Hereditary Colorectal Neoplasia (Sanford R. Weiss, MD)

Welcome

Greetings!

Welcome to the website of the Sanford R. Weiss, MD Center for Hereditary Colorectal Neoplasia. The goal of this site is to provide helpful information and resources about inherited colorectal cancer syndromes. Please browse any or all of the provided information and feel free to contact us with any questions.

Approximately 5% of all colorectal cancer cases are hereditary, in which a tendency to form cancers is passed down from a parent to a child. Just like any other trait such as eye color or height runs in families, an increased risk to form certain types of cancers can also run in families. This happens when a mutation that predisposes to cancer formation occurs by bad luck at conception or is inherited from an affected parent. Inherited colorectal cancer can look just like other colorectal cancers, but tends to develop at a young age and there can be more than one cancer in the colon.

Another feature of inherited colorectal cancer syndromes is the risk of developing cancer in other organs. Multiple family members with the same types of cancer, especially in more than one generation, raises suspicion of a hereditary colorectal cancer syndrome. It is very important to diagnose inherited colorectal cancer when it is present, because then there is the opportunity to screen family members by genetic testing, and to prevent cancer by timely testing and treatment.

It is our mission at the Sanford R. Weiss, MD Center for Hereditary Colorectal Neoplasia to prevent deaths from inherited colorectal cancer, and we dedicate ourselves to this task every day. We approach this through patient care, education, and research. In fulfilling our mission we develop a partnership with our patients, their families, and their family physicians. It is important that our patients follow suggested appointments and treatment recommendations, to the best of their ability. On our part we are always ready to accommodate special requests and circumstances, and are used to helping families from all over the United States, and the World. We offer “one-stop shopping” by coordinating appointments for the different specialties that need to be seen, all in one day. We are grateful to our patients who trust us with their health. Our registry is one of the largest in the world, is backed by years of experience, and offers the highest levels of expertise. Our role is to set the standard of excellence of care of patients with inherited colorectal cancer.

While our priority is to diagnose, screen, treat, and follow-up our patients, we are also committed to education and research. Not only do we teach our patients and families about their disease, we take great pride in educating physicians, nurses, and other caregivers about these syndromes. We have prepared accurate and easy to understand information for patients on the internet. We have also provided education for other healthcare providers, are active at national meetings, and have important leadership roles in national and international societies.

The third pillar of our mission is research on the diseases we treat. Hereditary colorectal cancer syndromes are uncommon, and there is much that is not known. We have the privilege of caring for large numbers of affected patients, and also the responsibility of using our experience to further the knowledge of these conditions.

Lastly, please know that we at the Sanford R. Weiss, MD Center for Hereditary Colorectal Neoplasia, feel honored to be able to care for you and your families. We are available to help you live your life to the fullest. Accessing this website is the start. Thank you for visiting with us.

Sincerely,

David Liska, MD
Director, Weiss Center

About Us

The Sanford R. Weiss, MD Center for Hereditary Colorectal Neoplasia (The Weiss Center) is made up of healthcare providers from multiple disciplines who are dedicated to the care of patients with or at risk for hereditary colorectal cancer syndromes. In 1979, Dr. David G. Jagelman established Cleveland Clinic’s Familial Polyposis Registry. His intent was to follow high-risk colorectal patients and families with a personal and/or family history of familial adenomatous polyposis. In 1989 Dr. Jagelman left the Cleveland Clinic and his role was taken over by Dr. James Church. At that time, Hereditary Non-Polyposis Colorectal Cancer was added to the registry. In 2008 The Weiss Center was established after receiving an endowment from Sanford R. Weiss, MD. Dr. David Liska is the current Director of the Weiss Center.

The mission of the Jagelman Registry and The Weiss Center is to prevent death from colorectal cancer or cancer of other organs involved in these syndromes by maintaining excellent patient care, effective education, and clinically relevant research.

Patient care

• To identify patients at high risk for colorectal cancer by virtue of their family history or tumor genetics.
• To advise such patients of their risk and help in the selection of appropriate screening tests and therapy.
• To provide specialized counseling and genetic testing where appropriate.
• To act as a patient advocate, and facilitate testing, counseling, and treatment appointments.

Cleveland Clinic is committed to identifying patients who may have Lynch syndrome by a routine screening process of patient tissues. The Digestive Disease & Surgery Institute, Genomic Medicine Institute, Pathology and Laboratory Medicine Institute, and Women’s Health Institute have collaborated to screen all colorectal and endometrial cancers that are surgically removed at the Cleveland Clinic for Lynch syndrome by microsatellite instability (MSI) and/or immunohistochemistry (IHC) testing. Routine screening of colorectal cancers with MSI or IHC was initially recommended in 2009 by the Evaluation of Genomic Applications in Practice and Prevention (EGAPP).

