + RT resulting in worse outcomes?) but we don't have "data that it's not good" and it's what med-oncs get excited about.

Surgery not unreasonable but need a very good description of what ENT is planning. If ENT is planning surgery without doing additional imaging, would not let ENT cut on him. This is regardless of whether it is academic or community ENT.
 
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@evilbooyaa What i meant is that the chemo data in skin cancer is generally not good. There was a recent paper in skin cancer using chemotherapy in the adjuvant post op setting which is more negative data for chemo in skin. I was not talking about cemiplimab being detrimental. I re-read what i wrote and thats what i meant.


Counter Is that it is a “bad” study because they used carboplatin
 
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Per OP, it seems like time from H&N unknown primary to nasal vestibule mass was ~4 months, so patient can be considered T2N1.

If this is skin cancer (p16+, HPV-) and PET/CT is negative for distant disease,, I would treat with RT (+/- chemo since this is skin, + if he has HR path features) to 60-70 Gy to primary and 54-56 Gy to ipsilateral neck. My reasoning for ipsi neck radiation is because he has not had a R SLND (which he should have since he has N1 disease).
 
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Let me add: “about 6 mos” ago the node did appear, but 4-5 mos ago would probably be more accurate. It was removed 4-21. The “primary” began bothering the patient by May 1. Assuming the primary is the nasal vestibule.

To BobbyHeenan’s point... I know of no convincing way (to convince me!) that I can place the node and the vestibule in separate boxes. The pathologists tell me they appear to be similar, but they just have a small amount of tissue from the vestibule right now.

I feel a surgery leaves patients like this in a state where you’re talking prosthetic nose. We will see what surgeons say.
 
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Let me add: “about 6 mos” ago the node did appear, but 4-5 mos ago would probably be more accurate. It was removed 4-21. The “primary” began bothering the patient by May 1. Assuming the primary is the nasal vestibule.

To BobbyHeenan’s point... I know of no convincing way (to convince me!) that I can place the node and the vestibule in separate boxes. The pathologists tell me they appear to be similar, but they just have a small amount of tissue from the vestibule right now.

I feel a surgery leaves patients like this in a state where you’re talking prosthetic nose. We will see what surgeons say.

that is why the PCR may be helpful:

node= p16+, HPV PCR negative
Nasal vestibule: p16+, HPV PCR negative.
Then perhaps same source but if
node= p16+, HPV PCR+
Nasal vestibule: p16+, HPV PCR negative.
Then Separate angles, perhaps.
 
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that is why the PCR may be helpful:

node= p16+, HPV PCR negative
Nasal vestibule: p16+, HPV PCR negative.
Then perhaps same source but if
node= p16+, HPV PCR
Nasal vestibule: p16+, HPV PCR negative.
Then Separate angles.
Clever. I’ll see if we can try that
 
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Clever. I’ll see if we can try that

Fair enough. Very short time frame. Could be an aggressive skin leading to nodal disease.

Carbonionangle's point is well taken, but I'm suspicious that this is actually a skin cancer with nodal spread given the short temporal relation.

I think in that scenario I would favor more comprehensive treatment, including primary + margin and right facial/neck disease. I feel that because of the fact it's along the medial aspect I would worry about microscopic contralateral disease as well, but not sure how high of a risk contralateral nodal disease is in a skin cancer (although this is maybe a skin cancer that's more aggressive since it already had nodal mets?).

Could have a discussion with patient about +/- for left neck coverage. Could also have a discussion of +/- for concurrent chemotherapy. Again I'd probably favor something like concurrent cempilimab.
 
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Clever. I’ll see if we can try that
I think it is very likely to be hpv related. Get papillomas in the nasal area. Would not suggest surgery as very likely would need xrt after surgery anyway. If he is young and healthy, dont see much of downside to cisp. Not going to have much mucosal irritation or swallowing dysfunction, especially if use low dose of elective xrt to neck and facial nodes.
 
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HPV-related multiphenotypic sinonasal carcinoma is a relatively recently described entity of a complex pathologic picture - salivary gland and squamous differentiation


Worth review by a head and neck pathologist. Agree with endoscopic exam, MRI imaging to assess extent and evaluate nodes. Consider PET/CT or PET/MRI (I have started to really like these but getting them insurance approved is a PITA). Challenging area for definitive RT due to cartilage necrosis - facial nodes are a bear to treat but certainly at risk as well.
 
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Unusual order of events and interesting case. Would make a great teaching case. Maybe I missed it, did he get a completion neck dissection proving he was truly N1 (i.e. single node). That would be what my original recommendation would have been, though if still pN1, observation makes sense.

