Katherine Braier, a free-spirited human – a former teacher and social worker – started writing a blog about her experience of living with a diagnosis of Stage 4 Pancreatic Cancer which she described as a physical hell’ in 2019. Her struggles with Pancreatic Cancer and failure to respond to treatments led her to the darkest realms of reality – her cancer was fatal and its agony might only end with the end of her beautiful life – and it did, in 2021.
Kathy’s last blog is reminiscent of her love for her family, and her crushing grief for living with her misery. But, we remember Kathy as a warrior who became a martyr. Kathy didn’t start her blog to be a spokesperson for people with Pancreatic cancer, but rather to pen her imaginary thoughts, find comfort and solace in her last days of life, and spread that love beyond to the survivors and sufferers.
Most of the cases diagnosed with Pancreatic Cancer witness a heartbreaking story as they near the end of their lives. But, does it always have to be this way?
What is Pancreas?
The pancreas is an exocrine and endocrine gland found in the upper abdomen which secretes hormones primarily for aiding in the digestion of food. It is situated along the first part of the small intestine, also known as the duodenum, and the spleen, just behind the stomach.
The endocrine pancreas secretes Insulin, a hormone required to balance the blood sugar level, directly into the bloodstream of the body. Its exocrine function, on the other hand, plays a vital role in the digestion of sugars, fats, and protein, by releasing digestive enzymes via a duct into the duodenum.
What Is Pancreatic Cancer (ICD-10 code: C25.9 Malignant neoplasm: Pancreas)
The pancreas comprises 4 parts: the head, neck, body, and tail. The head of the pancreas is the most common location of pancreatic cancer, accounting for over 60% of cases.
Despite being rare, Pancreatic Cancer is notoriously ranked as the 4th most aggressive, malignant cancer among all cancers today. It is one of the most life-threatening and chronically fatal cancers that carry a poor prognosis and still warrants an in-depth study to understand its etiology completely.
According to the American Cancer Society, pancreatic cancer affects around 3% of the entire U.S population, with 7% of such ending in untimely deaths. Pancreatic cancer is twice as common in men, but that doesn’t rule out the possibility of its occurrence in women, though.
What Causes Pancreatic Cancer?
Pancreatic cancer is most commonly diagnosed in the elderly aged 65 – 80 years old, but can also affect people in their third or fifth decades. When a person is talking about their diagnosis of pancreatic cancer, they are usually referring to Pancreatic Ductal Carcinoma.
Pancreatic cancer is largely due to DNA mutations which cause the pancreatic cells to grow out of proportion rapidly. Mutations can be inherited, which means they can be passed down from one family to the other or acquired. These DNA mutations can lead the pancreatic cells to grow out of control, leading to distant and metastatic disease.
Despite inheriting the mutated gene, many individuals might not express the disease due to incomplete or reduced penetrance of the genetic trait.
- Penetrance refers to the likelihood and probability of the genetic trait being expressed in an individual. For instance, a family member with pancreatic cancer can pass down the gene to the next generation, who despite carrying the genetic mutation, might not exhibit any signs or symptoms of the disease.
- In addition, pancreatic cancer also carries a variable degree of expressivity of symptoms. For instance, the symptoms of pancreatic cancer in an individual might be more grave and serious as compared to another relatively well patient with the disease.
Cigarette smoking, diabetes mellitus (sugar), obesity, alcohol intake, and a diet high in fats and cholesterol, are also categorized as important risk factors for developing pancreatic cancer.
- Excessive alcohol intake can lead to chronic inflammation of the pancreas, otherwise known as pancreatitis, which can later on go on to develop pancreatic cancer.
- While smoking and alcohol consumption are two modifiable risk factors that if stopped, can prevent the development or progression of the disease.
But, other risk factors such as race, age, ethnicity, and gender also contribute to the development of pancreatic cancer. Pancreatic cancer is more prevalent in males, blacks, and Ashkenazi Jews, and should prompt further investigations toward a provisional diagnosis if symptoms match those of the disease.
Types of Pancreatic Cancer
Pancreatic cancer is broadly classified into two types: exocrine pancreatic cancer and neuroendocrine pancreatic cancer.
- Pancreatic Ductal Adenocarcinoma is the most common, rapidly growing, and lethal type of pancreatic cancer. It usually arises from the pancreatic ducts and lining, but can also originate from the pancreatic cells which produce digestive enzymes for digestion.
- Neuroendocrine pancreatic cancer, also known as islet cell tumors, arises from the cells of the pancreas which produce endocrine hormones for the regulation of metabolism in the body. Islet cell tumors are relatively rare and occur in about 5% of people diagnosed with pancreatic cancer.