The Weiss Center also holds specialized multidisciplinary clinics for patients with hereditary colorectal syndromes. Physicians and caregivers work together to provide a team approach to patients and families to facilitate screening and interventions as needed.

Education

• To educate patients and their families on the nature and implications of inherited colorectal cancer.
• To educate physicians and other healthcare professionals about the basics of colorectal cancer genetics and their clinical implications.
• To encourage and foster the development of inherited colorectal cancer registries throughout this country and the world.

As part of our commitment to educating patients about the importance of family history, we help to determine colorectal cancer risk assessment by asking questions about personal and family history of colon polyp and cancer to determine an individual’s risk of developing colorectal cancer and what type of colorectal cancer screening would be appropriate.

Research

• To carry out clinically important research on inherited colorectal cancer syndromes.

Frequently asked questions

The cornerstone of the Weiss Center is the David G. Jagelman Inherited Colorectal Cancer Registries. Here are some frequently asked questions about the Registries.

What is a registry?

A registry is a database of patients and families with hereditary colorectal cancer that forms the basis of patient care, education and research. It allows coordination and integration of care and its site is the setting where patients and families can meet with specialists.

What is the role of the registry?

The main role of a registry is to allow for effective coordination of evaluation and care of patients at high familial risk of colorectal cancer. The aim is cancer prevention. In addition, a registry:

• Promotes the knowledge of the risks and implication of a family history of colorectal cancer.
• Provides the best care to patients and families.
• Conducts research.

The registry will:

• Educate patients and their families about the disease, tests, surgery options, surveillance protocols and studies.
• Refer patients and families to meet with a genetic counselor for genetic consult.
• Provide support and counseling.
• Ensure that patients receive the best care.

How can a registry help?

The registry educates patients and their families, provides support and counseling, and ensures that patients receive the appropriate care needed to live a long healthy life.

Education:

The Registry provides patients and their families with information about their disease. The coordinators will help patients and families understand who is at risk, and what type of surveillance testing should be done and when. The registry coordinators will also review the different types of procedures, procedure instructions, different types of surgical options as well as current clinical trials available to patients and families.

Support and counseling:

The Registry will be a resource for patients and their families if they have questions regarding their disease. The coordinators will also be available to listen to patients when they need to talk and to provide patients with questions to ask their physicians when looking for the best care at outside facilities when unable to be seen at the Cleveland Clinic. The Registry encourages patients to educate themselves on their disease and will provide patients with the available resources to do that. The coordinators also encourage patients to meet with genetic counselors to discuss in greater detail the benefits of genetic testing and how that will affect the whole family.

Patient care:

The Registry coordinators will help facilitate patients in making their initial appointments with the appropriate specialists as well as scheduling appointments in our hereditary cancer “High-Risk Clinic”. Coordinators will be available to patients before, during and after physician appointments to answer questions. Patients in the Registry may receive reminders of annual examinations, and coordinators can assist patients in scheduling follow-up appointments and tests.

How do I join the Registry at Cleveland Clinic?

A patient needs to talk with a registry coordinator, who will take a personal and family history to determine if participation in the registry is appropriate. This can be done in person or over the phone. Once eligibility for participation is determined, a coordinator will discuss the consent form for participation. The consent form summarizes information on the registry, risks and benefits, confidentiality, and voluntary participation. If the patient is interested in participation, verbalizes an understanding and has no questions they can sign the consent, when being seen in person. An original copy of the consent is kept on file with the registry and one copy is given to the patient. If the patient is not seen in person then the registry coordinator will mail out a study fact sheet to the patient with a medical release form that is accompanied by an envelope to be completed and sent back to the registry. Once received the patient is enrolled into the registry.

Being in a registry should not affect your relationship with your own physician. It is important to note that patients in a registry are still under the care of their referring doctors. Registries do not assume the care of their participants. The main role of the registry is to educate and support patients.

What information will the Registry need?

Once a consent form is signed, a confidential chart is started which consists of 6 parts; a pedigree (a family tree), clinical records, diagnostic test results, correspondence, outside records and the original signed consent form. The Registry will collect all records regarding medical and surgical treatment pertaining to the disease. Every patient will sign a medical release form to help us gather the patient’s outside records. The Registry will also collect death certificates of deceased family members to confirm the actual cause of death. The Registry will do an extensive personal and family history by developing a family pedigree, which will be an ongoing project since families are continually changing.

What is done with this information?

All records obtained by the Registry are kept in a confidential chart. Each family is assigned a Registry identification number and each member of that family is given a patient number. All records collected are filed in a family chart under the appropriate family member. The records are also entered into a password-protected database. The information is used to learn more about the patient and their family history, track the patient’s medical care, and is also used for research purposes.

What about confidentiality?

