The world mourned the loss of Aretha Franklin, the undisputed Queen of Soul, in August 2018. While her passing was deeply felt, many sought to understand the illness that led to her death. A year after her passing, Dr. Philip A. Philip, a Detroit-based oncologist who treated Aretha Franklin, provided insights into the rare cancer she battled: pancreatic neuroendocrine tumors. In this revealing interview, originally published by the Detroit Free Press and revisited to shed light on Aretha Franklin Diagnosis, Dr. Philip, head of gastrointestinal and neuroendocrine oncology at the Barbara Ann Karmanos Cancer Institute and professor at Wayne State University School of Medicine, clarifies the nature of this disease, its treatments, and its differences from more common pancreatic cancers.
Alt text: Aretha Franklin’s final Detroit concert appearance, where she requested prayers from the audience amidst her health battle with pancreatic neuroendocrine tumor.
What are Pancreatic Neuroendocrine Tumors?
Question: Could you explain what pancreatic neuroendocrine tumors are, and how they differ from the more prevalent type of pancreatic cancer, adenocarcinoma?
Answer: Pancreatic neuroendocrine tumors, or pNETs, originate from neuroendocrine cells. These specialized cells are found throughout the body, but in this context, we are focusing on those within the pancreas. It’s crucial to distinguish pNETs from the more common form of pancreatic cancer, pancreatic adenocarcinoma. In fact, adenocarcinomas constitute over 95% of pancreatic cancer cases. They arise from different cells within the pancreas and represent a completely distinct disease. Therefore, when we generally refer to pancreatic cancer, we are typically talking about adenocarcinoma. It’s essential to understand that Ms. Franklin’s diagnosis, concerning Aretha Franklin diagnosis, was specifically pancreatic neuroendocrine tumor, not the common pancreatic adenocarcinoma.
Treatment and Life Expectancy Differences
Question: What are the key differences in treatment approaches and life expectancy between pancreatic adenocarcinoma and pancreatic neuroendocrine tumors?
Answer: For localized disease, meaning the cancer is confined to the pancreas and hasn’t spread, surgery is the primary treatment for both pancreatic adenocarcinoma and pancreatic neuroendocrine tumors. In such cases, surgery offers the potential for a cure. However, the prognosis differs significantly. Patients with pancreatic neuroendocrine tumors generally have a much better chance of survival compared to those with adenocarcinoma, even when the disease is advanced. Furthermore, there is a broader range of treatment options available for advanced pancreatic neuroendocrine tumors than for pancreatic adenocarcinoma. This, along with the typically less aggressive nature of pNETs, contributes to a more favorable outlook. Patients with pNETs can live for many years – five, six, seven, eight years, or even longer – with appropriate management. While this isn’t guaranteed for every patient, it represents a considerably more positive scenario than with pancreatic adenocarcinoma.
Symptom Presentation: Are There Differences?
Question: Are the symptoms of pancreatic adenocarcinoma and pancreatic neuroendocrine tumors distinct, or do they manifest in similar ways?
Answer: The symptoms associated with pancreatic adenocarcinoma, particularly in advanced stages, tend to be more severe and progress more rapidly than those of neuroendocrine tumors. Pancreatic neuroendocrine tumors often grow slowly, resulting in less immediate and dramatic impact on the body, unless the disease reaches a very advanced stage. To illustrate, I recently saw a patient who had been living with pNETs for four years and appeared remarkably healthy. In contrast, a patient with advanced pancreatic adenocarcinoma that has metastasized to the liver would typically present as very ill and emaciated. The patient with neuroendocrine tumors I mentioned had not experienced significant weight loss even four years after being diagnosed with stage-four disease.
Aretha Franklin’s Appearance and Treatment Personalization
Question: Aretha Franklin did not appear to be emaciated by cancer. Was this due to the specific type of cancer and the treatment approach she received?
Answer: Because patients with pancreatic neuroendocrine tumors often have a longer life expectancy, personalized treatment planning is critical. Factors such as age, lifestyle, daily activities, and pre-existing health conditions are all carefully considered when designing a treatment strategy for each patient. This individualized approach contrasts with the treatment of pancreatic adenocarcinoma, where limited options often restrict the ability to tailor treatment to individual patient characteristics and preferences. Moreover, significant advancements have been made in recent years in the treatment of pancreatic neuroendocrine tumors, with newer therapies becoming available. One notable example is Lutathera, a targeted radiopharmaceutical therapy. Previously, patients had to travel to Europe to access this treatment; however, it is now available in the United States, with our institution being among the first to offer it in Michigan starting in 2017.
