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Neuroendocrine Tumors
Director: Dr Julien Hadoux
Contact Information
+33 (0)1 42 11 42 42, 42 57, 63 61, or 50 25
Understanding the Disease
Neuroendocrine tumors, or NETs, develop from neuroendocrine cells found in many organs. These cells receive signals and can produce hormones. A NET can therefore develop in various parts of the body, most commonly in the digestive tract, the pancreas, or the lung, but also in other, rarer locations.
Neuroendocrine neoplasms constitute a highly heterogeneous group. Some well-differentiated tumors grow slowly over several years. Others are more aggressive. Poorly differentiated neuroendocrine carcinomas form a distinct category, typically progressing rapidly, which requires a specific treatment strategy.
Gustave Roussy's Expertise
Care involves a combination of endocrine oncology, medical oncology, surgery, imaging, interventional radiology, nuclear medicine, pathology, and supportive care. Cases are discussed during a specialized multidisciplinary team meeting.
How is a neuroendocrine tumor classified?
The diagnosis identifies the organ in which the tumor originated. Tumors of the digestive tract, pancreas, and lungs are among the most common. When the primary tumor cannot be identified, the evaluation aims to pinpoint its location without delaying necessary treatment.
A well-differentiated TNE retains a structure similar to that of normal neuroendocrine cells. A poorly differentiated neuroendocrine carcinoma consists of highly abnormal cells and exhibits different biological characteristics. This distinction is based on histopathological analysis.
The grade provides information about the tumor's growth rate. It helps in estimating how quickly the tumor is progressing and in selecting a treatment. The grade should not be confused with the stage.
The stage describes the extent of the disease, ranging from a localized tumor to disease that has spread to lymph nodes or other organs. The liver is a common site for metastases in several gastrointestinal NETs, but the evaluation is tailored to each individual.
A tumor is considered functional when it produces hormones in sufficient quantities to cause a clinical syndrome. A tumor may express neuroendocrine markers without causing hormonal symptoms.
Risk Factors and Predispositions
In most cases, no single cause is identified, and there is no specific way to prevent a TNE. However, certain situations warrant a special evaluation.
Some NETs may be part of an inherited syndrome, including multiple endocrine neoplasia type 1, von Hippel-Lindau disease, neurofibromatosis type 1, or Bourneville tuberous sclerosis. An oncogenetic consultation is recommended when the patient’s age, tumor location, number of tumors, or personal and family history warrant it.
Risk factors are not the same for all neuroendocrine tumors. Smoking is notably associated with pulmonary neuroendocrine neoplasms, particularly high-grade neuroendocrine carcinomas.
Key Takeaways
A TNE is generally not the result of any identifiable lifestyle factor. Screening for a genetic predisposition applies only to a subset of patients and is based on an individualized medical evaluation.
Symptoms
Symptoms vary depending on the location, tumor size, extent of the disease, and whether the tumor secretes hormones. Some NETs are discovered incidentally during an examination performed for another reason.
- Abdominal pain, bowel dysfunction, obstruction, gastrointestinal bleeding, or weight loss for certain gastrointestinal locations.
- Persistent cough, shortness of breath, recurrent lung infections, or chest pain for certain bronchopulmonary locations.
- Hypoglycemia in cases of insulinoma, recurrent gastrointestinal ulcers in cases of gastrinoma, or other hormone-related symptoms depending on the type of functional tumor.
- Fatigue, weight loss, pain, or symptoms related to metastases in advanced stages.
When should you seek immediate medical attention?
Severe hypoglycemia, difficulty breathing, severe pain, persistent vomiting, suspected obstruction, or a rapid deterioration in general condition require immediate medical evaluation.
The Diagnosis
The diagnosis must specify the exact nature of the tumor, its location, differentiation, grade, extent, rate of progression, and any hormonal activity. A comprehensive interpretation of pathology, laboratory test results, and imaging findings is essential.
The biopsy or surgical specimen confirms the neuroendocrine nature of the tumor. The pathology report specifies, in particular, the degree of differentiation and proliferation parameters. A second opinion from an expert pathologist may be requested, particularly in cases where diagnosis is difficult or before a major treatment decision is made.
Tests are selected based on the location and symptoms. They can detect hormonal secretion and aid in monitoring when a marker is relevant to the patient. Chromogranin A is not a screening test, and its interpretation may be influenced by other medical conditions or certain treatments.
CT and MRI scans are used to evaluate the primary tumor, lymph nodes, and any metastases. A liver MRI may be useful for characterizing liver lesions. The tests are tailored to the location of the tumor and the clinical context.
A PET scan using a tracer that targets somatostatin receptors may be recommended for certain well-differentiated TNE tumors. It helps determine the extent of the disease and whether treatment targeting these receptors may be appropriate.
FDG-PET may be useful for characterizing more proliferative or aggressive tumors. It does not systematically replace somatostatin receptor imaging. The choice depends on the type of tumor and the clinical question.