All records obtained by the Registry are kept in secure confidential files within the Registry Office. The only people permitted to review those records are the Registry personnel and physicians caring for those patients. The Registry is approved by the Institutional Review Board (IRB) at the Cleveland Clinic and is bound by regulatory guidelines to ensure patient confidentiality.

How do I get in touch with the Registry?

The David G. Jagelman Inherited Colorectal Cancer Registry
Colorectal Surgery - A30
Cleveland Clinic Foundation
9500 Euclid Avenue
Cleveland, OH 44195
Telephone: 216.444.6470, 800.223.2273 ext. 46470

What We Treat

We treat hereditary colorectal cancer syndromes, which cause approximately 5% of all colorectal cancer cases. Hereditary cancer syndromes can be passed through a family, such as from a parent to a child. Just like any other trait such as eye color or height runs in families, an increased risk to form certain types of cancers can also run in families. This happens when a mutation that predisposes to cancer formation occurs by bad luck at conception or is inherited from an affected parent.

Inherited colorectal cancer can look just like other colorectal cancers but tends to develop at a young age. Individuals with hereditary colorectal cancer syndromes are often at risk to develop more than one colorectal cancer and/or another type of cancer. It is important to identify if someone has a hereditary cancer syndrome so we can ensure we are taking appropriate steps to help that individual reduce the risk of developing cancer. This might include things like more frequent cancer screening, beginning at a young age, medications to help reduce the risk of getting cancer, and, in some cases, preventative surgery.

Below is a list of some of the more common hereditary cancer syndromes that we treat.

Polyposis syndromes

• Familial adenomatous polyposis.
• MYH-associated polyposis.
• Serrated polyposis syndrome/hyperplastic polyposis syndrome.
• Juvenile polyposis syndrome.
• Peutz-Jeghers syndrome.
• PTEN Hamartoma Tumor Syndrome, Cowden Syndrome, and Bannayan-Riley-Ruvalcaba Syndrome.

Nonpolyposis syndromes

• Lynch syndrome/HNPCC.
• Familial colorectal cancer.
• Familial colorectal cancer type X.

High Risk Clinics

The staff of the Sanford R. Weiss, MD Center for Hereditary Colorectal Neoplasia is dedicated to serving patients and families with hereditary colon cancer. To minimize the burdens that can be associated with coordinating medical appointments recommended for patients with hereditary colorectal cancer syndromes, we have created a number of high-risk clinics. The high-risk clinic is offered a few times a month, with the goal of coordinating all of your appointments related to hereditary colorectal cancer in one day.

Services

The registry coordinators will assist you in scheduling appointments depending on your syndrome and needs. The high-risk clinic is staffed by healthcare providers from a variety of departments who have interests and expertise in hereditary colorectal cancer syndromes. To learn more about our providers, please see the “Our Team” tab.

High-risk clinic

High-risk clinics have been structured for individuals and families with inherited colorectal cancer syndromes who need to see multiple specialists. The goal is to make screenings and appointments easier by assisting you to schedule all of your hereditary colorectal cancer appointments. We try our best to accommodate scheduling these appointments for you in one day.

Here is an example of a schedule for a patient with Lynch syndrome being seen in the high-risk clinic.

• 8 a.m. – Consultation with gynecologic oncologist.
• 9:15 a.m. – Consultation with dermatology.
• 10:30 a.m. – Consultation with colorectal surgeon.
• 11 a.m. – Colonoscopy and upper endoscopy.
• Noon – Lab appointment for blood work.

Here is an example of a schedule for a patient with familial adenomatous polyposis being seen in the high-risk clinic.

• 9 a.m. – Thyroid ultrasound.
• 10 a.m. – Consultation with colorectal surgeon.
• 10:30 a.m. – Consultation with genetic counselor.
• 11:30 a.m. – Flexible sigmoidoscopy.

During your visit, a registry coordinator will also be available to provide you with assistance. Your healthcare providers will be informed about your assessment and the recommendations made by the specialists in The Weiss Center.

Preparation for your appointment

When you call to make your appointment, the registry coordinator will take a family and medical history. Depending on your schedule, you may require special instructions. For example, if you are scheduled for a colonoscopy you will be required clean out your colon. These instructions will be discussed with you at the time of making the appointment. You may be asked to bring copies of your medical records with you, or to sign a release prior to your visit so that we may obtain the necessary medical records.

What to bring with you

• Photo identification and insurance card.
• Support person (or driver if having a procedure).
  • We plan on reviewing information with you and although we are always here to answer your questions a support person can assist with remembering the information.
  • If you are having a procedure with sedation (colonoscopy/EGD) you will be required to have a responsible adult accompany you.
• Medical records.
  • Any medical records, including copies of lab results, surgical pathology results, upper endoscopy/colonoscopy procedures, surgeries, genetic testing results, a list of medications, and anything else you may feel is important.
• Family history.
  • A family history is very important in determining proper care and treatment for you. You can use the following file to make a record of your family history or you can write down a list of important information about your family members such as cancers (and age occurred), surgeries, polyps, children, names, ages, and any genetic testing.