Easier to think of it as a skin cancer. p16 is a red herring, can be positive in skin cancer but have nothing to do with HPV. Absolutely do not extrapolate from oropharynx in this case.

Surgery can likely be done with reconstruction, but cosmetically may not be a great outcome. If being done, would recommend ipsi neck dissection. Assuming still cN0? Given young age, single node (that we know about), and associated toxicity, would spare contra neck for now unless multiple positive nodes on ipsi neck dissection.

If he decides to go with primary RT (very reasonable), would treat to 60-66 @2Gy/day, and prophylactic dose to ipsilateral buccofacial/levels 1B-3 to 50 assuming he didn't have a completion neck dissection at the outset.

Make sure a thorough cranial nerve exam is documented. Have seen PNI at V2 missed since it isn't always part of the routine exam. Probably wouldn't get a MRI if asymptomatic.
 
FYI: Used to have head and neck and skin service. Never saw cartilage necrosis. Probably about as common as humeral head necrosis or rib necrosis(with photons not protons) in breast cancer. Skin around nose and anus probably subject to hpv infection but just speculating here, based on propensity to develop papillomas. Reasonsable that you have prophylactic low dose level back to ipsalateral pterygopalatine fossa (cross roads of all the nerves) which really wont carry much toxicity .
 
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Unusual order of events and interesting case. Would make a great teaching case. Maybe I missed it, did he get a completion neck dissection proving he was truly N1 (i.e. single node). That would be what my original recommendation would have been, though if still pN1, observation makes sense.
Yes it was a little outside the box to observe. However the ENT described a very encapsulated node (he thought it was benign visually). The PET and CT negative. Exam negative. Tonsillectomies and scopes all negative. If I had treated, I would have given elective RT to the oropharynx. I am glad in retrospect I didn't treat; imagine the headache trying to match fields now (and/or overlap, yuck). I thought a completion neck dissection showing no more nodes would have not changed my recommendation and shown me what I already "knew." And if positive, I was thinking it should declare itself clinically soon and close observation would have been OK.
Assuming still cN0?
He is.
Given young age, single node (that we know about), and associated toxicity, would spare contra neck for now unless multiple positive nodes on ipsi neck dissection.
Honestly I will probably treat both necks. On exam I have a little suspicion about midline crossing. Will see what the MRI shows. The MRI I think is a little helpful too for RT planning (we'll fuse it). The more imaging the merrier for contouring. Another PET I'm not doing. We had a negative one right after the node was removed which was just only about 3-3.5 mos ago now.
If he decides to go with primary RT (very reasonable), would treat to 60-66 @2Gy/day, and prophylactic dose to ipsilateral buccofacial/levels 1B-3 to 50 assuming he didn't have a completion neck dissection at the outset.
Same (except for ipsilaterality). Although ~70Gy to primary is my goal. I also am still leaning toward some weekly CDDP.
Make sure a thorough cranial nerve exam is documented.
Totally negative exam there.
 
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What happened to this guy?. I’m still waking up thinking about case.
 
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What happened to this guy?. I’m still waking up thinking about case.
After many, many... things... he decided surgery route. This happened ~2 weeks ago. I do not know the visual outcome on that yet but seems to be pT2 (can't recall PNI or LVSI status, or p16). I am sched to see him in clinic soon. Academic ctr decided still to do obs on neck/let XRT handle it. More to come, I promise you a thorough followup.
 
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Patient returns to clinic today. Approx 5-6 weeks out from surgery. ECOG PS 0, still actively working etc. Over last 24-48h a hard "knot" has arisen in L neck. Initial disease as you can see above was small R neck node and R nasal vestibule disease. Here is the current clinical exam:

Y3m22XE.jpg


And the plan:

6JNUAOu.jpg


I don't feel a need to biopsy the acutely new L neck node or send him back to surgery, for various reasons.
 
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Original question:
Should the patient get surgery or radiation for definitive management?

The cancer:
1602011693062.png


Godspeed. The pre-surgery MRI didn't show any LNs in L neck? This is aggressive af for a skin cancer. Still like my original Cemimplimab + RT suggestion.
 
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Original question:
Should the patient get surgery or radiation for definitive management?

The cancer:
View attachment 319901

Godspeed. The pre-surgery MRI didn't show any LNs in L neck? This is aggressive af for a skin cancer. Still like my original Cemimplimab + RT suggestion.
It did not. As I said the fellow was like this lump in my left neck just came up overnight almost in last day or two. I counseled him extensively about the prognosis which is progressively seeming more dire, I agree.
 
