Phaeo vs. Neuro

[KLPM]

---

Members don't see this ad.

So we had a 3yo who presented with hypertension with systolics in the 220s on arrival. Later CT and MRI of the abdomen showed a huge adrenal medullary mass. The query was phaeochromocytoma or neuroblastoma. The oncology people said that they have a test that will tell them the difference.

Does anyone know what test they mean? Because I have been looking through the notes of this kid and his pathology results. All I can find is that they did catecholamine metabolites, routine stuff like FBE, electrolytes and one unvalidated test saying chromogranin A (which I thought was a stain).

 

[Enkidu]

So we had a 3yo who presented with hypertension with systolics in the 220s on arrival. Later CT and MRI of the abdomen showed a huge adrenal medullary mass. The query was phaeochromocytoma or neuroblastoma. The oncology people said that they have a test that will tell them the difference.

Does anyone know what test they mean? Because I have been looking through the notes of this kid and his pathology results. All I can find is that they did catecholamine metabolites, routine stuff like FBE, electrolytes and one unvalidated test saying chromogranin A (which I thought was a stain).

I think I read that they produce different catecholamine metabolites.

 

[flindophile]

This
http://www.arupconsult.com/assets/print/Neuroblastoma.pdf


vs this:

http://www.arupconsult.com/Topics/Pheochromocytoma.html

 

[thebouque]

Urinary HVA VMA for the neuroblastoma, urinary metanephrines for the pheo. I hope the kid has a pheo, neuroblastoma has a pretty bad prognosis at 3.

 

[KLPM]

Urinary HVA VMA for the neuroblastoma, urinary metanephrines for the pheo. I hope the kid has a pheo, neuroblastoma has a pretty bad prognosis at 3.

They said that the biochemistry equivocal regarding which one it was. The clinical situation is as thus:

- Kid presented with a hypertensive episode with seizures and hyponatraemia.
- Took a fair bit of anti-hypertensives to get that BP down.
- CT and MRI both produced pretty **** images but definitely a huge left adrenal mass.
- Biochemistry didn't quite pinpoint one or the other.
- Paed surgeons and Oncology had a big argument

• Oncology want primary resection without chemo to determine type of tumour
• Surgery wants chemo to shrink tumour since it was wrapping around the renal vessels and they want to make sure the kidney is spared
• Surgery wants CT-guided biopsy to determine tumour
• Oncology worried that kid will become hypertensive again during the biopsy

CT-guided biopsy done yesterday. Awaiting result.

 

[pianoguy05]

Bx was the correct call, in my humble opinion. Hypertension can generally be managed...it's silly to put off a diagnosis and treatment for fear of this particular complication. Hopefully H&E/IHC yields an answer for your patient.

 

[KLPM]

Bx was the correct call, in my humble opinion. Hypertension can generally be managed...it's silly to put off a diagnosis and treatment for fear of this particular complication. Hopefully H&E/IHC yields an answer for your patient.

He had his biopsy under general anaesthesia. I saw him this morning and he was fine and completely happy.

I am curious to hear other people's opinions about this issue too.

 

[KLPM]

Diagnosis: ganglioneuroblastoma

 

[Enkidu]

Diagnosis: ganglioneuroblastoma

What's the prognosis for that? It's better than neuroblastoma, right?

 

[Substance]

What's the prognosis for that? It's better than neuroblastoma, right?

Depends on what kind of ganglioneuroblastoma it is: nodular or intermixed? What's the classification of the nodular component if the patient has one?

 

[malchik]

I think ganglioneuroblastoma of any subclassification has a better prognosis than neuroblastoma.

 

[Substance]

I think ganglioneuroblastoma of any subclassification has a better prognosis than neuroblastoma.

I thought that if the neuroblastoma component of a ganglioneuroblastoma was a really bad classification(undifferentiated or whatever its called), then the prognosis was bad. In other words, the prognosis of a nodular gangioneuroblastoma is dependent on the badness of the neuroblastomatous component.

 

[KLPM]

No expert but what I have compiled from reading Rosai, Sternberg and DDx is:

Ganglioneuroblastoma can be broadly recognised as either "nodular" or "intermixed".

• Noduar - nodules of neuroblastic components well demarcated from ganglioneuromatous components
• Intermixed - neuroblastic components are randomly scattered in nests in ganglioneuromatous components

Rosai says that some authors see the intermixed variety as part of the spectrum of differentiating neuroblastoma. It seems that the nodular form has a poorer prognosis than the intermixed ones.

Then there is this paper: http://onlinelibrary.wiley.com/doi/10.1002/cncr.11773/full

It has a table that goes through the variants of the nodular type and then looks at which ones seem to fall under favourable or unfavourable subsets.

 

[dermpathdoc]

keep us posted as this is extremely interesting!