[Histonet] Chimerism

[Tony Henwood]

I believe this is known as Proteus syndrome. The following is from a
paper I am writing on the cytological findings of a hydrocele fluid from
a boy with Proteus syndrome (I must pull my finger out and submit it).

As an aside, it is believed that the "elephant man" suffered from this
disorder.


Proteus syndrome is a rare disorder and it estimated that there are only
several hundred patients affected. It is postulated that the syndrome is
caused by a post-zygotic mosaic alteration in a gene that is lethal in
its non-mosaic state (4). Apart from connective tissue and epidermal
nevi, disproportionate overgrowth of some bones and other malformations,
bilateral ovarian cystadenomas are regarded as having diagnostic value
in Proteus syndrome when occurring in the first two decades of life.

Papillary lesions, resembling common epithelial ovarian tumours are rare
in the testis with only a handful being described in pre-pubertal males
(4). It has been suggested that these papillary lesions, so called
serous papillary cystadenomas of low malignant potential, may prove to
have the same diagnostic value in Proteus syndrome, as do bilateral
cystic ovarian tumours (4).

Some references follows:

3.	Bale, P.M., Watson, G., Collins, F., (1993) "Pathology of
Osseous and Genitourinary Lesions of Proteus Syndrome" Ped. Pathol
13:797-809.
4.	Raju, R.R., Hart, W.R., Magnuson, D.K., Reid, J.R., Rogers,
D.G., (2002) "Genital Tract Tumours in Proteus Syndrome: Report of a
case of Bilateral Paraovarian Endometrioid Cystic Tumours of Borderline
Malignancy and Review of the Literature" Mod Pathol 15(2):172-180.
5.	McClure, R.F., Keeney, G.L., Sebo, T.J., Cheville, J.C., (2001)
"Serous Borderline Tumour of the Paratestis: A Report of Seven Cases" Am
J Surg Pathol 25(3):373-378.
6.	Austin, P.F., Rink, R.C., Cain, M.P., Casale, A.J., Davis, M.M.,
Faught, P.R., (1998) "Testicular Serous Papillary Cystadenomatous Tumor
of Low Malignant Potential in a Child" J Urol 160(6-I):2002-2003.


Regards

Tony Henwood JP, MSc, BAppSc, GradDipSysAnalys, CT(ASC)
Laboratory Manager & Senior Scientist
The Children's Hospital at Westmead,
Locked Bag 4001, Westmead, 2145, AUSTRALIA.
Tel: 612 9845 3306
Fax: 612 9845 3318




-----Original Message-----
From: histonet-bounces <@t> lists.utsouthwestern.edu
[mailto:histonet-bounces <@t> lists.utsouthwestern.edu] On Behalf Of Paul
Bradbury
Sent: Tuesday, 11 April 2006 12:19 AM
To: Kemlo Rogerson; HistoNet Server
Subject: Re: [Histonet] Chimerism


Hi Kemlo,

>The only times I have come across chimerism has been associated with 
>blood transfusion. The patient presents with two different, but 
>apparently coexisting, cell populations. So my best guess to look for 
>further info would be the National Blood Transfusion Service, or a 
>similar body. I believe this is an exceedingly rare condition, but 
>certainly a fascinating one, should make an interesting thesis topic.
>

Paul Bradbury
Kamloops, BC, Canada


Kemlo Rogerson wrote:

>I have a student that wants to follow a NVQ (National Vocational
>Qualification) and has chosen Chimerism (Genetic Mosaicism) about which

>I know nowt. Does anyone out there know anything about this subject or 
>anyone who does?
>
>I've vainly been trying to find my feminine side and wonder if I've 
>been looking in the wrong place; if this fascinating subject is to be 
>believed it could, genetically, be anywhere <finds torch>.
>
>Kemlo Rogerson
>Pathology Manager
>Ext  3311
>DD   01934 647057
>Mob 07749 754194
> 
>
>  
>

> 
>
>
>
>
>  
>



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