Great case though sad outcome. He should have had a completion neck dissection at the time of his surgery (easy for me to say now). But excis biopsy only for positive node, and then definitive surgery, going that route - do the neck. Don’t understand that decision (which wasn’t yours).

ct his neck and chest now And I would recommend bilateral necks if he’s not metastatic followed by RT. but no right answers here
 
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Great case though sad outcome. He should have had a completion neck dissection at the time of his surgery (easy for me to say now). But excis biopsy only for positive node, and then definitive surgery, going that route - do the neck. Don’t understand that decision (which wasn’t yours).

ct his neck and chest now And I would recommend bilateral necks if he’s not metastatic followed by RT. but no right answers here
Getting a PET CT ASAP. Are you suggesting neck surgery followed by RT. I had considered but I don’t like the possible delays involved. And he initially presented with that N1 *right* neck unk primary. I am sure they would have dissected the right neck only. Whether that would have prevented a left neck recurrence we have now who knows. And yes at this point all I have done (decision wise) is observe and punt on initial RT for what looked like a favorable N1 unk primary right neck. (When he came back later with a lesion in his nose it felt like the best decision I had made all year.) The local ENT didn’t do a right neck dissection then. And then at the academic center they did the above “large whack” and still didn’t touch either side of the neck.
 
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All your decisions were reasonable and defensible. Not performing a neck dissection at time of primary surgery for known node positive by excis biopsy, regardless of imaging, may not be defensible but was outside your decision making.

Could all be disparate events. But the supposition is that he had/has microscopic disease in other nodes, even on an ipsi dissection, and would have prompted maybe bilateral RT then. Or it could have missed all of it, but that was the decision with the greatest chance of changing things. And he could still be fine and salvaged now. But N1 excis and negative pet is not equal to N1 post ND.
 
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The patient contacted the academic center. They want to bring him back in for either excision or neck dissection; the feeling I get is excision. Surgery will be 10/16. Then the center requested I start radiation "two weeks" after that. PET/CT last week showed ~3.5 cm L neck node, about level 3, SUV 8.5. No other uptake anywhere identified, including nasal (operative) region, or R neck (original site of disease).
 
He needs a neck dissection not just an excisional biopsy. But don't disagree with surgery first in a proposed skin cancer.
 
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If an excision all biopsy is done as the only step, I would strongly recommend referral to another surgeon. Sorry for unsolicited advice which carries no burden to me, but feel strongly ND followed by RT is this persons best treatment. There is no data to support CRT as a replacement in skin cancer, though it is done.
 
If an excision all biopsy is done as the only step, I would strongly recommend referral to another surgeon. Sorry for unsolicited advice which carries no burden to me, but feel strongly ND followed by RT is this persons best treatment. There is no data to support CRT as a replacement in skin cancer, though it is done.
At this point, stuck. The patient is calling the shots. And the surgeon is the leading surgeon at the academic center. I never made the referral as it were in the first place. He started with a local ENT and then sought out the academic place on his own.
 
To recap, original disease R neck node “CUP” (exc biopsy only) and then R nasal vestibule primary showed up sometime later. Resected, reconstructed. Then L neck node appeared very suddenly some time after that. Two weeks ago: L neck diss. 5/31 positive, biggest 4cm with ENE. Plan, now, is for chemoRT; RT in a style/target volume outlined earlier this month. Easily my most challenging case of 2020, high stakes, young patient, very technical, etc. And I haven’t even beam on’ed yet.
 
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To recap, original disease R neck node “CUP” (exc biopsy only) and then R nasal vestibule primary showed up sometime later. Resected, reconstructed. Then L neck node appeared very suddenly some time after that. Two weeks ago: L neck diss. 5/31 positive, biggest 4cm with ENE. Plan, now, is for chemoRT; RT in a style/target volume outlined earlier this month. Easily my most challenging case of 2020, high stakes, young patient, very technical, etc. And I haven’t even beam on’ed yet.

A tough case for the smartest of us

You've got this. If only you had FLASH. Could've cured him with no surgery needed. No side effects!
 
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I would now treat primary tumor bed/nasal vestibule/residual concha stuffed with vaseline gauze to 60Gy, facial lymphatics and bilat necks levels I-IV to 60 with concurrent weekly cisplatin. Primary was on the right side and he had LN on the right so that R neck is still at high risk. Left neck at high risk due to ENE. I dont think surgery helped in this situation, was mostly just disfiguring.
 
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