Recognizing Decline in Pancreatic Neuroendocrine Tumors
Question: If individuals with neuroendocrine tumors can live for extended periods and may not exhibit outward signs of illness, what indicators suggest that their condition is worsening or approaching a critical stage?
Answer: When pancreatic neuroendocrine tumor progresses to a very advanced stage, key symptoms to watch for include increasing pain, noticeable wasting or weight loss, and progressive weakness. A significant decline in energy levels, changes in appetite leading to anorexia, and abdominal distension or bloating are also indicative of disease progression and a worsening prognosis. These signs suggest that the cancer is advancing more rapidly and impacting the patient’s overall health more significantly.
Average Life Expectancy: Understanding the Statistics
Question: What is the average life expectancy for someone diagnosed with pancreatic neuroendocrine tumors?
Answer: When discussing average life expectancy, it’s crucial to remember that it’s just an average. Patient experiences vary considerably. Generally, the average life expectancy is around five to six years, but this is merely a statistical average. It’s vital to emphasize that survival time with this disease is typically measured in years, not months or fractions of years, as is often the case with pancreatic adenocarcinoma. I have observed patients who have lived for nine, ten years, or even longer, maintaining a good quality of life without significant illness during that time. Conversely, some patients may have a less favorable course, with survival times shorter than five years. The disease’s biology is unique in each individual, influencing the course of the illness. As our scientific and medical understanding expands, we are increasingly utilizing molecular tests to better predict which patients are likely to have a more favorable outcome and those who may face a less positive prognosis.
Risk Factors and Genetic Predisposition
Question: What are the known risk factors for pancreatic neuroendocrine tumors? Is there a genetic component that predisposes individuals to this type of cancer?
Answer: In most cases, the exact cause of pancreatic neuroendocrine tumors in an individual remains unknown. The majority of patients we see have what we classify as sporadic cancer, meaning it arises without a clear hereditary link. However, a small subset of patients, less than 10%, have a genetic predisposition. These individuals may have well-defined genetic syndromes characterized by specific gene mutations that increase their susceptibility to developing pancreatic neuroendocrine tumors. These genetic syndromes, such as MEN-1 and neurofibromatosis-related syndromes, can also involve tumors in other organs beyond the pancreas. It’s important to note that these genetic predispositions are quite rare.
Screening and Early Detection Challenges
Question: Is there an effective screening method for pancreatic neuroendocrine tumors that can facilitate early detection?
Answer: Early detection is critical, as many patients are diagnosed at later stages of the disease. Unfortunately, there is currently no reliable screening test suitable for widespread use in the general population for pancreatic neuroendocrine tumors. Unlike colonoscopy for colon cancer or mammography for breast cancer, we lack a broadly applicable screening tool for either type of pancreatic cancer. This absence of effective screening contributes significantly to the frequency of late-stage diagnoses. While individuals with known genetic mutations that increase their risk may be eligible for specialized screenings like ultrasound or MRI, these are not routinely recommended for the general population.
Symptoms of Pancreatic Neuroendocrine Tumors
Question: What are the typical symptoms that might indicate the presence of pancreatic neuroendocrine tumors?
Answer: The symptoms of pancreatic neuroendocrine tumors can sometimes overlap with those of pancreatic adenocarcinoma. For instance, if a tumor obstructs the bile duct, it can lead to jaundice, characterized by yellowing of the eyes and skin. Weight loss can also be a presenting symptom. However, in general, symptoms like pain and weight loss tend to be less pronounced and develop more gradually in patients with pancreatic neuroendocrine tumors compared to those with pancreatic adenocarcinoma. A distinctive feature of pancreatic neuroendocrine tumors is their potential to secrete hormones into the bloodstream in a subset of patients, though this occurs in less than 50% of cases. These excess hormones can lead to specific syndromes. For example, a tumor producing excessive insulin, known as an insulinoma, can cause hypoglycemia, resulting in symptoms like fainting or low blood sugar episodes. Interestingly, pancreatic neuroendocrine tumors are sometimes discovered incidentally during imaging scans performed for unrelated reasons, such as investigating kidney stones. This incidental finding can lead to diagnosis at an early, more treatable stage where curative surgery is an option. For individuals in middle age or older, any persistent or unusual symptoms warrant prompt medical attention. Unexplained weight loss, progressive fatigue, and a family history of cancer should heighten vigilance. Specifically, persistent abdominal pain accompanied by weight loss, back pain, and loss of appetite should be thoroughly investigated. If symptoms persist or worsen despite initial medical consultation, seeking a second opinion is advisable. It’s crucial to advocate for oneself and ensure timely and thorough medical evaluation, as delays in diagnosis can sometimes lead to more advanced disease stages. As we age, the importance of paying attention to new or changing symptoms increases, as cancer risk generally rises with age.
