Personal Story: Pamela Munster's story of cancer in the family
|At a glance||Clinical trials|
|Pancreatic cancer and||Questions for your doctor|
|What does this mean for me?||Resources|
ARTICLE AT A GLANCE
This article is about:
a personal story about a father and daughter with an inherited
Doctors are people too
Dr. Pamela Munster is a breast cancer oncologist at University of California, San Francisco. When she was diagnosed with breast cancer at age 48, she found that she carried a mutation. Women carrying mutations have a 45-56% risk of breast cancer and 23-31% risk of ovarian cancer by age 70. Dr. Munster had a double mastectomy as part of her treatment.
"Despite my years as a practicing oncologist—specializing in breast cancer, of all things—I was not prepared for it to come to me."
A family history from her father's side
mutations may be inherited from either side of the family with the same impact - increased risk of breast and ovarian cancer. In Pamela Munster's family, her paternal grandmother Gertrud was diagnosed with breast cancer at 65. Gertrud survived her breast cancer and was diagnosed with pancreatic cancer which lead to her death in her 80's. Pamela's father voiced words of concern at her surgery they proved prophetic:
"This is just not right...This should be me, not you! You should not have to go through this at your age."
When her otherwise healthy 78-year-old father Norbert complained of stomach pains, she became concerned. She recognized that stomach pains as a potential symptom of pancreatic cancer. Knowing that she had inherited her mutation from Norbert, she realized he was also at increased risk of BRCA2-related cancers including pancreatic cancer. Norbert was indeed diagnosed with pancreatic cancer in 2013.
Coping with pancreatic cancer
Pancreatic cancer is the fourth most common cause of cancer death. Five year survival rates are low—less than 9%—in part due to these cancers being found late. Pancreatic cancer has been called a "silent disease" because symptoms, which mimic those of more common diseases, are often not recognized until the cancer has advanced.
At the time of his pancreatic cancer diagnosis, Norbert's doctor stated that surgery was not an option due to his age and the size of his tumor. Dr. Munster, her father's self-stated "advocate and protector" asked his doctor whether treatment would be an option if they could successfully shrink the tumor. She said to her father:
"The odds are against you...But knowing you, I think you would rather go down in flames than not try at all."
They proceeded with aggressive chemotherapy knowing that side effects might be difficult to tolerate and that the treatment might not work. This tradeoff between the hope of treatment and the decrease in quality of life while enduring treatment is one that is balanced differently by each individual. Fortunately, Norbert's tumor responded to chemotherapy and in eight weeks was reduced to half its original size when it was surgically removed.
Tumors of carriers are more sensitive to some chemotherapy treatments
The responsiveness of Norbert's tumor to chemotherapy, while surprising, may reflect the biology of the mutation. Because cells with mutations lack functional protein, their ability to repair damage, including damage in tumor cells that is caused by chemotherapy, is impaired. This poor repair can also lead to other mutations which contribute to increased cancer risk in people with mutations.
The advent of new treatments like PARP inhibitors for maintenance of cancers holds promise. PARP inhibitors work by inhibiting a protein that is involved in repair, and in doing so, may help to kill cancer cells. PARP inhibitors are currently approved for treatment of breast and ovarian cancer but not yet for pancreatic cancers. Several ongoing clinical trials are enrolling participants to test the usefulness of PARP inhibitors (Lynparza, , Veliparib, and ) for treating pancreatic cancer .
The story is not over
Five years after his diagnosis, Norbert is still alive. However, his pancreatic cancer returned two years following his surgery. He underwent more chemotherapy and radiation treatments and is now living with cancer.
As Dr. Munster points out:
" cancer is an ongoing battle. It requires steadfast support, resources, stamina, and, most of all, courage and hope. There are endless setbacks and bad days. But seeing my father emerge from his struggle, my own blue eyes meeting his, I am proud. We are now connected by more than blood and ."
She notes that her father's life long habits of exercise and good diet were important. While she does not suggest that these lifestyle choices impacted his cancer, his good physical health before chemotherapy did mean that he was in a better state to endure the difficulties of cancer treatment.
For more about Pamela and Norbert Munster's cancer journey, you can read Dr. Munster's book Twisting Fate: My Journey with
- From Breast Cancer Doctor to Patient, and Back.
What does this mean for me?
People who test positive for an inherited mutation in , , and other genes, have an increased risk of pancreatic cancer. There are no reliable and proven tests for early detection of pancreatic cancer. While experts do not have guidelines for pancreatic screening for everyone at increased risk, there has been some research looking at a procedure known as endoscopic to find abnormalities in the pancreas of high risk people. Endoscopic (EUS) involves the doctor passing a tiny into the patient's stomach to get a closer look at their pancreas. EUS requires anesthesia. Some cancer centers offer endoscopic screening for people with an inherited mutation and a family history of pancreatic cancer although in many cases the procedure is only available through a research study.
