Lynch Syndrome, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions
By Kenneth Kee
()
About this ebook
Lynch syndrome is an inherited medical disorder that confers a person a higher risk of cancers of the digestive tract, gynecologic tract, and other organs.
In Singapore Lynch syndrome has an estimated incidence of 1 in 300 in women and 1 in 500 in the general population.
People who have Lynch syndrome have a considerably higher danger of forming:
1. Colorectal cancer (CRC),
2. Endometrial (uterine) cancer,
3. Gastric (stomach) cancer,
4. Ovarian cancer,
5. Small bowel (small intestinal) cancer,
6. Pancreatic cancer,
7. Urinary tract cancer (bladder or ureter cancer),
8. Kidney cancer,
9. Bile duct cancer,
10. Certain skin tumors (sebaceous tumors of the skin or sebaceous adenomas), and
11. Brain tumors.
People with Lynch syndrome may also be at slightly higher risk of:
1. Breast cancer and
2. Prostate cancer.
The old term for Lynch syndrome was “hereditary non-polyposis colorectal cancer” (HNPCC).
There is no likelihood toward polyp formation and hence the name HNPCC.
It is the most frequent genetic syndrome linked with a higher vulnerability to Colon cancer.
Patients with Lynch syndrome have a 60%-80% risk for Colorectal Cancer (CRC), as well as a higher risk for cancers, most conspicuously endometrial cancer
Endometrial cancer is the second most frequent malignancy in Lynch syndrome with a lifetime danger between 40% and 60%, often happening before Colorectal Cancer (CRC) in females.
The average age for colorectal cancer to be diagnosed in a person with Lynch syndrome is 45 in contrast with the average age of 72 for a new diagnosis of colorectal cancer in the general population.
In Lynch syndrome, colorectal cancer is rather more likely to form on the right side of the colon.
Cause:
Lynch syndrome is a genetic disorder, indicating that the cancer risk can be passed from generation to generation in a family.
Several genes have been identified that are linked to Lynch syndrome.
They are MLH1, MSH2, MSH6, PMS2, and EPCAM.
A mutation (modification) in any of these genes causes the person to have a higher lifetime danger of forming colorectal cancer and other related cancers.
Women also have a higher risk of forming endometrial and ovarian cancers.
Making the diagnosis of Lynch syndrome is normally a 3-stage process, such as:
1. Review of the family cancer history,
2. Tumor testing, and
3. Genetic testing
Lynch syndrome, autosomal dominant inheritance disorder, can also be established through a blood test.
The test can detect if someone has a mutation in one of the genes linked with Lynch syndrome.
Presently testing is provided for the MLH1, MSH2, MSH6, and EPCAM genes.
For patients who have a family history that indicates Lynch syndrome, screening tests can be done on tumor (cancer) tissue to help detect if Lynch syndrome is possible.
The two screening tests indicated are micro-satellite instability testing (MSI) and immuno-histo-chemistry testing (IHC).
The results of these tests can show whether more specific genetic testing should be done.
Treatment:
Removal of the entire colon is the only way to completely avoid the development of colon cancer or to treat a present colon cancer.
1. Subtotal colectomy with ileorectal anastomosis
2. Total colectomy with ileoanal pull-through (pouch procedure)
3. Total colectomy with ileostomy
Subtotal colectomy with ileo-rectal anastomosis and post-surgical rectal surveillance are advised when colorectal cancer forms in patients with Lynch Syndrome.
This surgery may be regarded for prevention in selected mismatch repair (MMR) gene mutation carriers.
Prognosis
The 5-year survival rate in patients with Lynch Syndrome is evaluated to be about 60%, compared with 40-50% for sporadic cases (normal colorectal cases).
TABLE OF CONTENT
Introduction
Chapter 1 Lynch Syndrome
Chapter 2 Causes
Kenneth Kee
Medical doctor since 1972.Started Kee Clinic in 1974 at 15 Holland Dr #03-102, relocated to 36 Holland Dr #01-10 in 2009.Did my M.Sc (Health Management ) in 1991 and Ph.D (Healthcare Administration) in 1993.Dr Kenneth Kee is still working as a family doctor at the age of 74However he has reduced his consultation hours to 3 hours in the morning and 2 hours inthe afternoon.He first started writing free blogs on medical disorders seen in the clinic in 2007 on http://kennethkee.blogspot.com.His purpose in writing these simple guides was for the health education of his patients which is also his dissertation for his Ph.D (Healthcare Administration). He then wrote an autobiography account of his journey as a medical student to family doctor on his other blog http://afamilydoctorstale.blogspot.comThis autobiography account “A Family Doctor’s Tale” was combined with his early “A Simple Guide to Medical Disorders” into a new Wordpress Blog “A Family Doctor’s Tale” on http://ken-med.com.From which many free articles from the blog was taken and put together into 1000 eBooks.He apologized for typos and spelling mistakes in his earlier books.He will endeavor to improve the writing in futures.Some people have complained that the simple guides are too simple.For their information they are made simple in order to educate the patients.The later books go into more details of medical disorders.He has published 1000 eBooks on various subjects on health, 1 autobiography of his medical journey, another on the autobiography of a Cancer survivor, 2 children stories and one how to study for his nephew and grand-daughter.The purpose of these simple guides is to educate patient on health disorders and not meant as textbooks.He does not do any night duty since 2000 ever since Dr Tan had his second stroke.His clinic is now relocated to the Buona Vista Community Centre.The 2 units of his original clinic are being demolished to make way for a new Shopping Mall.He is now doing some blogging and internet surfing (bulletin boards since the 1980's) startingwith the Apple computer and going to PC.The entire PC is upgraded by himself from XT to the present Pentium duo core.The present Intel i7 CPU is out of reach at the moment because the CPU is still expensive.He is also into DIY changing his own toilet cistern and other electric appliance.His hunger for knowledge has not abated and he is a lifelong learner.The children have all grown up and there are 2 grandchildren who are even more technically advanced than the grandfather where mobile phones are concerned.This book is taken from some of the many articles in his blog (now with 740 posts) A Family Doctor’s Tale.Dr Kee is the author of:"A Family Doctor's Tale""Life Lessons Learned From The Study And Practice Of Medicine""Case Notes From A Family Doctor"
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Lynch Syndrome, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions - Kenneth Kee
Lynch Syndrome,
A
Simple
Guide
To
The Condition,
Diagnosis,
Treatment
And
Related Conditions
By
Dr Kenneth Kee
M.B.,B.S. (Singapore)
Ph.D (Healthcare Administration)
Copyright Kenneth Kee 2017 Smashwords Edition
Published by Kenneth Kee at Smashwords.com
Dedication
This book is dedicated
To my wife Dorothy
And my children
Carolyn, Grace
And Kelvin
This book describes Lynch Syndrome, Diagnosis and Treatment and Related Diseases which is seen in some of my patients in my Family Clinic.
