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Treatments for Neuroendocrine Tumors
The goal of treating a neuroendocrine tumor is to cure the disease when it is localized—particularly through surgery—or to control its progression over the long term when it is more widespread. Treatment also aims to manage symptoms related to any hormonal secretions. The treatment strategy depends on the tumor’s location, differentiation, grade, rate of progression, extent, and somatostatin receptor expression. It is determined during a specialized multidisciplinary team meeting.
Well-differentiated TNE or neuroendocrine carcinoma: different treatments
Well-differentiated neuroendocrine tumors may grow slowly and be managed using a stepwise approach. Poorly differentiated neuroendocrine carcinomas are generally more aggressive, and their treatment relies primarily on appropriate chemotherapy.
Understanding the Treatment Strategy
There is no single treatment for TNE. The team selects and prescribes treatments based on the disease profile and the patient’s condition. Several approaches may be combined or used sequentially.
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Controlling the Tumor
Remove the disease when it is operable or slow its progression.
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Managing Hormones
Reduce symptoms caused by hormone secretion.
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Preserving Quality of Life
Adjust the treatment schedule, treatment tolerance, and supportive care.
Active Surveillance
Watchful waiting without immediate treatment may be recommended for certain well-differentiated NEIs that are slow-growing and do not cause concerning symptoms. It can also provide for a treatment-free period between two courses of therapy. It involves regular follow-up visits and examinations, with treatment initiated if the disease progresses or becomes symptomatic.
Monitoring is a therapeutic decision
Active monitoring does not mean that there are no treatment options. It prevents exposing the patient too early to the adverse effects of a treatment when the immediate benefit would be limited.
Localized Tumors
When a TNE is localized and resectable, surgery is the primary treatment aimed at a cure. The goal is to remove the primary tumor and, depending on its location and extent, the associated lymph nodes. For certain gastrointestinal locations, endoscopic resection may be considered. Thoracic NETs or complex procedures may require a coordinated surgical pathway with an expert partner institution.
In some patients with disease that is limited to or primarily localized in the liver, surgery to remove the primary tumor, certain metastases, or a combination of these procedures may be considered. The decision is based on the location, tumor size, rate of progression, and other treatment options.
A specialized surgical decision
Surgery for a TNE must take into account its origin, its biology, and the disease as a whole. The indication for surgery and the timing of the procedure are discussed prior to the operation during a specialized multidisciplinary team meeting.
When administered as regular injections, they can reduce symptoms associated with certain hormonal secretions and slow the growth of several well-differentiated NETs that express somatostatin receptors. They may be prescribed alone or in combination with other treatments, depending on the course of the disease.
Targeted therapies administered orally may be recommended for certain progressive NE tumors. Their use depends, in particular, on the tumor’s location, previous treatments, and the patient’s profile. These therapies require clinical and laboratory monitoring for adverse effects.
Chemotherapy can be administered orally or intravenously. It is used to treat certain progressive, well-differentiated NETs, particularly depending on their origin and grade. For poorly differentiated neuroendocrine carcinomas, it is generally the standard of care, with treatment regimens that differ from those used for well-differentiated NETs.
Nuclear Medicine and Theranostics
Theranostics combines imaging to confirm the presence of a target on tumor cells with a treatment that targets that same target. For neuroendocrine tumors (NETs) that express somatostatin receptors, functional imaging helps identify patients who are likely to benefit from targeted internal radiation therapy.
Vector-mediated internal radiation therapy, also known as RIV, involves the intravenous administration of a radioactive molecule bound to a vector that binds to somatostatin receptors. This allows the radiation to be delivered as close as possible to the tumor cells. It may be recommended for certain well-differentiated, advanced TNE tumors that sufficiently express the target, following approval by the multidisciplinary tumor board.
Before and during the RIV, the team assesses, among other things, renal function, blood counts, and the patient’s general condition. The Nuclear Medicine and Endocrine Oncology Department explains the practical procedures, the number of administrations, and radiation protection precautions.
Locoregional Treatments
When the disease primarily affects the liver or involves a limited number of lesions, local techniques can complement medical treatments. Their goal may be to reduce tumor volume, control a progressive lesion, or alleviate hormonal symptoms.
Depending on the situation, targeted ablation using heat or cold, embolization, or chemoembolization may be recommended. The choice depends on the number, size, and location of the lesions, as well as liver function and any previous treatments received.
External beam radiation therapy can be used to treat a localized lesion, a painful or life-threatening metastasis, or a condition that cannot be treated with surgery. The technique and dose are tailored to the location of the lesion and the surrounding organs.
In cases of advanced or metastatic disease
Metastatic TNE can often be controlled through several successive lines of treatment. The sequence of treatment options is not the same for all patients. It takes into account symptoms, tumor volume, observed progression, tumor location, tumor grade, findings on functional imaging, and prior treatments.
A strategy that is regularly reviewed
The case may be reviewed again at an ENDOCAN-RENATEN multidisciplinary team meeting during follow-up, particularly in the event of disease progression, new symptoms, or before a significant change in treatment.
Gustave Roussy's Expertise
Care involves a multidisciplinary approach combining endocrine oncology, surgery, nuclear medicine, diagnostic and interventional radiology, pathology, clinical laboratory science, genetics, and supportive care. Gustave Roussy is one of the coordinating centers of the ENDOCAN-RENATEN national network dedicated to rare neuroendocrine neoplasms.
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Expert Diagnosis
Confirmation of differentiation, grade, and characteristics relevant to treatment.
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Specialized RCP
Collective decision-making regarding the treatment plan and follow-up discussions during the course of care.
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Coordinated Care Pathway
Treatments provided at Gustave Roussy, in the city, or through partners, as needed.
Follow-up and supportive care
Follow-up care combines clinical examination, morphological and functional imaging as appropriate, and laboratory tests when warranted. The frequency of follow-up is tailored to the type of tumor, its progression, and the treatments administered. Supportive care may include nutritional, psychological, and social support; management of pain, fatigue, and hormonal symptoms; and an oncogenetic consultation when indicated.
Patient Information Booklet on Neuroendocrine Tumors
Frequently Asked Questions
Because some TNE progress very slowly. When the disease is stable and does not cause any concerning symptoms, close monitoring may offer a better balance between benefits and risks than immediate treatment.
No. Their use depends, in particular, on the tumor’s well-differentiated nature, hormonal symptoms, and somatostatin receptor expression. The indication is evaluated by the specialized team.
No. It is a nuclear medicine treatment that uses a carrier that targets somatostatin receptors to deliver a radionuclide to tumor cells.
Because their biology, rate of progression, and response to treatment differ. This anatomopathological distinction is essential for selecting a treatment strategy.