Knowing the signs and symptoms of pancreatic cancer may be helpful. Symptoms include:
- jaundice (a yellowing of the skin and eyes)
- belly or back pain
- unintended weight loss and poor appetite
- nausea and vomiting
- stool changes
- changes in diabetes
- blood clots.
NCCN guidelines recommend genetic testing for inherited gene mutations in people diagnosed with pancreatic cancer. Genetic test results may affect treatment recommendations and be useful information for your family members.
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Munster P, "My Father's Fight Against the Breast-Cancer Gene." The Wall Street Journal. September 28, 2018.
Munster P. Twisting Fate: My Journey with —from Breast Cancer Doctor to Patient and Back".
FORCE receives funding from industry sponsors, including companies that manufacture cancer drugs, tests and devices. All XRAYS articles are written independently of any sponsor and are reviewed by members of our Scientific Advisory Board prior to publication to assure scientific integrity.
This article is relevant for:
People with an inherited mutation linked to cancer
This article is also relevant for:
People with a genetic mutation linked to cancer risk
Pancreatic cancer survivors
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The National Comprehensive Cancer Network has guidelines on who should undergo genetic counseling and testing. The NCCN recommends genetic counseling and testing for anyone diagnosed with pancreatic cancer, using a comprehensive gene panel for .
National Comprehensive Cancer Network (NCCN) Guidelines
The NCCN recommends the following screening for people at increased risk for pancreatic cancer:
- Discuss the benefits and risks of screening with their doctor. Screening should be performed by a facility that is experienced with screening for pancreatic cancer. The recommended age for considering screening depends on a person’s family history of pancreatic cancer and varies by type of gene mutation.
- Consider screening with magnetic resonance cholangiopancreatography (MRCP) and/or endoscopic (EUS)
- Consider participating in a pancreatic cancer screening study
NCCN recommends that people with inherited mutations in the following genes (with or without a family history of cancer) "consider pancreatic cancer screening" with MRCP or EUS:
- : (): Consider pancreatic cancer screening by MRCP or EUS every 1-2 years beginning at age 30-35 or 10 years younger than the earliest pancreatic cancer in the family.
- CDKN2A: Consider pancreatic cancer screening beginning at age 40 or 10 years earlier than the earliest pancreatic cancer diagnosis in the family.
NCCN guidelines recommend that people with an inherited mutation in one of the following genes and a family history of cancer "consider pancreatic cancer screening" with MRCP or EUS beginning at age 50 or 10 years earlier than the earliest pancreatic cancer diagnosis in the family: , , , , , , , , .
NCCN does not currently recommend pancreatic cancer screening for people with the above mutations who do not have a family history of cancer.
American Society for Gastrointestinal Endoscopy (ASGE) Guidelines
In February 2022, the ASGE released updated guidelines on pancreatic cancer screening for people with a or mutation. These guidelines recommended:
- All patients with a mutation regardless of family history of pancreatic cancer, should undergo annual screening for pancreatic cancer with MRI/MRCP or EUS beginning at age 50 (or 10 years earlier than the earliest pancreatic cancer in the family).
- How do my genetic test results impact my risk of pancreatic cancer and other cancers?
- Given my risk for pancreatic cance, should I have pancreatic cancer screening?
- As a pancreatic cancer patient, am I eligible for genetic testing?
- Can you refer me for genetic counseling?
- Are there any clinical trials that I qualify for?
The following are studies looking at risk management for pancreatic cancer:
- NCT03250078: A Pancreatic Cancer Screening Study in Hereditary High-Risk Individuals. The main goal of this study is to screen and detect pancreatic cancer and precursor lesions in individuals with a strong family history or genetic predisposition to pancreatic cancer. Magnetic Resonance Imaging and Magnetic cholangiopancreatography (MRI/MRCP) will be utilized to screen for pancreatic cancer or precursor lesions.
- NCT02478892: Preliminary Evaluation of Screening for Pancreatic Cancer in Patients With Inherited Genetic Risk. The study is a , observational study evaluating the utility of endoscopic or for the identification of preneoplastic and neoplastic pancreatic lesions in patients at high risk for pancreatic cancer, specifically those with BRCA1/2, or mutations.
- NCT03568630: Blood Markers of Early Pancreas Cancer. Identifying biomarkers of early pancreatic ductal () could facilitate screening for individuals at higher-than-average risk, expedite the diagnosis in individuals with symptoms and substantially improve an individual's chance of surviving the disease.