- Colloid Carcinoma, otherwise known as mucinous or gelatinous non-cystic carcinoma, is a rare subtype of pancreatic ductal carcinoma which carries a relatively superior prognosis and better long-term survival rates.
- Other types of pancreatic cancer include Adenosquamous Carcinoma and Squamous Carcinoma which are rare but highly aggressive cancers with poor prognoses.
Pancreatic Cancer Symptoms: What Are The Early Warning Signs Of Pancreatic Cancer?
Symptoms may vary with the location of the tumor in the pancreas, but most commonly, they consist of the following:
- Epigastric pain:
- Left upper abdominal or epigastric pain is the first and most common presenting symptom at diagnosis, that is when the disease is in its advanced stage.
- The abdominal pain radiates to the back and is relieved by bending or sitting forward.
- Pain can either be continuous or intermittent, that is it may be constant or occur in intervals. Some pancreatic tumors can also be painless, prompting cancer to go unseen prior to diagnosis.
- At least 71% of cases report severe abdominal pain especially if the tumor is located in the body or tail of the pancreas. Tumors in the head of the pancreas can be painless, but the pain is reported in over 70% of people with pancreatic adenocarcinoma.
- Vague Symptoms
- Abdominal swelling or mass
- Jaundice (yellowing of the skin and sclera of the eyes)
- Enlarged liver
- Itchiness (also known as pruritus)
- Intestinal or gastric bleeding
- Blood clots
- Dysphagia (difficulty in swallowing)
- Dark Urine
- Pale stools with fat
- Newly diagnosed diabetes or worsening of diabetes making it difficult to manage
Diagnosis of Pancreatic Cancer
Initially, endoscopic ultrasound with biopsy can help to identify pancreatic lesions. Further imaging tests such as abdominal CT scan, MRI, or laparoscopy are also otherwise used for identifying patients with pancreatic cancer.
Definitive diagnosis is made by Helical CT (Triphasic contrast-enhanced Abdominal CT) which can detect pancreatic cancer as well as aid in staging with 89-97% accuracy rates.
Serum Cancer antigen, CA 19-9, is a tumor marker, which can be used to follow up with patients with progressive disease. It is also present at diagnosis, as the cancer is usually identified in its late stages. But a normal CA 19-9 level does not necessarily exclude the absence of progression of the disease or its recurrence in treated patients with pancreatic cancer.
Stages of Pancreatic Cancer
Staging of cancer aims to denote the progression and spread of the disease. The TNM staging system is most commonly used and can assist in assessing whether the cancer is restricted within the pancreas or has metastasized and spread to nearby and distant organs.
The TNM staging system is divided into three parts:
- T: denotes the size of the primary tumor and its spread or extension beyond the pancreas. T is further divided into 5 groups from 0 – 4, and assesses the size of tumors in cms.
- N: denotes the involvement of nearby or distant lymph nodes. For instance, did cancer spread to the regional or distant lymph nodes?
- M: denotes the progression or metastasis of cancer to distant organs of the body. The lungs, liver, and abdominal lining are more commonly affected than any other organ. Pancreatic cancer can, however, expand to other sites such as the brain and bones in the body.
Each of the TNM categories is divided into subtypes depending on the degree of extension of cancer, but are too complex and detail-oriented. The TNM stages are further grouped into stage groups which form the numerical staging system. This system helps doctors declare whether the disease is in its ‘early’ or ‘advanced’ stages, and decide the most adequate treatment if any.
- STAGE 0: also known as carcinoma in situ, refers to cancerous cells which are restricted to the lining or superficial cell layers of the pancreas. Cancer can neither be evaluated on any imaging tests nor assessed by the naked eye.
- STAGE 1: refers to cancerous cells which grow locally within the structure of the pancreas. This stage is subdivided into STAGE 1A and 1B as per the size of the cancerous mass.
- Stage 1A: refers to a cancerous mass that is less or equal to 2 cm in size.
- Stage 1B refers to a tumor that ranges from 2 – 4 cm in size.
- STAGE II: refers to the local spread of pancreatic cancer, in which the growth of cancerous cells expands beyond the pancreas and may affect regional lymph nodes.
- Stage 11a: cancerous cells invade neighboring tissues but have not yet spread to nerves, blood vessels, lymph nodes, or distant sites.
- Stage 11b: typically stage 11a with the involvement of nearby lymph nodes.
