Talk:Atypical teratoid rhabdoid tumor

Redo Intro Statistics
The Statistics on AT/RT occurance rate 30 per year; yet 3% of the 3,000 cases (90 per appear); appear to contridict each other. Review References and update. ITBLAIR

Article too long
This Wiki page was originally based on a Public Domain Paper by Loice Swisher MD. Her child died of AT/RT. Preliminary Draft: June 24, 2002

I have been slowly rewriting her work and bringing it up to date. For example, I have been putting in Intro/Content materialm, adding a more 2005 St. Jude Study and doubling the number of citations. I propose to write a section on Pediatric CNS Cancer Funding (very limited). Also, I will try to include some graphs on Mortality. ITBlair 05:22, 8 June 2007 (UTC)

This page is very long, especially compared to articles on other kinds of brain tumors, even those which are much more common than ATRT. It just seems a little odd. Dylan Slade 14:06, 17 May 2007 (UTC)

This is true, it is a Long Article. Ms. Swisher put together a comprehensive overview. I hope to capture the major articles on AT/RT since 2001. Also, longer term I plan a Wiki Page on Pediatric CNS Cancer, which I expect will be at least as large.

I intent to break off some sections (e.g., Clinical Trials; Cancer Resources) into separate articles. ITBlair 05:55, 18 May 2007 (UTC)

Some chunks of text would be more appropriate on - or already exist on - other pages that already exist. I have done a little clean up. Una Smith 21:02, 11 June 2007 (UTC)

Some other chunks of text can be eliminated, substituting appropriate navigation templates, as on the teratoma page. The article on which this page is based was evidently written to stand alone, as it explains many topics relevant to but not specific to AT/RT. In contrast, a wikipedia article should gracefully link to those other topics and state the relevance. Una Smith 04:06, 12 June 2007 (UTC)

Clinical Trials
A large part of the Clinical Trials section was deleted and not moved to the Clinical Trial Article. In the main this consisted of Hospitals and Other Institutions that run Clinical Trials. Often their trails eventually get into the Federal Clinical trial Registry. I intend to replace this information. ITBlair 05:29, 30 June 2007 (UTC)


 * The consensus of clinical trial editors is to delete all such lists, the reason being that Wikipedia is not a directory (WP:NOT) and such lists both are and attract link spam. IIRC, talk:clinical trial has a long discussion of this.  I don't remember if I deleted the list, but I do remember deciding not to move it.  Personally, I don't care much, and even if I did care of course you are welcome to add the list, but I recommend you don't bother:  someone else will delete it.  Also, I would rather hospitals came here to recruit patients, rather than we "beat the bush" to send patients to them. --Una Smith 02:38, 8 July 2007 (UTC)

Teratoid
Please explain the term teratoid. Una Smith 04:30, 11 June 2007 (UTC)

From an Online Medical Dictionary (easy to Google): ter·a·toid (tr-toid) adj. Resembling a teras; grotesquely deformed.

The doctors who identified this disease used this adjective because the AT/RT tumor cells are often deformed.


 * So put that in the article. Perhaps add a note that teratoid in this case has nothing to do with teratoma. Una Smith 20:04, 11 June 2007 (UTC)

I will do so. I have been trying to get copies of the original 1975 article where the made a Distinction between Wilm's Tumors and Rhabdoid Tumors. Next I am after the 1985 and 1987 articles that distinguish AT/RT from Rhabdoid tumors. It seems that it is hard to make a definitive AT/RT diagnosis. An AT/RT tumor looks like a range of other CNS tumours; genetic analysis appears to be the key. Basically, I am after this diagnostic history behind the Word Teratoid. ITBlair 04:30, 13 June 2007 (UTC)

AFP
In what sense is AFP not found in AT/RT tissues? Babies normally have elevated AFP, and AFP is a transport molecule that enters cells. Does the immunohistology test for AFP test for AFP per se, or for expression of AFP (eg, for mRNA encoding AFP)? Una Smith 04:30, 11 June 2007 (UTC)

The Absense of AFP means that an antibody test cannot find it in the CNS tissue sample. This is a Pathologist using Immunohistochemistry to localize proteins in the cells of a tissue section  The following link shows the lack of AFP in a tissue section is a standard Pathology criteria for AT/RT and other CNS cancers.