Curative Potential of Surgery
Question: Can surgery alone be curative for pancreatic neuroendocrine tumors?
Answer: Yes, surgery can be curative, provided that the cancer is localized to the pancreas and has not spread to other organs.
Surgical Procedures Explained
Question: What types of surgical procedures are performed for pancreatic neuroendocrine tumors?
Answer: The Whipple procedure is commonly performed when tumors are located in the head of the pancreas. Another procedure, distal pancreatectomy, is used for tumors in the tail of the pancreas. Whipple procedures are typically performed as open surgeries, while distal pancreatectomy can sometimes be done laparoscopically, using minimally invasive techniques. The Whipple procedure is a major surgery, and recovery can take several weeks, potentially extending to months, sometimes up to six months. Some patients may experience long-term complications, particularly nutritional issues, following pancreatic surgery, especially Whipple procedures. It requires a highly skilled surgical team and a medical center experienced in managing complex post-operative care.
Rarity and Prevalence of Pancreatic Neuroendocrine Tumors
Question: How rare is pancreatic neuroendocrine tumor as a disease?
Answer: Pancreatic neuroendocrine tumors are considered rare in terms of incidence, meaning the number of new cases diagnosed each year is relatively low. However, they are not as rare in terms of prevalence. This is because patients with pNETs often live longer compared to those with pancreatic adenocarcinoma. Therefore, while fewer new cases are diagnosed annually, there is a growing population of individuals living with pancreatic neuroendocrine tumors for extended periods. So, while the annual incidence is rare, the overall prevalence – the number of people living with the disease – is more substantial than with pancreatic adenocarcinoma.
Treatment Options Beyond Surgery
Question: Beyond surgery, what other treatment options are available for pancreatic neuroendocrine tumors?
Answer: A significant advantage in managing pancreatic neuroendocrine tumors is the availability of a diverse range of treatment options, allowing for tailored approaches based on individual patient circumstances. While surgery remains a cornerstone of treatment, even for some patients with advanced disease, it’s not the only option. In certain cases of advanced disease, surgical removal of liver metastases may be considered, as patients with pNETs often have longer survival horizons, making such interventions potentially beneficial. This is generally not justifiable for pancreatic adenocarcinoma due to its more aggressive nature and poorer prognosis. Liver metastases are a common site of spread for pancreatic neuroendocrine tumors. When surgery on liver metastases is not feasible or appropriate, other strategies to control tumor growth in the liver are employed. These include liver-directed therapies like embolization, which blocks blood supply to the tumor, or radioembolization, which delivers radioactive particles directly to the tumor via the hepatic artery. These procedures can help slow or halt tumor growth and, in some cases, even destroy tumor cells. Targeted therapies, including injectable drugs that target specific molecules on neuroendocrine tumor cells, are also valuable. These agents can help to “switch off” tumor cell growth and proliferation. Oral targeted drugs have also been approved for pNETs. Furthermore, the recent introduction of Lutathera, approved by the FDA in January 2018, has marked a significant advancement. Lutathera has demonstrated remarkable success in extending the lives of patients with certain types of pNETs. In some situations, conventional chemotherapy may also play a role in treatment. Given the complexity of available treatments and the diverse clinical scenarios patients may present with, treatment strategies for pancreatic neuroendocrine tumors are highly individualized. Factors such as quality of life, patient preferences, lifestyle, age, and overall health are all carefully weighed to determine the most appropriate and effective treatment plan for each patient.
Contact Kristen Jordan Shamus: 313-222-5997 or [email protected]. Follow her on Twitter @kristenshamus.
Originally published by Detroit Free Press.