- STAGE III: The cancerous cells may occupy the regional lymph nodes, and go on to further invade surrounding nerves such as the celiac plexus in the abdomen, or major blood vessels such as the portal vein.
- STAGE IV: This is an end-stage disease, as cancer has metastasized to distant organs such as the lungs, liver, and abdominal cavity.
Treatment of Pancreatic Cancer
Staging of pancreatic cancer by the TNM and Numerical Staging System aims to elaborate the course of treatment and whether the patient can benefit from surgery or ongoing clinical trials.
- Resectable tumors: Ideally, 10% of noninvasive pancreatic cancers (Stage 0 – II) can be removed by surgery after diagnosis, but there are exceptions.
- Unresectable tumors involve cancerous cells which have invaded the main blood vessels or nerves of the body and its evidence is elaborated on imaging tests in extent (Stage III)
- Metastatic: Usually Stage 4, rendering surgery to remove cancer impossible.
Why is Pancreatic Cancer Deadly?
Pancreatic cancer is notorious for displaying vague symptoms which usually go unseen and undiagnosed until after the disease takes a metastatic course. Vague symptoms may be present at least a year before diagnosis which if diagnosed in their early stages, could help prevent the progression of pancreatic cancer to advanced disease.
Unfortunately, there is no screening modality for pancreatic cancer to catch the disease in its early stages. Genetic evidence and poor lifestyle can help create a ground for establishing further investigations toward getting a diagnosis. Genetics pose a very broad window for screening, but the BRCA 2 gene can be found in familial cases of pancreatic cancer.
Symptoms of jaundice, itching, dark urine, and pale stools along with abdominal pain radiating to the back must warrant an urgent referral to a gastroenterologist as it may be indicative of advanced stage or stage 4 pancreatic cancer.
Pancreatic Cancer Treatment
The time of diagnosis and stage of pancreatic cancer establish the course of treatment. Since pancreatic cancer is usually diagnosed in Stage 4, its response to chemotherapy and radiotherapy varies. Other conventionally therapeutic treatments for cancer such as immunotherapy have been useless in pancreatic cancer.
Resectable cancers are often managed with surgery, combination chemotherapy, and radiotherapy to improve survival rates as surgery alone cannot remove locally advanced tumors completely.
- Chemotherapy agents include 5-Fluorouracil and Gemcitabine which are given intravenously.
- Oral Capecitabine often replaces 5-FU if chemotherapy is given along with radiation.
There are a number of chemotherapy agents for metastatic cancer, but the most commonly used is Gemcitabine.
- FOLFIRINOX is a recent chemotherapy agent which comprises 4 drugs including FOLinic acid, Fluorouracil, IRINotecan HCL, and OXaliplatin, and has been approved by the FDA for the treatment of metastatic pancreatic cancer.
- Patients with the BRCA gene mutation in metastatic pancreatic cancer can be treated with olaparib (Lynparza) after an adequate and positive response to chemotherapy.
Other treatments such as pain
5-year Survival Rate of Pancreatic Cancer
Overall, the 5-year survival rate of pancreatic cancer varies on a patient-to-patient basis, and in accordance with the presenting symptoms and stage of the disease.
Pancreatic cancer, usually diagnosed in its last stages, more commonly Stage 4, carries a more vicious course with a 5-year survival rate of only 3%. However, if caught in its early stages and treated with surgery, the 5-year survival rate can be as optimistic as 42%. Unfortunately, about 13% of patients with pancreatic cancer present this early.
Men have, however, shown to have a poor prognosis as compared to women with pancreatic cancer. In 2017, women aged 15-49 demonstrated a 5-year survival rate of 26% whereas men of the same age group had only 17%. Similarly, patients aged 80-99 showed a 5-year survival rate of only 2%, regardless of gender.
But, generally, pancreatic cancer carries a poor prognosis with a general 5-year survival rate of only 11% (or roughly 5-15%).
When to See Your Doctor for Pancreatic Cancer?
A positive family history with evidence of genetic syndromes or long-standing vague symptoms comprising nausea, vomiting, and altered bowel habits, should prompt a visit to your general practitioner. If red flags are identified, your general practitioner may further refer you to a gastroenterologist for further workup.
This blog is for informational & educational purposes only, and does not intend to substitute any professional medical advice or consultation. For any health related concerns, please consult with your physician, or call 911.
About The AuthorDr. Syra Hanif M.D.
Board Certified Primary Care Physician
Dr. Syra Hanif is a board-certified Primary Care Physician (PCP) dedicated to providing compassionate, patient-centered healthcare.Read More