Making an appointment

To make an appointment or to learn more about the David G. Jagelman, MD Registries and high-risk clinic please contact one of our coordinators at: 216.444.6470 or toll-free at 800.223.2273 extension 46470.

Our Team

Leadership

David Liska, MD Director

Carol Burke, MD Section Head, Colorectal Polyposis

Surgical specialties

Mariam AlHilli, MD Gynecology	Steven Campbell, MD, PhD Urology	David Liska, MD Colorectal Surgery
Joyce Shin, MD Thyroid & Endocrinology	Scott Steele, MD Colorectal Surgery	Anil Vaidya, MD Intestinal Transplant Surgery
R. Matthew Walsh, MD Upper Gastroenterology Surgery	Joshua Sommovilla, MD Colorectal Surgery

Medical specialties

Amit Bhatt, MD Gastroenterology	Carol Burke, MD Gastroenterology	Jacob Kurowski, MD Gastroenterology
Joseph Parambil, MD Pulmonary	Holly Pederson, MD Breast Diseases	Tyler Stevens, MD Gastroenterology

Genetics

Charis Eng, MD, PhD Department Chair

Jessica Marquard Genetic Counselor

Sarah McGee, MS, CGC Genetic Counselor

Pathology

Michael Cruise, MD, PhD Staff

Thomas Plesec, MD Director, Gastrointestinal Pathology

Psychology

Karen Hurley, PhD

Registry coordinators

Lisa Laguardia

Margaret O’Malley

Susan Milicia

Administrative assistant

Lakeisha Sellers

Make an Appointment

To schedule an appointment or learn more about our services, please call 216.444.6470 or toll free at 800.223.2273 ext. 46470

Virtual second opinions

If you cannot travel to Cleveland Clinic, help is available. You can connect with Cleveland Clinic specialists from any location in the world via a phone, tablet, or computer, eliminating the burden of travel time and other obstacles.

If you’re facing a significant medical condition or treatment such as surgery, this program provides virtual access to a Cleveland Clinic physician who will review the diagnosis and treatment plan. Following a comprehensive evaluation of medical records and labs, you’ll receive an educational second opinion from an expert in their medical condition covering diagnosis, treatment options or alternatives as well as recommendations regarding future therapeutic considerations. You’ll also have the unique opportunity to speak with the physician expert directly to address questions or concerns.

Learn more about virtual second opinions

Resources

Family Matters

A Weiss Center publication for people with colorectal cancer in their families

• December 2020 Edition.
• June 2020 Edition.
• December 2019 Edition.
• August 2019 Edition.

Patient stories

• Young Dad Recognizes Symptoms, Wins Battle with Colorectal Cancer.
• Florida Woman Road Trips to Cleveland for Specialized Hereditary Colorectal Surgery.
  

Podcasts

Butts and Guts explores your digestive and surgical health from end to end with our host, Colorectal Surgery Chairman Scott Steele, MD.

• Bringing Awareness to Hereditary Colorectal Cancer.
• Young Adults and Colorectal Cancer.
• Colorectal Cancer Screening Guidelines.
• Psychosocial Impact of Colorectal Cancer and Other Colon Disorders.

Health Essentials

Find helpful posts from Cleveland Clinic’s Health Essentials site. Discover the latest colorectal cancer health and wellness tips.

• When Colorectal Cancer Runs in Your Family.
• Under Age 50? 4 Tips to Help You Avoid Colon Cancer.
• What You Can Do to Catch Colon Cancer Early.

Virtual Patient Event: Young-Onset and Hereditary Colorectal Cancer Education Day

During our virtual patient seminar Young-Onset and Hereditary Colorectal Cancer Education Day, members of Cleveland Clinic's Digestive Disease & Surgery Institute and Cancer Center discuss the rise in young-onset colorectal cancer, who is most at risk, nutrition monitoring, genetic counseling and more.

For Medical Professionals

Hereditary Gastrointestinal Cancer Syndromes Fellowship

Learn more about this unique training program and apply here

Consult QD

Discover the latest and greatest innovative research at Consult QD, Cleveland Clinic's site for physicians and healthcare professionals.

• Phase 3 Study Tests Sulindac/Eflornithine Combination for Familial Adenomatous Polyposis.
• Intraoperative Enteroscopy in Peutz–Jeghers Syndrome.
• Colorectal Cancers in African Americans Have Unique Biology.
• Should We Rethink Cancer Risk Staging in Familial Adenomatous Polyposis?

Podcasts

Cancer Advances is a podcast for medical professionals, exploring the latest innovative research and clinical advances in the field of oncology with host Dale Shepard, MD.

• Screening Guidelines and Incidence Rates for Colorectal Cancer.