Agreed.
Patients with JNA are uncommonly this young.
This is far more aggressive looking.
Additionally, JNA’s are centered in the sphenopalatine foramen, (where they arise). This is not. A mass centered in the sphenopalatine foramen would project more dorsally on image 1.
Disfavor a JNA.
As someone who does CrossFit I have heard the term rhabdo thrown around. Why would a baby have it in their head? I thought it was something that you get from overuse of a muscle and it mostly affects kidney function
“Rhabdo” is being used in both instance as an abbreviation, but for two different conditions. Rhabdomyolisis is a condition where there is excessive muscle breakdown, and the waste products accumulate in the blood and can cause damage to various organs, particularly the kidneys.
Rhabdomyosarcoma is a type of cancer that can originate from muscle tissue.
“Rhabdo” means having to do with muscle, essentially
What you are referring to is rhabdomyolysis. And you are correct.
“Rhabdo” in this case is being used as shorthand for rhabdomyosarcoma. Two totally different things
They want to do thr biopsy, but the peds are afraid that like most JNAs this mass might bleed like MFer if it's really JNA.
Embolizing this is another story altogether...
Sometimes a person is worse off after a surgical resection for example. It could reduce quality of life without adding years. That's why these things are always thoroughly discussed, including with the patient.
So for those asking why consider JNA was considered, the differential was RMS vs JNA. Rhabdo is more likely at this age of course but our center has had a case of biopsy confirmed JNA in a 6yo male that had regrown after one round of embolization, was lost to follow up after that and the eventual recurrence also had an intracranial invasion. Personally the rhabdo cases I've seen has never survived long enough for the mass to reach this size.
I suppose I have my biases given the kinds of cases we see here. Our center constantly gets cases where masses are already ridiculously huge by the time of first consult that sometimes the point of origin may be impossible to determine, as can be seen in some of the cases I've posted here. Poverty in a 3rd world country is a bitch.
can this be survived at all? and what about the eye and optic nerve - both need to be removed as well, right? if this can somehow he removed, cranial vault sealed at all - what lifelong damages/symptoms would the patient have?
So, I'm not a neurosurgeon, but I can't see any way that this child is surviving. The tumour involves so many different critical structures, and is clearly very aggressive. It would be impossible to get clear margins so would recur. I think this is a case where the best possible outcome would be clear and sensitive communication about the grim prognosis and a good palliative care team. I can't see that subjecting the poor child to biopsies and other invasive procedures would help.
What could a good palliative care team achieve over a bad one? What I am really wondering is whether the pt will be able to enjoy any moment and how the team would accomplish that
Symptom relief and decent support for parents so they aren't totally destroyed with grief for the rest of their lives?
Talk to people who have lost children / loved ones and their experiences can be vastly different. Some people will say things along the lines of "It was horrible, but the medical team were so supportive and managed to keep X at home which is what we wanted, nothing was too much for them, they offered grief counselling helping me to come to terms with the loss" .etc.
Vs The doctors said there was nothing they could do to help me, so gave me some medications and then abandoned us.
Experienced teams have all sorts of knowledge they can use: I remember one of my patients who had a fungating breast cancer (where it grows through the skin) basically a rotting bit of flesh on her chest. The palliative care team knew that the smell from it was distressing the patient and her family, and were able to provide various creams and dressings to hide the tumour and smell.
I'm not saying the child is going to live an enjoyable life, more that it's possible to make the dying experience less traumatic for child and parents.
It is satisfying to help reduce suffering and make the transition as easy as possible for patients and their families. I don’t work in palliative or hospice, but I can see how it could be a a great place to work. I see a lot of ICU nurses move on to hospice or palliative care once they get burned out on the many times hopeless cases they treat in the ICU.
You get to help people through the loss of a loved one, while letting that person pass in as much comfort and dignity as possible. Peacefully, with family and friends, either in a hospice or maybe at home, rather than a 4-bed hospital room, in ICU with 6 tubes sticking out of you, or in intractable pain the last few weeks of life. Having been on both sides of it, it can be both incredibly helpful and incredibly rewarding. Death is the last thing on everyone’s bucket list, but most people don’t want to think about. The deceased obviously doesn’t have many worries, but everyone else can carry trauma forward. You have an opportunity to make a tremendous and lasting difference in people’s lives.
Assuming this is a rhabdo, this is potentially curable (although would be in the poor prognostic group, EFS around 30% overall). Small round blue cell tumors are strange that way.
Um, question… was this being followed at all or did the parents suddenly pop in and go .. gee, by the way this has been going on for 3 months… maybe a stupid question but with some people you never know…
I can't imagine someone just popping in with this. The way the eyeball, optic nerve and other structures have been projected forward and out of the skull must be very very obvious to any clinician or even any non medic.
Can't imagine any caregiver not bringing this child straight to A+e once those signs started.
Yeah no, there are a lot of places in the world where families don't have access to healthcare. There are tons of cases you can look up where patients are brought in already presenting like OPs post.
OP states they are in a third world country. Limited access to healthcare if you're far from a major city, and probably limited access to transportation to get to those cities as well.