http://www.chicagopathology.org/IRAP/2003-2004/lumc04-May-ho.htm

The Wiki page on AFP notes that it is a Tumour Marker, but the discusion is about AFP levels increasing in Serum or CSF (but apparently not tissue samples). However, I do not know what AFP test on tissues a Pathologist uses. I will do some more digging. ITBlair 07:32, 11 June 2007 (UTC)

I ran across an interesting Web Page on using a new cancer marker, named RECAF, which exhibits high sensitivity and specificity on tissue sections and serum of patients with diverse types of malignancies. It notes that Normal Tissue and Benign Tumors do not Test positive for AT/RT. It appears that AT/RT and other CNS cancers also do not test positive.

http://www.devicelink.com/ivdt/archive/05/06/004.html

ITBlair 07:42, 11 June 2007 (UTC)

My guess is that there are three to six tests commercially available to Pathologists to test Tissue Sections for AFP.

http://www.devicelink.com/ivdt/archive/05/06/004.html


 * There are immunohistochemical tests for AFP in fixed tissue samples. The question is:  what test was used to produce the results mentioned in this article?  My point is that a baby conceivably can have AT/RT despite testing positive for AFP.  AFP is more complicated than some other tumor markers.  The wiki page on AFP is a work in progress. Una Smith 20:04, 11 June 2007 (UTC)

Over the next 2 months I will read a book Pathology and bring out the AT/RT papers that I only have abstracts for. Hopefully, I can get more specific information on the AFP tests that are normall used. This will all take time. ITBlair 04:15, 13 June 2007 (UTC)

Futures
Note getting protein analysis from spinal fluid cells. Add Subsection on Protonomics - DONE

Put in Info-Box as to AT/RT Diagnostic Categories -  DONE

Add Work-Up data from Dr. Kieran's article. Plus sort out and list the treatment trends he notes. - DONE

Get Ref. on RIII study and add. - DONE

Check with Kieran and Zimerman on updates, also ask if IRS III LT surviors are just a result of increased age. This was not the case with the Olsen Paper.

More information on pallative care Note details on Rhabdoid tumors, changing definition of this cancer Link to Renal Cancer (if any) -  Think and REDO This Material. It appears that around 25% of other CNS cancers are actually AT/RT. Wonder if many AT/RT cancers are actually other types.

Rethink list of treatment options. Test Cancer in vitrio to select Chemotherapy, what drug combinations work best. Better discussion on Stem Cell Rescue.

Add graphic on AT/RT mortality from govt.

WHO Classification of Brain Tumours find and Use. Circa 2000

Better Chemo Discussion - cite studies, to link on each Drug Type. Note Global vs. Local Chemo. Eventually want to list each drug combination and the results achieved. Better discusion of Treatment Alternatives, include Salvage Surgery. Gross and Sub Resections on Surgery. Size of Tumor Metatisis Induction vs. Maintenance Stem Cell Transplant

Add Reference to Cerebellar Mutism/Posterior Fossa Syndrome Resource Center

Add subsection on Polyclonal PolyPhenotypic

Add Doctor Doctor link to Pathology Section. Send the links to this Pathologist.

Add Section on Histology, list proteins found in Staining and how they characterize AT/RT. Define each protein from the IHOP (http://www.ihop-net.org/UniPub/iHOP/gs/90100.html ) Web Site. Link to Proteomics Lab and work.

Break out section on Clinical Trials into a separate Section with a Link Back. Link to other Clinical Trial Wiki pages Discusse Phases and Legal Issues with Trials. Note Off-Label.

Also note cell line and animal models prior to Phase I; think about how to speed research and integrate Trials with HealthCare E-Records Note tie into general Cancer Statistics Registries.

For each Citation, add the URL where it is available. Try to add in a small Abstract.

Pick up all the Journal References in Rhaboid Kids Web site. Also check papers published by Major Authors

Cite on Wired article on clinical trials - make in formal

Fix links to Yahoo List Servers

DO a Section on Standard(s) of Care for X Disease, relate to Clinical Trials, ERecords/Registry, and Mal-practice. Each Standard of Care should have a Time Box (e.g., as of 2007 X was the Standard of Care of AT/RT).

Add More WIKI Categories.

Add the Diagnostic Categories from the Code Books for AT/RT to the Article. Ask if insurance provides any coverage where there is to Std. of treatmente.