A specialized consultation may be offered when a hereditary form is suspected. Genetic testing is performed after the patient has been informed and has given consent. The results may have implications for the patient’s follow-up care and, when a hereditary abnormality is identified, for the support provided to the family.
Care at Gustave Roussy
At the initial consultation, the team reviews the medical records, imaging studies, laboratory results, and, when necessary, tumor slides or tissue blocks. Additional tests are ordered only if they are useful for confirming the diagnosis, determining the extent of the disease, or guiding treatment.
The treatment strategy is discussed during a specialized multidisciplinary team meeting. It takes into account the tumor’s location, differentiation, grade, rate of progression, somatostatin receptor expression, hormonal secretion, extent of the disease, prior treatments, and the patient’s overall condition.
Before the Initial Consultation
To facilitate review of the medical record, it is helpful to gather medical reports, the pathology report, laboratory test results, imaging reports, and the images themselves. The practical procedures for transmitting these documents must be specified in the appointment schedule, in accordance with current protocols.
Supportive Care and Support Services
Care may include nutritional, psychological, and social support; pain management; fatigue management; a nursing consultation; management of hormonal symptoms; and, if indicated, an oncogenetics consultation. Needs are reassessed during follow-up visits.
A center of expertise and specialized networks
Gustave Roussy participates in the RENATEN Paris-Sud Multidisciplinary Tumor Board, the European EURACAN network, and the Endocrine Tumor Study Group.
Treatments
The goal of treatment is to remove or control the tumor, prevent or slow its progression, treat metastases, and manage hormonal symptoms. Active surveillance may be preferable to immediate treatment for certain well-differentiated, small, stable NETs that do not cause life-threatening symptoms.
It is based on consultations, imaging, and, when appropriate, regular laboratory tests. It does not mean a lack of care. It helps prevent or delay the adverse effects of treatment when no immediate benefit is expected.
Surgery is the standard of care for many localized NETs when complete resection is possible. It may also be considered in certain metastatic diseases, particularly to treat the primary tumor, reduce tumor volume, or manage selected metastases. The decision depends on the location, extent, and surgical risk.
They are used to control the symptoms of certain functional tumors. They can also slow the progression of certain well-differentiated neuroendocrine tumors (NETs) that express somatostatin receptors. They are generally administered through repeated injections, depending on the medication and the treatment regimen chosen.
Drugs that target specific tumor growth mechanisms may be recommended for specific indications. The choice depends, in particular, on the location of the TNE, its progression, prior treatments, and current approvals.
Chemotherapy may be recommended for certain well-differentiated NETs, particularly those of the pancreas or those that progress rapidly. It plays a central role in the treatment of poorly differentiated neuroendocrine carcinomas. The specific drugs and treatment combinations vary depending on the diagnosis and clinical situation.
This treatment combines a molecule that targets somatostatin receptors with a radioactive isotope. It may be offered to certain patients whose PNETs express these receptors to a sufficient degree, after a specialized nuclear medicine team has verified the indication and safety requirements.
It can be used to control a localized lesion, relieve symptoms, treat certain metastases—particularly those in the bones or brain—or as part of the treatment for certain thoracic neuroendocrine tumors (NETs). The indication depends on the type of tumor and the clinical context.
For certain metastases, particularly those in the liver, thermal ablation, embolization, chemoembolization, radioembolization, or surgery may be considered. The choice depends on the number, size, and distribution of the lesions, liver function, and other sites of the disease.
A phased strategy
Several treatments may be used sequentially or in combination. The order depends on the primary goal, the rate of disease progression, and patient tolerance. A period without anticancer treatment may be scheduled between two treatment cycles, with appropriate monitoring.
Research and Clinical Trials
Clinical research evaluates new drugs, new combinations, optimized modalities of targeted internal radiation therapy, and strategies tailored to different subtypes of neuroendocrine neoplasms. It also seeks to identify better markers of response and resistance.
A clinical trial may be offered when the patient’s condition meets the protocol’s criteria. The team explains the study’s objective, the treatments being compared, any restrictions, known risks, and available alternatives. Participation is voluntary and requires informed consent.
Join a clinical trial
Many clinical trials are open to patients with cancer. Participating in a clinical trial provides access to innovative treatments while contributing to the development of new therapies.
Follow-up
Follow-up is tailored to the type of tumor, its grade, its extent, the treatments received, and its rate of progression. It combines clinical evaluation, imaging, and—when informative—hormone levels or other markers. The case may be reviewed again at a multidisciplinary team (MDT) meeting at each major stage.
Patient Information Booklet: Neuroendocrine Tumors
This informational document from Gustave Roussy explains the main types of neuroendocrine tumors (NETs), how they are detected, diagnosis, tests, treatments, monitoring, and living with the disease.
Contacts
Head: Dr Julien Hadoux
Phone: +33 (0)1 42 11 42 42, 42 57, 63 61, or 50 25
Appointments and Second Opinions
