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Killer case (no pun intended here)! Does this excellent image demonstrate the interface between the lesion and normal myocardium?

----Comment by: Iago on 7/15/2008 2:54:47 PM

Welcome CManavi! A great first post. I'm going to hold an opinion... until Iago figures it out :-)

----Comment by: PathDoc15 on 7/15/2008 5:20:25 PM

I can only hold on to my opinion for so long - were there multiple distinct lesions in the heart? Is there disease anywhere else?

----Comment by: PathDoc15 on 7/16/2008 4:29:10 AM

PathDoc15 wrote: "Welcome CManavi! A great first post. I'm going to hold an opinion... until Iago figures it out :-)" Thanks. There were multiple round, tan/yellow distinct lesions grossly visible on the epicardium as well. The multiplicity of the lesions were discovered upon microscopic examination of different sections of the heart.

----Comment by: cmanavi on 7/16/2008 4:37:00 AM

Iago wrote: "Killer case (no pun intended here)! Does this excellent image demonstrate the interface between the lesion and normal myocardium?" That is correct.

----Comment by: cmanavi on 7/16/2008 4:37:29 AM

PathDoc15 wrote: "I can only hold on to my opinion for so long - were there multiple distinct lesions in the heart? Is there disease anywhere else?" I am sorry, I didn't answer the second part of your question. The lesion was only in the heart and no other organ had such a lesion. The child was a completely normal looking child with no known medical or genetic problems.

----Comment by: cmanavi on 7/16/2008 8:51:50 AM

Cytologic and nuclear features are not particularly high grade. Could this be some kind of reactive process?

----Comment by: Iago on 7/16/2008 2:19:36 PM

Last questions - how big were the nodules? My low power impression was neoplastic (rhabdomyosarcoma or rhabdomyoma). My high power impression is a little less certain. Multiple nodules is also not great for primary cardiac rhadomyosarcoma. Although certain tumor syndromes can have multiple rhabdomyoma (TS comes to mind - but as an isolated finding this would be unusual) Concerning reactive - Just not sure - Iago what are you thinking? I would probably get a consult on this one ;-)

----Comment by: PathDoc15 on 7/17/2008 8:15:18 AM

Sorry pathdoc15. If your surg path skillz can’t take you straight to the diagnosis on this one, you may want to think about a career in something like Molecular Diagnostics or better yet Dermpath. Wait…actually, I agree with you so far, but I’ll probably go down the rhabdomyoma tubes here. Clinically, some rhabdomyomas can present with sudden death due to conduction system interruptions and they can be multifocal. However, I have been told that the key to this case rests in the cytoplasmic features of the cells in question. That’s where I’m at so far.

----Comment by: Iago on 7/17/2008 9:49:33 AM

It's not right for malignant. Not right for rhabdomyoma either. I want it to be not-neoplastic, but I'm just at a loss for a localized, multifocal reactive or infectious process in the heart that looks like this. Don't give the answer yet - I want to see if I'm the only dummy who can't figure this one out.

----Comment by: PathDoc15 on 7/17/2008 10:13:46 AM

Does the image rater average ratings (image is a 4.5)? If so, what a function! This image has been viewed quite a bit but it seems to be stumping even the most intrepid users. Time is running out…..Neoplastic or non-neoplastic. I vote non-neoplastic.

----Comment by: Iago on 7/21/2008 7:57:17 PM

How about Gaucher's disease ? Are mom and dad related ? Are they Ashkenazy jews or other sect ?

----Comment by: savco on 7/22/2008 5:34:20 AM

What about an extra renal rhabdoid tumor? Cmanavi, we need some help bro.

----Comment by: Iago on 7/24/2008 5:40:26 AM

Savco - Good to hear from you. Hope all is going well. My first though when I saw this image (after rhabdo) was abnormal histocytes. They certainly have a foamy sort of appearance that are seen with Gaucher cells. Glycogen storage diseases also came to mind but the histology isn't right at all ( [Ref #1] - BTW, this educator does a nice job with basic histology - even for appropriate for new pathology residents...) As far as Gaucher - that would be sneaky since cardiac abnormalities would be a very unlikely isolated finding without hepatospenomegally - [Ref #2] However, I like the histocyte idea. Can we have a CD68 please.

----Comment by: PathDoc15 on 7/24/2008 4:51:44 PM

And yes, the ratings are averages, and you only get to vote once. On a side note - I recently learned how to write webservices, so I plan on 'optimizing' (or breaking) the site once I get some free time... If it all works, you will notice absolutely no difference - except the site will be a bit faster.

----Comment by: PathDoc15 on 7/24/2008 4:58:02 PM

my vote for benign lesion

----Comment by: mudassira on 4/1/2009 2:40:06 AM

I would suggest we do an S100 to rule out a granular cell tumor.

----Comment by: kyouens on 4/1/2009 9:04:19 PM

How about a primary cardiac granular cell tumor?

----Comment by: PathDoc15 on 5/27/2009 6:47:09 AM

Ah, I see that is what our intrepid KYouens suggested previously. I do like the granular cell tumor idea. A rare tumor of the heart, but very nice histology for that disease. It only took me a year to get around to diagnosing this - how about that for turn around time!

----Comment by: PathDoc15 on 5/27/2009 10:09:23 AM

     

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