VIPoma is a rare neuroendocrine tumour that primarily affects the pancreas, producing excessive levels of vasoactive intestinal peptide (VIP). This condition, though uncommon, can have significant effects on the body’s electrolyte balance and overall health. These tumours present a distinctive set of symptoms collectively known as Verner-Morrison syndrome, pancreatic cholera, or watery diarrhoea syndrome.
Our latest article related to neuroendocrine cancer covers all of these facets concerning VIPoma, plus insights into research and future developments in addressing the condition.
Neuroendocrine Cancer Australia (NECA), is dedicated to assisting individuals with VIPoma and their families as well as other neuroendocrine tumours (NETs). NECA offers a wealth of resources, educational programs, and advocacy efforts aimed at deepening the understanding of NETs, improving patient care, and encouraging research advancements. Patients diagnosed with VIPoma, can engage with NECA’s comprehensive support and information by calling the NET nurse line.
Understanding the causes, symptoms, and diagnostic approaches is essential for effective management and treatment of VIPoma. So firstly, what is VIP?
Vasoactive intestinal peptide (VIP) is a hormone produced by neuroendocrine cells that play multiple roles in the body, including regulating smooth muscle activity, epithelial cell secretion, and dilation of blood vessels.
VIPoma is a notably rare form of neuroendocrine tumour. Specific Australian statistics are not commonly available due to the rarity of the condition. Generally, VIPomas constitute only a minor portion of all pancreatic neuroendocrine tumours (pNETs), which are also uncommon. While pNETs are more frequently diagnosed in males and typically found in individuals over the age of 55, they can occur in people of any age and gender.
The exact cause of VIPoma is not well understood, but like many neuroendocrine tumours, genetic factors and random mutations in neuroendocrine cells may play a role. While most VIPomas are sporadic, some cases are associated with genetic syndromes such as Multiple Endocrine Neoplasia type 1 (MEN1).
VIPoma dramatically affects the body by causing the overproduction of VIP, which in turn increases the secretion of water and electrolytes into the intestines and inhibits the absorption of water from the intestines. This leads to severe chronic watery diarrhoea and subsequent dehydration, electrolyte imbalances, and acid-base disturbances.
The primary symptom of VIPoma is copious watery diarrhoea, which can lead to dehydration and weight loss. If symptoms are left untreated, the electrolyte imbalances caused by dehydration can become life-threatening.
Other symptoms may include:
The symptoms of VIPoma are often similar to those seen in more common gastrointestinal conditions, leading to potential misdiagnosis or delays in identifying the correct underlying cause.
Diagnosing VIPoma poses significant challenges, primarily due to its rarity and the ambiguous nature of its clinical presentation. The non-specific symptoms can vary widely among patients, complicating the diagnostic process further.
This necessitates a high degree of clinical suspicion and often requires a series of sophisticated tests, including hormonal assays and imaging studies, to accurately diagnose this rare neuroendocrine tumour.
The diagnosis of VIPoma starts with a detailed medical history and a thorough physical examination. Physicians assess for the characteristic symptoms of watery diarrhoea and dehydration, alongside a history of the symptoms’ progression. A physical exam can provide further clues and help rule out other conditions.
Initial blood tests are crucial and focus on assessing electrolyte levels, kidney function, and acid-base status to gauge the impact of the diarrhoea. Elevated levels of potassium and chloride can indicate the presence of a VIPoma. Imaging studies like CT scans, MRI, and ultrasonography of the abdomen are employed to locate the tumour and assess its size and spread.
The definitive diagnosis of VIPoma involves measuring VIP levels in the blood. Significantly elevated VIP levels, typically several times higher than normal, confirm the diagnosis. In some cases, a specialised test called somatostatin receptor scintigraphy (SRS) is used to visualise the tumour and any potential metastases.
Managing VIPoma effectively requires a multidisciplinary approach involving gastroenterologists, oncologists, surgeons, and other specialists. The primary treatment is the surgical removal of the tumour, which can often result in a cure. For inoperable tumours or metastatic disease, medication to control symptoms and slow tumour growth is necessary. These may include somatostatin analogs, which reduce VIP secretion and alleviate symptoms.
Ongoing monitoring is crucial for managing VIPoma, as it helps ensure there is no recurrence of the tumour and allows for timely intervention if complications arise. Patient education on hydration and diet management is also critical to mitigate the symptoms and improve quality of life.
