Cholangiocarcinoma, in one in 4 people affected is discovered by accident
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Cholangiocarcinoma is a rare and difficult to detect disease. So much so that one in 4 cases is discovered by chance, in the course of investigations carried out for other reasons. And this is why even today 70 percent of diagnoses occur at an advanced stage, when the possibilities of treatment are very limited: to date, the 5-year survival is only 17% in men and 15% in women.
Progress has been made in all areas of care in recent years, but it is not enough: they must be accompanied by a cultural change in the approach to the disease. Non-specialist doctors must be made aware, so that they can recognize the first signs of cancer, and a register of reference centers must be established, which can guarantee a multidisciplinary approach with dedicated teams. Furthermore, all patients must undergo genomic profiling, to identify any molecular alterations for the choice of the best therapy. Requests come from clinicians today in a virtual press conference.
The difficulties from diagnosis to treatment
Rare neoplasia, yes, but constantly growing, and every year in Italy about 5400 new cases are estimated. The age of onset of the disease, in parallel with the increase in its incidence, is changing: historically most of the diagnoses concerned people between 60 and 70 years of age, on average, while today it is decreasing. In patients with genetic tumor mutations, in particular, diagnoses often involve young people, 40 to 50 years of age.
Furthermore, one of the complexities of cholangiocarcinoma is that it includes a spectrum of different cancers. In general, however, the scarcity of symptoms and their non-specificity do not allow us to find out in time.
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Risk factors and red flags
The risk factors are also still poorly understood. In recent years, however, the occurrence in the Western world has been increasing, and doctors have noted a correlation with some diseases of the modern lifestyle, such as metabolic syndrome. “The disease certainly correlates with chronic non-alcoholic liver disease, which appears to be a risk factor for intrahepatic cholangiocarcinoma,” says Lorenza Rimassa, Associate Professor of Medical Oncology at Humanitas University, IRCCS Humanitas Research Hospital in Rozzano (Milan).
“Risk factors include metabolic syndrome, obesity, fatty liver and cirrhosis, chronic liver disease, alcohol abuse and cigarette smoking. But, in most cases, it is difficult to identify a specific cause, and there is not yet enough knowledge to implement screening programs. “
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When is it possible to have a suspicion? “If they persist, if the weight loss continues, and with it the difficulty in digesting, the abdominal pain – Rimassa suggests – it is certainly advisable to have a blood test and an abdominal ultrasound. If jaundice appears it is easier for the patient to notice that something is wrong, while as regards the appearance of intrahepatic forms with non-specific and vague symptoms, the role of the family doctor is very important “.
But not only: “Evaluation in a reference center, discussion of the treatment path by a multidisciplinary team and molecular profiling for the search for mutations are the three fundamental steps to ensure the best care for patients with cholangiocarcinoma. It is important – he reiterates. the expert – that a register of centers of reference that treat a high volume of cases every year be established as soon as possible, following the example of the Breast Units for breast cancer “. The multidisciplinary approach and genomic profiling were the focus of the national conference “FIrST-in Colangiocarcinoma”, which took place recently in Naples (the materials of the conference are available on the website www.colangiocarcinoma.net).
The two main types of cancer
Cholangiocarcinoma is a type of primary liver cancer that originates from cholangiocytes, the cells that line the bile ducts, the channels that carry bile from the liver to the intestine. It is distinguished, based on the site of onset, into intrahepatic, if it develops within the liver, and extrahepatic, if it arises from the extrahepatic biliary tract. The first, intrahepatic, is also the most difficult to diagnose.
Inside the liver, in fact, there is no innervation, and until the tumor reaches the capsule that surrounds the organ – and is highly innervated – or grows to the point of compromising the functionality of the liver itself, it is difficult to experience alarming symptoms. or clear. On the other hand, some initial nonspecific symptoms such as abdominal pain, weight loss, digestive difficulties and nausea may occur. In the case of external cholangiocarcinoma, however, this often ends up occluding the biliary tract and causing jaundice, dark urine, whitish stools and itching (due to increased levels of bile salts in the blood).