Ideal would be to get with Medical Wiki and get a similar write-up on each of the 120 or so Pediatric Cancers. Need a general section on CNS cancers.

As a separate matter it would be nice to get an integrated Single Registry for all these AT/RT cancers.

ITBlair 04:31, 11 June 2007 (UTC)

Use Wiki Medicine Format?
Consider reorganizing the article using the following Wiki headings and perhaps adding a few of our own. Probably create a references section with the citaitons.


 * Classification
 * Signs and symptoms or Characteristics
 * Causes or Genetics
 * Pathophysiology or Mechanism
 * Diagnosis (including Characteristic biopsy findings)
 * Treatment or Management
 * Prognosis (social impact may also be discussed)
 * Prevention or Screening
 * Epidemiology
 * History (not patient history)
 * Cultural references
 * See also (avoid if possible, use wikilinks in the main article)
 * Notes
 * References
 * Further reading or Bibliography (paper resources such as books, not web sites)
 * External links (avoid if possible)

NOTE: We Should have a STANDARD of TREATMENT Section (even if a standard does not yet exist). Each cancer should have this. Also, each Cancer article should display a Kaplan-Meier estimator.

Thanks
Thanks, Sandy. This is really improving quickly! The focused ==External links== section is a dramatic improvement. WhatamIdoing (talk) 01:57, 17 April 2008 (UTC)
 * Guess not; after four to five hours of cleanup, it was tagged needing cleanup. Sandy Georgia  (Talk) 22:00, 17 April 2008 (UTC)
 * I put the cleanup tag on the article because it is being worked on now, heavily; it is only fair to alert the reader that the article is unstable. I would have tagged it long ago except no one (including me) had the energy to work on it then, and I did not want to leave it tagged indefinitely. --Una Smith (talk) 03:31, 18 April 2008 (UTC)


 * I think that cleanup is conventionally used to indicate articles needing copyediting, wikifying, restructuring, deletion of obvious garbage, and so forth. I don't want to imply that it's inappropriate to use the cleanup template for a technical rewrite, but perhaps inuse or underconstruction would be more descriptive the work you're doing, if you happen to like them.
 * Is there something in the list below that's suitable for the non-expert? I can make an hour for you over the next couple of days, and I'm happy to be useful, if there's something simple enough that you think I've got a chance of doing it right.  WhatamIdoing (talk) 06:09, 18 April 2008 (UTC)
 * Neither of those tags quite work for me. inuse asks other editors to refrain from editing, and underconstruction has irrelevant stuff about tagging for deletion.  I don't especially care what tag is used, only that it be tagged.  WhatamIdoing, could you add some of the missing refs?  You might also take a stab at restructuring more per MEDMOS, deleting or moving tangents into related articles, and adding introductory and transitional sentences. --Una Smith (talk) 13:56, 18 April 2008 (UTC)

Cleanup
I tagged the article "cleanup". Here is why. --Una Smith (talk) 04:11, 18 April 2008 (UTC)

Classification and History
The article currently is a little confusing to read in part because AT/RT is a fairly recent concept. So, I think the History section needs to come much earlier in the article, perhaps before Classification. --Una Smith (talk) 21:10, 17 April 2008 (UTC)
 * History in a nutshell: medulloblastoma defined;  rhabdoid tumor (usu in kidney) split off from medulloblastoma;  AT/RT (CNS) split off from rhabdoid tumor (kidney only);  AT/RT and rhabdoid tumor reunited to an extent because share some genetic basis and treatment issues. --Una Smith (talk) 14:21, 18 April 2008 (UTC)


 * I think any of these might work for the missing refs in the classification section:
 * "Aberrations of the hSNF5/INI1 gene are restricted to malignant rhabdoid tumors or atypical teratoid/rhabdoid tumors in pediatric solid tumors"
 * "Molecular genetics of atypical teratoid/rhabdoid tumor" (Quote:  "Rhabdoid tumors in all anatomical locations have a similar molecular origin.")
 * "Immunohistochemistry of INI1 expression: a new tool for old challenges in CNS and soft tissue pathology."
 * "Cytogenetics and molecular genetics of childhood brain tumors." Free full text
 * I think the last one will be particularly helpful if you want to talk about the genetic details later in the article. WhatamIdoing (talk) 05:03, 19 April 2008 (UTC)

