[CIS-PAGID] Hyper IgM patient with dysphagia and stridor

[Church, Joseph]

Thanks, Kate.  JC

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Sullivan, Kathleen
Sent: Saturday, April 14, 2012 5:15 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hyper IgM patient with dysphagia and stridor

I would be worried about a tumor.  a biopsy is definitely the next step.  Although the progression seems slow for a tumor and the location not so typical- I think neuroendocrine tumors can happen anywhere.


On Apr 14, 2012, at 2:58 PM, Church, Joseph wrote:


Colleagues:

I follow an 18yo with XHIM.  He was generally stable until about a year ago when he developed dysphagia and stridor.  These symptoms have progressed and he is currently hospitalized with wasting and severe stridor (although SaO2s run 95-100% even when he is asleep).

Below are the latest of multiple MRIs.
FINDINGS:
Neck:    Again noted is fullness/swelling of the posterior pharynx
subglottic region. This could be due to edema of the esophagus which
is seen more distally on the MRI of the chest. Alternatively, it may
be true retropharyngeal swelling or a mass. There is continued
narrowing of the trachea at the level of vocal cords and inferiorly.
At the level of the vocal cords the tracheal lumen measures
approximately 1-2 mm transversely.  Inferior to the vocal cords the
trachea lumen measures approximately 2-3 mm.  The narrowing extends
approximately 1-2 cm below the vocal cords. There is no evidence of
significant lymphadenopathy. The thyroid gland is normal.

Chest:  There is no significant change.  Again seen is marked mucosal
thickening of the mid and distal esophagus.  No large mediastinal mass
is noted. Please note the post contrast images are limited due to
pulsation artifact such that much smaller lesions may not be seen.
Also calcifications may not be appreciated. As mentioned on the prior
MRI, developing fibrosing mediastinitis cannot be excluded.

IMPRESSION:
1.  Stable marked mucosal of thickening of the esophagus as described
above.
2.  No large mediastinal mass.  Please see above.

His stridor is associated with vocal cord paralysis, presumed to be due to esophogeal process.  ENT and GI have scoped him several times.  Superficial biopsies have not shown evidence of tumor or other inflammatory processes.  Blood PCRs for herpes viruses have been negative.

We are planning a more aggressive biopsy procedure.

Has anyone seen this type of process in HIM previously?  Any suggestions on what to look for on next biopsy?

Thank you for your input.

Joe Church
Children's Hospital Los Angeles








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