VIPoma requires a comprehensive treatment approach tailored to individual patient needs. Understanding and managing this condition involves a combination of:
Surgery is the cornerstone of treatment for VIPoma, particularly when the tumour is localised and has not spread. The primary goal of surgical intervention is to remove the tumour completely, which can potentially cure the patient. In cases where the tumour is inoperable or has metastasised, debulking surgery may be performed to remove as much of the tumour mass as possible, thereby reducing symptoms and improving quality of life.
For patients where surgery is not an option or for those dealing with recurrent symptoms, medications play a critical role. Drugs such as somatostatin analogs (e.g., octreotide or lanreotide) are used to control the levels of vasoactive intestinal peptide (VIP) and alleviate the severe diarrheal symptoms associated with the condition. These medications can inhibit the growth of neuroendocrine tumours and are a mainstay of non-surgical treatment.
In addition to surgery and medications, other therapeutic strategies may include peptide receptor radionuclide therapy (PRRT), which targets tumour cells with radioactive substances, and targeted therapy, which focuses on specific genes and proteins that contribute to tumour growth. Chemotherapy may be considered in advanced cases, particularly when the tumour is aggressive or poorly responsive to other treatments.
Living with VIPoma often involves managing chronic symptoms such as severe diarrhoea and electrolyte imbalances, requiring ongoing medical attention and lifestyle adjustments to maintain quality of life. However, with help from medical professionals, patients can often vastly improve their quality of life.
Dietary management is crucial for those living with VIPoma. A diet low in fat and rich in nutrients that can be easily absorbed is typically recommended to manage diarrhoea and prevent malnutrition. Here are some tips to effectively manage one’s diet:
Given the complexity of dietary management in VIPoma, consultation with a dietitian who specialises in gastrointestinal disorders is beneficial. A dietitian can tailor dietary recommendations to suit individual nutritional needs, helping to manage symptoms effectively and improve overall health outcomes.
Regular monitoring of symptoms is vital to adjust treatment plans promptly and effectively. Patients should keep track of their diarrhoea frequency, hydration status, and any new symptoms that may arise. This ongoing monitoring helps in early detection of complications and in assessing the effectiveness of the current treatment strategy.
Supportive care for VIPoma patients includes not only medical treatment but also psychological and emotional support. Neuroendocrine Cancer Australia’s website can offer valuable information, resources and support networks. These resources include patient education materials, support groups, and counselling services to help patients and their families navigate the challenges of living with a chronic condition. For more personalised information VIPoma patients and their families can contact the NET nurse for free and confidential support.
Research and future directions in the treatment of VIPoma are focused on developing targeted therapies and refining diagnostic techniques to improve detection rates and tailor treatments more effectively to individual patients’ needs.
Research into VIPoma is ongoing, with numerous studies and clinical trials underway to find more effective treatments and understand the underlying mechanisms of the disease. These studies often explore new drugs, innovative surgical techniques, and novel therapeutic approaches that could potentially improve patient outcomes.
The future of VIPoma treatment looks promising with several potential breakthroughs on the horizon. Advances in genetic and molecular research may lead to more personalised medicine approaches, targeting specific pathways involved in tumour growth and progression.
A study reviewed records from 1990 to 2016 of patients with VIPoma, treated in seven French expert centres to evaluate the effects of treatments on secretory syndrome, tumour burden, and survival. Most of the 22 patients included had metastatic grade 2 tumours.
The study found that surgical excision of non-metastatic VIPoma effectively managed the secretory syndrome, though metastatic recurrences were common. For metastatic VIPoma, treatments including surgery, chemotherapy, and sunitinib showed the best combination of antitumour and antisecretory efficacy.
The five-year overall survival rate stood at 63.6%, with the prognosis negatively impacted by higher Ki-67 indices and plasma VIP levels. The results underscored that while surgical resection of localised VIPoma is effective, it is rarely curative, and highlighted the therapeutic options for metastatic VIPoma that best manage both tumour control and symptom relief.
Additionally, improvements in imaging and diagnostic techniques will likely enhance the early detection and precise localisation of tumours, which is crucial for effective treatment planning.
In conclusion, although VIPoma is a rare condition, understanding its causes, effects, symptoms, and diagnostic processes is vital for effective treatment and management. With the right medical approach and ongoing care, individuals with VIPoma can lead a controlled and improved quality of life. As research continues and medical technology advances, the prospects for successfully managing VIPoma continue to improve, offering hope to those affected by this challenging condition.
For people diagnosed with VIPoma, further support and information is available by calling the NeuroEndocrine Cancer Australia NET nurse line.