(What You Need to Treat Lynch Syndrome)
This eBook is licensed for the personal enjoyment only. This eBook may not be re-sold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each reader.
If you’re reading this book and did not purchase it, or it was not purchased for your use only, then please return to Smashwords.com and purchase your own copy.
Thank you for respecting the hard work of this author.
Introduction
I have been writing medical articles for my blog http://kennethkee.blogspot.com (A Simple Guide to Medical Condition) for the benefit of my patients since 2007.
My purpose in writing these simple guides was for the health education of my patients.
Health Education was also my dissertation for my Ph.D (Healthcare Administration).
I then wrote an autobiolographical account of his journey as a medical student to family doctor on his other blog http://afamilydoctorstale.blogspot.com.
This autobiolographical account A Family Doctor’s Tale
was combined with my early A Simple Guide to Medical Conditions
into a new Wordpress Blog A Family Doctor’s Tale
on http://kenkee481.wordpress.com.
From which many free articles from the blog was taken and put together into 800 amazon kindle books and 200 into Smashwords.com eBooks.
Some people have complained that the simple guides are too simple.
For their information they are made simple in order to educate the patients.
The later books go into more details of medical conditions.
The first chapter is always from my earlier blogs which unfortunately tends to have typos and spelling mistakes.
Since 2013, I have tried to improve my spelling and writing.
As I tried to bring you the latest information about a condition or illness by reading the latest journals both online and offline, I find that I am learning more and improving on my own medical knowledge in diagnosis and treatment for my patients.
Just by writing all these simple guides I find that I have learned a lot from your reviews (good or bad), criticism and advice.
I am sorry for the repetitions in these simple guides as the second chapters onwards have new information as compared to my first chapter taken from my blog.
I also find repetition definitely help me and maybe some readers to remember the facts in the books more easily.
I apologize if these repetitions are irritating to some readers.
Chapter 1
Lynch syndrome
What is Lynch syndrome?
Lynch syndrome is an inherited medical disorder that confers a person a higher risk of cancers of the digestive tract, gynecologic tract, and other organs.
In Singapore Lynch syndrome has an estimated incidence of 1 in 300 in women and 1 in 500 in the general population.
People who have Lynch syndrome have a considerably higher danger of forming:
1. Colorectal cancer (CRC),
2. Endometrial (uterine) cancer,
3. Gastric (stomach) cancer,
4. Ovarian cancer,
5. Small bowel (small intestinal) cancer,
6. Pancreatic cancer,
7. Urinary tract cancer (bladder or ureter cancer),
8. Kidney cancer,
9. Bile duct cancer,
10. Certain skin tumors (sebaceous tumors of the skin or sebaceous adenomas), and
11. Brain tumors.
People with Lynch syndrome may also be at slightly higher risk of:
1. Breast cancer and
2. Prostate cancer.
The old term for Lynch syndrome was hereditary non-polyposis colorectal cancer
(HNPCC).
There is no likelihood toward polyp formation and hence the name HNPCC.
It is the most frequent genetic syndrome linked with a higher vulnerability to Colon cancer.
Typical Features of Lynch syndrome
Patients with Lynch syndrome have a 60%-80% risk for Colorectal Cancer (CRC), as well as a higher risk for cancers, most conspicuously endometrial cancer
Endometrial cancer is the second most frequent malignancy in Lynch syndrome with a lifetime danger between 40% and 60%, often happening before Colorectal Cancer (CRC) in females.
Lynch syndrome is also linked with a higher relative danger for cancer in the stomach, ovary, small bowel, pancreas, ureter, renal pelvis, biliary tract, and brain
One of the major features (phenotype) of Lynch syndrome carriers is that cancer often forms at an earlier age than in the normal population
The average age of start for colorectal cancer and endometrial cancer for patients with Lynch syndrome is about 44 and 48 years, respectively, compared to 65 years for colorectal and 60 years for endometrial sporadic carcinomas in the normal population
The incidence of synchronous (multiple primary cancers happening simultaneously) and metachronous (multiple cancers happening at intervals) CRCs happen in up to 50% of individuals with Lynch syndrome, compared to less than 20% risk in patients