Lymphoma question

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qwert

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How does a malignant pericardial effusion impact Ann Arbor stage?

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I'd say it's an "E"...

So, if you have supradiaphragmal lymph nodes (usually large mediastinal tumor mass +/- more nodes) and also a pericardial effusion, you get a Stage IIE.
 
I agree, but I can also see how pericardial effusion may count as "extensive organ involvement" and upstage pt to IV.
 
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Agree with you Q.
Regardless if its II or IV officially, I can't imagine these people do well, you'd probably tx them like an advanced case, right?
-S
 
The patients with pericardial effusion I have seen so far usually had aggressive lymphomas or very extensive disease of less aggressive lymphomas.

Pericardial (and pleural) effusion is common for example if T-lymphoblastic lymphoma with large mediasinal masses and these patients are often treated like a T-ALL.

I have also seen a couple of patients with aggressive lymphomas (primary mediastinal diffuse large b-cell lymphoma / angioimmunoblastic lymphoma) presenting with pericardial effusion. They all had large mediastinal mass on presentation.

Last but not least, one should not forget that even if there is pericardial effusion, it does not mean that it is necessary malignant. Patients with large mediastinal masses can indeed have a lymphoma-free pericardial or pleural effusion, because of compromised hemodynamics because of the lymphoma. I would opt for cytological confirmation, if treatment strategy would change according to the presence or not of a malignant pericardial effusion.

On management: We generally do not treat the entire heart, if the pericardial effusion has disappeared after chemotherapy and only treat the lymphoma-involved mediastinum. There's still quite some incidental dose going to the pericard, but treating the whole heart could lead into a DVH-disaster for the lungs and could bear long term heart-toxicity (especially taking into consideration the large amount of chemo these patients generally receive).



Concerning staging and prognostic significance of pericardial effusion:
I think you need to distinguish between malignant pericardial effusion caused by direct invasion of the pericard by a large mediastinal mass and malignant pericardial effusion caused as "distant spread" in the absence of a direct invasion of the pericard by lymphoma.

Patient 1:
6 cm large mediastinal Hodgkin-mass, invading the pericard and causing pericardial effusion probably

Patient 2:
6 cm large infradiaphragmal paraaortal Hodgkin-mass with pericardial effusion without direct invasion of the mass into the pericard and in the absence of a mediastinal mass.

The second patient probably has a worse prognosis, because pericardial effusion is a result of distant spread. I would classify the first patient as IIE and the second patient as IV (and not IIIE).
 
As far as I know, staging of pts with pericardial effusion is one of those areas that is not explicitly defined in the Ann Arbor system.

Looking at a number of papers, these patients are usually classified as Stage IV if the effusion is positive for malignant cells. However, as Palex80 pointed out, there are certain situations where the effusion can be secondary to local extension of disease -- e.g. a large mediastinal tumor directly invading the pericardium -- in which case the staging should be IIE or IIIE.
 
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