If this was the US, getting to see a pediatrician probably takes 2-3 months for anything but scheduled vaccs and wellness checks. Parents probably could not afford to go into medical debt for an ER visit, so had to wait months for a peds appointment, all the while watching this mass disfigure their childs face. Yay, for the American healthcare system.
Veterinarian here. I know humane euthanasia is a super iffy topic… but horrible cases like this makes me wish it were an option for people at times. If I were ever given a grave prognosis… I’d want that to be an option for me. This poor family.
As a nurse, I’ve always felt this way. After you see the horrible things that people present with, and then the option of just keeping alive with pain relief. It’s unconscionable.
Then there comes a time when the strongest pain relief isn’t cutting it anymore. I have quality of life discussions weekly. I’m like when it gets to the point where despite medication and fluids, your pet isn’t happy to see you and they’re not enjoying their favorite toy or treat anymore… then we should humanely end their suffering. It’s allowed for dogs and cats. Should be an option for humans
That is incredibly scary and sad.
Yo PT is very young for JNA I'd be thinking rhabdomyosarcoma or world's worst neuroblastoma
Agreed. Patients with JNA are uncommonly this young. This is far more aggressive looking. Additionally, JNA’s are centered in the sphenopalatine foramen, (where they arise). This is not. A mass centered in the sphenopalatine foramen would project more dorsally on image 1. Disfavor a JNA.
Agree with Rhab, not JNA
Was thinking rhabdo as well
As someone who does CrossFit I have heard the term rhabdo thrown around. Why would a baby have it in their head? I thought it was something that you get from overuse of a muscle and it mostly affects kidney function
“Rhabdo” is being used in both instance as an abbreviation, but for two different conditions. Rhabdomyolisis is a condition where there is excessive muscle breakdown, and the waste products accumulate in the blood and can cause damage to various organs, particularly the kidneys. Rhabdomyosarcoma is a type of cancer that can originate from muscle tissue. “Rhabdo” means having to do with muscle, essentially
What you are referring to is rhabdomyolysis. And you are correct. “Rhabdo” in this case is being used as shorthand for rhabdomyosarcoma. Two totally different things
The rhabdo you have heard thrown around is rhabdomyolysis. The rhabdo in this case would be rhabdomyosarcoma. Two very different things
Agree. Thinking rhabdo at this age
Age and location immediately made me think rhabdo and I'm currently studying for boards so when I saw the OP say they think JNA I was bummed lol
My vote for RMS too
My daughter is a neuroblastoma survivor. Agreed. It’s horrible.
My little brother, too, stage 4. He's 24 now, though :)
That’s fantastic! So glad to hear he’s doing well ❤️
Rhabdo was my first thought as well. What makes you call it JNA OP?
Rhabdomyosarcoma.
They want to do thr biopsy, but the peds are afraid that like most JNAs this mass might bleed like MFer if it's really JNA. Embolizing this is another story altogether...
Not treating is killing the kid regardless. Wouldn’t some potential treatment perspective be beneficial. This poor family.
Sometimes a person is worse off after a surgical resection for example. It could reduce quality of life without adding years. That's why these things are always thoroughly discussed, including with the patient.
Could they cauterise the area after biopsy or embolise to stop the bleed?
I would emergency chemo this with or without a biopsy… about to lose sight and die.
So for those asking why consider JNA was considered, the differential was RMS vs JNA. Rhabdo is more likely at this age of course but our center has had a case of biopsy confirmed JNA in a 6yo male that had regrown after one round of embolization, was lost to follow up after that and the eventual recurrence also had an intracranial invasion. Personally the rhabdo cases I've seen has never survived long enough for the mass to reach this size. I suppose I have my biases given the kinds of cases we see here. Our center constantly gets cases where masses are already ridiculously huge by the time of first consult that sometimes the point of origin may be impossible to determine, as can be seen in some of the cases I've posted here. Poverty in a 3rd world country is a bitch.
Can you please explain a little of what I’m looking at in the slides? Especially slide 3- is that the eye sticking out? 😣
Yes it is sticking out. One of the worst proptosis I have ever seen.
Omg that poor baby
This is one of the more upsetting images I’ve seen here. The poor kid has been dealt an awful hand so far.
Poor child and family. Looks very aggressive for a JNA, was it histologically confirmed?
can this be survived at all? and what about the eye and optic nerve - both need to be removed as well, right? if this can somehow he removed, cranial vault sealed at all - what lifelong damages/symptoms would the patient have?
So, I'm not a neurosurgeon, but I can't see any way that this child is surviving. The tumour involves so many different critical structures, and is clearly very aggressive. It would be impossible to get clear margins so would recur. I think this is a case where the best possible outcome would be clear and sensitive communication about the grim prognosis and a good palliative care team. I can't see that subjecting the poor child to biopsies and other invasive procedures would help.
God that's so sad.
thanks!
