[PAGID] RE: A-T and hepatocarcinoma. .

[Sandlund, John]

Dear Drs. Rosenzweig and Ruprecht,
  Thank you for your inquiry regarding this patient.  I have not seen
this type of tumor in an A-T patient before (A-T patients most commonly
develop a malignant lymphoma or leukemia).  I discussed this child with
our institutional expert on hepatocellular carcinoma.  He said that
total surgical resection is the only known curative measure.  If the
tumor is completely resected, he would hold off on the use of chemo at
all, because complete surgical resection is the only modality shown to
be curative.
   I assume that your description of the surgical procedure as "total
macroscopic removal" of the tumor, means that you don't know yet whether
the margins are positive or not.  Is that correct?  The use of
chemotherapy for residual disease would be considered investigational.
If your patient has only microscopic residual disease, you would have no
way to measure activity of the experimental agents you select.  If the
patient has measurable residual disease, or if the tumor recurs, the
attached regimen could be consider; however, it is experimental and is
very toxic.  I'm also attaching a copy of the background section of our
St. Jude institutional protocol which reviews general principles for
giving chemotherapy to A-T patients.  For example, we make an 25%
reduction in the dose of topo inhibitors (eg., anthracyclines).
Best wishes,
Torrey
(I'm cc others who forwarded your e-mail to me, so that they are "in the
loop" regarding my response)
 
 
    


________________________________

	From: HOWARD M LEDERMAN [mailto:hlederm1 at jhem.jhmi.edu] 
	Sent: Tuesday, March 21, 2006 11:25 AM
	To: pagid at clinimmsoc.org; Kastan, Michael; Sandlund, John
	Subject: A-T and hepatocarcinoma. .
	
	

	Sergio,

	I am forwarding your message to Mike Kastan and Tory Sandlund at
St. Jude's Hospital in Memphis.  They are the oncologists who have
accumulated the largest current experience with treatment of cancers in
A-T patients.
	

	Howard
	Howard M. Lederman, M.D., Ph.D. 
	Professor of Pediatrics and Medicine 
	Division of Pediatric Allergy and Immunology 
	Johns Hopkins Hospital - CMSC 1102 
	600 N. Wolfe Street 
	Baltimore, MD 21287-3923 
	Phone:  410-955-5883 
	Fax:    410-955-0229 
	e-mail: Hlederm1 at jhem.jhmi.edu 
	
	
	

	

	----- Original Message ----- 

	

	From: Sergio Rosenzweig <sdrosenzweig at yahoo.com> 

	

	Date: Monday, March 20, 2006 5:56 pm 

	

	Subject: [PAGID] A-T and hepatocarcinoma 

	

	

	> Dear friends, 
	>  One of my colleagues in our unit is following an A-T pt with 
	> hapatocellular carcinoma. Here are her concerns and questions.

	>  Your advice will be apreciated. 
	>  Sergio 
	>   
	>  Sergio D. Rosenzweig, MD 
	>  Servicio de Inmunologia 
	>  Hospital Nacional de Pediatria "J.P. Garrahan" 
	>  Buenos Aires, Argentina 
	>  srosenzweig at garrahan.gov.ar 
	>   
	>  Dear Dr. 
	>  The patient of our concern is a 16 years old girl with AT and

	> hepatocellular carcinoma. 
	>  Since her immunological diagnosis she had no serious
infection 
	> or other complication. 
	>  Two months ago she started with right upper quadrant pain and

	> elevated liver enzymes (x2). Hepatitis B and C were ruled out
and 
	> alpha fetoprotein level was extremely high (>35.000 ng/ml for 
	> baseline of 500). The MRI showed a multilobular tumor of the
right 
	> lobe. A percutaneous biopsy showed hepatocellular carcinoma of
the 
	> fibrolamellar subtype. 
	>  She underwent surgical resection with total macroscopic
removal. 
	> During surgery, a 1 cm lesion was found on the left liver
lobe, 
	> and also a tumoral thrombosis of the right portal vein. Both 
	> lesions were removed. 
	>  Have you seen patients with AT and this kind of tumor? 
	>  Would you use chemotherapy after surgery? 
	>  In case you suggest chemo, what kind of protocol would you
choose? 
	>  Thanks for your help 
	>   
	>  Barbara Ruprecht, MD 
	> 
	> 
	> 
	>        
	> --------------------------------- 
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