The role of surgery
All cancers that go by the name of cholangiocarcinomas are infrequent and usually have a poor prognosis. The treatment of first choice is chemotherapy, in some cases associated with immunotherapy, which is not resolutive but helps to control the evolution of the tumor, even if the disease recurs in most patients.
But there is another possibility, much rarer and much more effective, that of surgery. “Surgery, if performed on early stage disease, can have a resolutive outcome,” he explains Alfredo Guglielmi, Full Professor of General and Hepatobiliary Surgery at the University of Verona. “Unfortunately, only 25% of patients are candidates for surgery, which is particularly difficult because it requires the use of advanced techniques, surgeons with specific training, multidisciplinary teams and highly specialized centers.”
With the refinement of surgical techniques, these interventions have become increasingly safer and can guarantee good long-term results. “We proceed with liver resection for cholangiocarcinoma that grows inside the liver – continues the expert – and with pancreatic resection for cancer that develops outside the liver or inside the head of the pancreas. Surgical treatment it aims at the complete removal of the neoplasm. In many cases, precautionary chemotherapy is indicated after the operation “.
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Patients who are candidates for surgery, explains Guglielmi, must be carefully selected. The tumor, in fact, must have net negative margins, that is, it must be able to be removed completely and, to its removal, the removal of the lymph nodes must also be associated to avoid metastases. These patients have a survival of up to five years, which is a very long period, considering that the average survival data is about one year. It is essential, however, to build a multidisciplinary team, consisting of a radiologist, endoscopist, gastroenterologist, oncologist and radiotherapist, in order to choose the most appropriate therapy for the patient and also to establish the treatment before and after surgery.
Looking for genetic mutations
“About half of intrahepatic cholangiocarcinomas have one or more gene mutations, some treatable with molecularly targeted drugs,” he explains. Giancarlo PruneriProfessor of Pathological Anatomy at the University of Milan and Director of the Department of Pathology and Laboratory Medicine of the IRCCS National Cancer Institute of Milan.
“Anatomo-pathological analysis and tumor staging must always be accompanied by the search for mutations, to be performed using new gene sequencing techniques”. Today, in particular, starting from a small sample it is possible to simultaneously identify the mutations of hundreds of genes.
The NGS (Next Generation Sequencing) test is one example: it is able to analyze over 300 gene mutations and can detect molecular alterations from minimal amounts of tissue. One of the most common mutations, present in 10 percent of intrahepatic cholangiocarcinomas, is that of the FGFR2 gene (fusion or rearrangement of the fibroblast growth factor receptor 2) (FGFR2). According to recent statistics, it occurs predominantly in young women.
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The new targeted drug
Before today, all forms of cholangiocarcinoma were treated equally with chemotherapy and immunotherapy. Now a new targeted therapy, available a few months ago in Italy, has proven to be effective in the treatment of advanced cancers with particular genetic alterations, and allows to improve the chances of survival, guaranteeing a good quality of life during treatment. The drug pemigatinib, an FGFR2 inhibitor, has been shown to offer an important benefit in terms of objective responses, i.e. tumor size reduction, in 37% of patients. It also showed a median survival of almost one and a half years (17.5 months).
“A very important result, because we are dealing with pretreated patients – underlines Rimassa -. To understand the extent of the data, just think that in the first line of treatment with chemotherapy the median survival is about one year”.
“This drug really looks like it can make a difference,” he says Onofrio Mastandrea, Associate Vice President and General Manager of Incyte Biosciences Italia, the company that developed the drug. “Pemigatinib represents the first approved target therapy in cholangiocarcinoma and the real big news after more than a decade in the treatment of this pathology. The next developments of the molecule are certainly at the forefront, through a phase three study in comparison with chemotherapy. alongside clinicians and scientific societies to improve the treatment of cholangiocarcinoma, also by promoting the work of multidisciplinary teams “.
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