Genetics
The Genetics section needs an introduction, stating the extent to which the suspected genetic error occurs; at present, the article enumerates only cases in which it does occur. But is that the norm or the exception? --Una Smith (talk) 04:11, 18 April 2008 (UTC)

Diagnosis
Most of the Diagnosis section applies to any suspected brain tumor, not specifically to AT/RT; that content belongs in Brain tumor. Explanations of the diagnostic tests should be pared back and/or moved to the relevant articles, wikilinked. --Una Smith (talk) 04:11, 18 April 2008 (UTC)

Pathology
The Pathology section introduces the idea that AT/RT resembles several other tumor types, but does not explain (clearly) how they can be distinguished. The Immunohistochemistry section has tangential content about the technique, and otherwise consists of a list of proteins that may be present in an AT/RT, but again lacks any information (and citations) re how these proteins help (if they do) to distinguish this tumor from others. --Una Smith (talk) 04:11, 18 April 2008 (UTC)

Treatment
Much of the content in this section is not specific to AT/RT, nor is the application of these treatments to AT/RT fully explained. --Una Smith (talk) 04:11, 18 April 2008 (UTC)

Prognosis
The Prognosis section, and elsewhere in the article, statements about younger children having a worse prognosis are unclear. These statements need careful explanation and qualification. In general, early diagnosis is better than late diagnosis because, all else being equal, tumor size and risk of metastasis increase with time. On the other hand, a very aggressive tumor may be diagnosed earlier because of its aggressiveness. Also, the diagnostic landscape is changing rapidly, so that statements re prognosis in the older literature may no longer apply. An example is sacrococcygeal teratoma; before prenatal ultrasound exams were routine in wealthier countries, the cases of these tumors that were found prenatally were the worst cases:  already massive and/or causing "mirror syndrome" in the mother. --Una Smith (talk) 04:11, 18 April 2008 (UTC)

Epidemiology

 * says that ATRT was 2.1% of primary pediatric brain tumors in a hospital series.
 * from 2002 has some incidence information.

17% of all pediatric cancers involve the CNS
Hi, on a first reading of the statement about "most common solid cancer in children" this seemed to refer specifically to ATRT which is, of course not true. Central nervous system (CNS) cancers are, collectively, the most commmon solid tumours in childhood and are second only to leukaemia - see the very useful references below.Leukaemia-ken (talk) 09:07, 26 June 2012 (UTC)

Una, I've been looking for the missing ref for the lead. Here's what I've found that's close:


 * "Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor in childhood. Between 2,500 and 3,500 children are diagnosed in the United States each year."


 * "Tumors of the central nervous system constitute the largest group of solid neoplasms in children and are second only to leukemia in their overall frequency during childhood. Each year, brain tumors develop in approximately 1500 to 2000 children in the United States..."


 * "Brain tumors are the second most common cancer in children after leukemia and constitute about 20% of all childhood cancers in the United States. The annual incidence is approximately 34 per million children under 15 years of age."


 * "CNS malignancies represented 16.6% of all malignancies during childhood (including adolescence). CNS cancer as a group was the second most frequent malignancy of childhood and the most common of the solid tumors.", which is a chapter in this SEER monograph

The last one, I suspect, is the source used for the statement in the lead, and I believe much of the rest of that paragraph is taken from a table in that doc. I'm not sure that it's the "one true source" though, so I'll let you compare the options and make the relevant changes. Hope this helps, WhatamIdoing (talk) 04:42, 19 April 2008 (UTC)


 * Thanks for that leg-work, WhatamIdoing! I regard SEER as the US authority on epidemiological data, especially for rare cancers, but there is no "one true source", especially not for the rarest of the rare pediatric cancers.  Doesn't this context info belong in Brain tumor rather than here? --Una Smith (talk) 04:31, 20 April 2008 (UTC)


 * I'm happy to help. This stuff does seem a little out of place here.  In particular, I don't think that the ATRT's introduction needs to spend so much time on brain tumors in general.  Part of it might be okay under ==Epidemiology==.  WhatamIdoing (talk) 20:49, 20 April 2008 (UTC)
 * Apparently the original article was written for parents of kids diagnosed with AT/RT, so it started from ground zero and touched on everything remotely relevant. --Una Smith (talk) 03:50, 21 April 2008 (UTC)

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