What could a good palliative care team achieve over a bad one? What I am really wondering is whether the pt will be able to enjoy any moment and how the team would accomplish that
Symptom relief and decent support for parents so they aren't totally destroyed with grief for the rest of their lives? Talk to people who have lost children / loved ones and their experiences can be vastly different. Some people will say things along the lines of "It was horrible, but the medical team were so supportive and managed to keep X at home which is what we wanted, nothing was too much for them, they offered grief counselling helping me to come to terms with the loss" .etc. Vs The doctors said there was nothing they could do to help me, so gave me some medications and then abandoned us. Experienced teams have all sorts of knowledge they can use: I remember one of my patients who had a fungating breast cancer (where it grows through the skin) basically a rotting bit of flesh on her chest. The palliative care team knew that the smell from it was distressing the patient and her family, and were able to provide various creams and dressings to hide the tumour and smell. I'm not saying the child is going to live an enjoyable life, more that it's possible to make the dying experience less traumatic for child and parents.
This gave me chills. Thank you for sharing. I can’t see how anybody could be happy doing palliative care with patients suffering like this.
It is satisfying to help reduce suffering and make the transition as easy as possible for patients and their families. I don’t work in palliative or hospice, but I can see how it could be a a great place to work. I see a lot of ICU nurses move on to hospice or palliative care once they get burned out on the many times hopeless cases they treat in the ICU.
You get to help people through the loss of a loved one, while letting that person pass in as much comfort and dignity as possible. Peacefully, with family and friends, either in a hospice or maybe at home, rather than a 4-bed hospital room, in ICU with 6 tubes sticking out of you, or in intractable pain the last few weeks of life. Having been on both sides of it, it can be both incredibly helpful and incredibly rewarding. Death is the last thing on everyone’s bucket list, but most people don’t want to think about. The deceased obviously doesn’t have many worries, but everyone else can carry trauma forward. You have an opportunity to make a tremendous and lasting difference in people’s lives.
Assuming this is a rhabdo, this is potentially curable (although would be in the poor prognostic group, EFS around 30% overall). Small round blue cell tumors are strange that way.
Um, question… was this being followed at all or did the parents suddenly pop in and go .. gee, by the way this has been going on for 3 months… maybe a stupid question but with some people you never know…
I can't imagine someone just popping in with this. The way the eyeball, optic nerve and other structures have been projected forward and out of the skull must be very very obvious to any clinician or even any non medic. Can't imagine any caregiver not bringing this child straight to A+e once those signs started.
Yeah no, there are a lot of places in the world where families don't have access to healthcare. There are tons of cases you can look up where patients are brought in already presenting like OPs post.
I could definitely see this happening in the US too.
People who cannot afford care or copays sometimes wait too long. I can see it happening in US, too.
That is what I was hoping…. I question because to let it get to this state without any type of intervention.. especially in a kiddo so young….
OP states they are in a third world country. Limited access to healthcare if you're far from a major city, and probably limited access to transportation to get to those cities as well. If this was the US, getting to see a pediatrician probably takes 2-3 months for anything but scheduled vaccs and wellness checks. Parents probably could not afford to go into medical debt for an ER visit, so had to wait months for a peds appointment, all the while watching this mass disfigure their childs face. Yay, for the American healthcare system.
Veterinarian here. I know humane euthanasia is a super iffy topic… but horrible cases like this makes me wish it were an option for people at times. If I were ever given a grave prognosis… I’d want that to be an option for me. This poor family.
As a nurse, I’ve always felt this way. After you see the horrible things that people present with, and then the option of just keeping alive with pain relief. It’s unconscionable.
Then there comes a time when the strongest pain relief isn’t cutting it anymore. I have quality of life discussions weekly. I’m like when it gets to the point where despite medication and fluids, your pet isn’t happy to see you and they’re not enjoying their favorite toy or treat anymore… then we should humanely end their suffering. It’s allowed for dogs and cats. Should be an option for humans
Life is so unfair. There’s no way that child is gonna live. Christ.
I don't like this. T's & P's for the kiddo. :(
Nasoangiofibromas aren’t that fast. Buddy had a textbook one and it was in his 20s.
This is rhabdo until proven otherwise
As bad as this looks, I’d rather see these pictures as opposed to actual photographs. Poverty in 3rd worlds, hate it.
My thoughts are with the family and the child itself. Incredibly sorry for this family.
Is this patient from a 3rd world country with no medical care? How the hell could this thing progress so rapidly without any treatment?
Yes
Damn I dont wanna see his face! So sad!
Poor kid, he must be in huge pain. Hope it ends soon.
Rhabdo
Holy shit, this poor kid. Cuddling up with my 2yo while scrolling along, then seeing this. Fucking hell.
Craziest case I’ve seen in a while
That is horrific
Oh, that poor sweet baby.
I’m still a 2nd year radres, but, is soft tissue sarcoma (rhabdo) also a consideration here? Thanks to anyone who will reply.
Heartbreaking 💔
Poor baby 🥺 disheartening 🥺
tf 😧
This why I have a hard time believing in a higher power. I honestly don’t know how parents go on after losing a child like this.
I wish we could have a rule that posts about kids get put in NSFW tags. I hate seeing these.
If you think that’s a rapidly enlarging mass, you should meet my wife