- NCT02206360: Pancreatic Cancer Early Detection Program (PCEDP). This is a study looking at esophageal as a screening for pancreatic cancer in high risk individuals.
- NCT03250078: A Pancreatic Cancer Screening Study in Hereditary High Risk Individuals. The goal is to screen for pancreatic cancer in individuals with a strong family history or genetic risk using Magnetic Resonance Imaging and Magnetic cholangiopancreatography (MRI/MRCP).
A number of other clinical trials for pancreatic cancer screening and prevention may be found here.
The following are studies enrolling people diagnosed with pancreatic cancer:
- NCT04548752: Adding Pembrolizumab to Olaparib to Treat Pancreatic Cancer in People with an Inherited Mutation. This study is researching whether adding the drug Pembrolizumab (Keytruda) to the Olaparib (Lynparza) works better than olaparib alone for treating pancreatic cancer in people with an inherited or mutation.
- NCT05252390: NUV-868 Alone and in Combination With PARP Inhibitors in Patients With Advanced .
This study will test how safe and effective the experimental drug NUV-868 is by itself and in combination with a in people with advanced .
- NCT04267939: ATR Inhibitor BAY 1895344 Plus Phase 1b Study in Advanced and Ovarian Cancer. This study will look at how well advanced respond to treatment with the BAY1895344 combined with the niraparib.
- NCT04493060: Treating Pancreatic Cancer with an Inherited or Tumor BRCA1/2 or Mutation with and Dostarlimab. This study looks at how well the and the drug dostarlimab work together in treating patients with pancreatic cancer, who have an inherited or tumor mutation in , , , , , or .
- NCT03337087: Treating Pancreatic, Colorectal, Gastroesophageal, or Biliary Cancer with Chemotherapy and This study will look at how the works with chemotherapy to treat people with pancreatic, colorectal, gastroesophageal or biliary cancer. The study will measure the best dose and look at the side effects of this combination of drugs.
- NCT04150042: SHARON: A Clinical Trial for Cancer With an Inherited or Mutation Using Chemotherapy and Patients’ Own Stem Cells. The purpose of this study is to see whether the combination of melphalan, BCNU, vitamin B12b, and vitamin C, followed by autologous (self) bone marrow stem cell infusion, is safe and effective for treating patients with advanced pancreatic cancer or 4, breast cancer who have a , or inherited mutation.
- NCT04666740: Pembrolizumab and Olaparib for Pancreatic Cancer with or Exceptional Response to Platinum Chemotherapy. This is a study for people diagnosed with pancreatic cancer with a tumor test result called HRD-positive, or whose disease has responded well to first-line or second-line platinum therapy. The study will look at whether combining the drug pembrolizumab and the olaparib is a more effective treatment for this cancer than olaparib alone.
- NCT04858334: Olaparib or in Patients with Surgically Removed Pancreatic Cancer who have a , or Mutation (APOLLO). The purpose of EA2192 / APOLLO is to compare the usual approach (observation) to treatment for one year with olaparib, in patients with a , or mutation.
- NCT04657068: Treatment with ATR Inhibitor for Advanced or Solid Tumors. This study will look at how well a new oral known as an ATR inhibitor works on advanced or solid tumors with mutations in genes linked to damage repair. The study will look at the response to treatment with the drug ART0380 in combination with the chemotherapy agent, gemcitabine.
- NCT04550494: Treating Solid Tumors with an Inherited or Acquired Gene Mutation Using the Talazoparib. This study is looking whether the drug (also known as ) is safe and effective for treating people with advanced breast, gastric, ovarian, pancreatic, or other with an inherited mutation or an acquired mutation in certain repair genes, such as , , , , and others.
Other clinical trials for people with pancreatic cancer can be found here.
The following organizations offer peer support services for people with or at high risk for pancreatic cancer:
- FORCE peer support
- Our Message Boards allow people to connect with others who share their situation. Once registered, you can post on the Diagnosed With Cancer board to connect with other people who have been diagnosed.
- Peer Navigation Program will match you with a volunteer who shares your mutation and situation.
- Private Facebook Group
- Virtual and in-person support meetings
- Join a Zoom community group meeting.
- American Sign Language
- People of Color
- Let's Win PC
- The Healing NET Foundation is a nonprofit organization for people with neuroendocrine cancers.
- The Neuroendocrine Cancer Awareness Network (NCAN) is a non-profit organization dedicated to raising awareness of neuroendocrine cancer and providing support for caregivers and people with NETs.