Talk:Hereditary leiomyomatosis and renal cell cancer syndrome

Scope of article?

Hello - While trying to do some copy editing I got a bit stuck in the lede (with bolding, MeSH term selection, etc) because the page seems to cover two overlapping entities: in MeSH terms, Reed's syndrome and Hereditary leiomyomatosis and renal cell cancer. This situation presumably arises because Wikipedia does not seem to have a separate article on Reed's syndrome. I'm wondering whether this new page should actually be two pages. Anyway, I hope I haven't made too much of a mess with my edits! —MistyMorn (talk) 17:29, 9 April 2012 (UTC)

Having given this a bit more thought, I guess the single article (as is) really is probably the better solution. But I'd like to learn how to deal with the technical problems I mentioned in the lede/infobox. At the moment I've provided only the MeSH term corresponding to the page title: maybe there's an acceptable way of including both MeSH term ids in the infobox. I think it might be confusing to bold Reed's syndrome (and synonyms) at the start of the lede. —MistyMorn (talk) 20:48, 9 April 2012 (UTC)

I am also thinking the 2 conditions are just one. Some literature suggests that only a subset of families are affected by the increased renal cancer risk. Another facet seems to be risk of progression to leiomyosarcomas. Classification like ICD and MeSH is often misleading when dealing with rarely diagnosed conditions. No idea about the technical issues. Richiez (talk) 21:50, 9 April 2012 (UTC)

Yes, that's the sort of direction I've been thinking in too. My only substantial concern regards the increased risk of malignancies pertaining only to a subset of families.
I think I've worked around the bolding issue (though it's a bit of a thicket, and I haven't even tried to insert acronyms!), and will ask for Template Help to try to find a way of fitting in the second Mesh ID. Cheers, —MistyMorn (talk) 22:15, 9 April 2012 (UTC)

Removal of sentence on treatment

Although a small number of cases of fibroids in this condition have been found to be sarcomatous, this is also the case in the so called sporadic fibroids. The risk of malignant change is very low in all cases. There have been no studies that I can find that have formally assessed the risk in these families. All that can be said at the minute is that there does not seem to be an increased risk of malignant change in the fibroids in this condition. For this reason recommendations that differ from normal management do not seem to be justified at present. DrMicro (talk) 11:32, 12 April 2012 (UTC)

This sentence has been redrafted and is now more reasonable. However I am still unaware of any studies showing a significantly increased risk in these families over that in sporadic cases. I'm not entirely happy with this statement for those reasons.DrMicro (talk) 11:49, 12 April 2012 (UTC)

The reference cited in the lede article is a review which expresses an opinion on follow up of any uterine fibroids found. It is a very reasonable opinion and one consistent with good medical practice. That having been said the evidence base for this opinion seems to be lacking. The article itself does not recommend that management of fibroids found in such a family should differ significantly from the usual practice other than careful follow up unless there is evidence exists of sarcomatous change. For those reasons I would suggest being very reluctant to make a definite statement here on treatment of of any fibroids that occur in this condition. DrMicro (talk) 12:02, 12 April 2012 (UTC)

This is a review that includes and lists the four cases of uterine leiomyosarcoma known to have occurred in this condition up to 2007.

Hodge JC, Morton CC (2007) Genetic heterogeneity among uterine leiomyomata: insights into malignant progression. Hum Mol Genet 16(1)1:R7-13.

Since virtually all women with this condition (>90%) have fibroids and the accepted rate for malignant change in sporadic fibroids is ~1/1000 there does not appear to be a significantly enhanced risk of malignant change in the fibroids that develop in this condition. That having been said given that almost all women with this condition develop fibroids and some of these may undergo malignant change surveillance in known cases of this syndrome does seem sensible. DrMicro (talk) 17:31, 12 April 2012 (UTC)

I was looking at PMID 20301430, "Whether all women with HLRCC have a higher risk of developing uterine leiomyosarcomas is unclear.....". There is no exact figure given and we won't know for a long while because as far as I know there are some 100 confirmed cases (families) altogether. However 2 out of 11 resp 6 out of ~100 families is a much higher rate than the 1/1000 for average population so we need to be aware of the possibility that the risk is substantially ellevated. Especially if you consider that in the previous century the hysterectomy rate for HLRCC was pretty close to 100% and the leiomyosarcoma risk might have been even greater if the hysterectomy rate were lower.

The wording could be different, the paper is not very clear about what to do. - Richiez (talk) 19:38, 13 April 2012 (UTC)

I agree with the above remark. The available data suggests there might be a higher risk of malignant change but it is certain that many fibroids are removed whose mutation status is unknown. I believe that the correct position is that malignant change has rarely been reported and that this should be kept in mind while treating such women. Presently there is insufficient evidence to recommend radical surgery routinely on these leiomyomas. This is definitely a condition that should be kept under review.DrMicro (talk) 14:58, 16 April 2012 (UTC)

Unsure about sources

Most, if not all sources appear to be primary sources. Is this because the disease is so rare? I would hate to starting culling sources without input on their appropriateness by other editors.

Best Regards,

Barbara (WVS) ✐ ✉ 02:09, 24 June 2017 (UTC)

Just 300 families, so very rare. Klbrain (talk) 07:32, 29 August 2022 (UTC)

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Merge proposal

Support March proposal to merge the 'disease' to the syndrome, given the heavy overlap in content and that the smaller disease article will benefit from the context provided here. Klbrain (talk) 22:49, 29 August 2022 (UTC)

  Y Merger complete. Klbrain (talk) 17:16, 31 December 2022 (UTC)

Wiki Education assignment: PHMD 2040 Service - Learning Fall 2023

This article was the subject of a Wiki Education Foundation-supported course assignment, between 31 May 2023 and 29 December 2023. Further details are available on the course page. Student editor(s): Shelbyorch (article contribs).

— Assignment last updated by Shelbyorch (talk) 01:44, 15 April 2024 (UTC)