Ποιο είναι το προσδόκιμο επιβίωσης στους νευροενδοκρινείς όγκους;

Δεν υπάρχει ένα ενιαίο προσδόκιμο επιβίωσης. Εξαρτάται από την εντόπιση, τον βαθμό διαφοροποίησης, το Ki-67, το στάδιο της νόσου και την ανταπόκριση στη θεραπεία. Η εξατομικευμένη εκτίμηση βασίζεται στον πλήρη ιατρικό φάκελο.

Ποια είναι τα πρώτα συμπτώματα του καρκινοειδούς συνδρόμου;

Πιθανά συμπτώματα είναι οι εξάψεις στο πρόσωπο, η διάρροια, ο συριγμός και οι ταχυπαλμίες. Πολλοί νευροενδοκρινείς όγκοι όμως δεν προκαλούν καρκινοειδές σύνδρομο, επομένως τα συμπτώματα χρειάζονται ιατρική αξιολόγηση.

Πότε πρέπει να ζητήσω δεύτερη ιατρική γνώμη;

Μια δεύτερη γνώμη είναι χρήσιμη μετά από νέα διάγνωση, όταν δεν είναι σαφές αν πρόκειται για NET ή NEC, πριν από σημαντική αλλαγή θεραπείας ή όταν εξετάζονται επιλογές όπως PRRT, χειρουργική επέμβαση ή συμμετοχή σε κλινική μελέτη.

Neuroendocrine Tumours and Carcinomas

NETs • NECs • Lung • Gastrointestinal Tract & Pancreas

Every neuroendocrine neoplasm is different, and each patient needs to understand what their own diagnosis means, which treatment options are genuinely relevant and what the next step should be. Management is planned individually, based on the biology and extent of the disease and the needs of the individual patient.

Why a specialist approach matters

Neuroendocrine neoplasms are not a single disease. A well-differentiated neuroendocrine tumour (NET) may have entirely different biological behaviour and treatment from a neuroendocrine carcinoma (NEC).

The appropriate treatment strategy depends on the tumour type, degree of differentiation and Ki-67, the primary site and the extent of disease. Accurate classification is therefore often the first essential step before any treatment decision is made.

Dr Georgios Evangelou focuses on the management of pulmonary and gastrointestinal neuroendocrine neoplasms. His clinical approach is informed directly by his active research work. He is:

• Coordinator and Scientific Lead of the Neuroendocrine Neoplasms Clinic (Sotiria General Hospital for Thoracic Diseases, Athens)

• Principal Investigator of the prospective LANCE study (ESMO)

Main Types of Neuroendocrine Neoplasms

Pulmonary Neuroendocrine Neoplasms

Pulmonary neuroendocrine neoplasms include typical and atypical carcinoids, large-cell neuroendocrine carcinoma (LCNEC) and small-cell lung cancer (SCLC). Possible symptoms include persistent cough, breathlessness, haemoptysis or unexplained fatigue, although some lesions are found incidentally. Treatment is determined by the precise tumour type, stage and molecular characteristics.

Gastrointestinal & Pancreatic NETs (GEP-NETs)

GEP-NETs may arise in the stomach, intestine, rectum or pancreas, and they do not all have the same biological behaviour. Some functioning tumours cause hormone-related symptoms. Carcinoid syndrome may present with flushing, diarrhoea, wheezing or palpitations, although many patients do not develop this syndrome. Treatment selection takes into account differentiation, Ki-67, somatostatin receptor expression, the extent of disease and symptoms.

Treatment Options for Neuroendocrine Tumours and Carcinomas

There is no single treatment that is appropriate for every neuroendocrine neoplasm. Selection depends on the primary site, differentiation and grade, Ki-67, the extent and pace of disease progression and, where relevant, somatostatin receptor expression and previous treatments.

Depending on the characteristics of the disease, the treatment strategy may include:

Somatostatin analogues and PRRT, principally for appropriately selected, well-differentiated, SSTR-positive NETs.
Targeted systemic therapies, for selected NETs according to the primary site and previous treatment course.
Chemotherapy and/or immunotherapy, when indicated by the specific tumour type and biology.
Surgery and local therapies, when they can contribute to disease control or management.
Management of hormone-related syndromes and symptoms in functioning tumours.

The appropriate treatment is determined not only by the diagnostic label but by the overall biology and extent of disease, as well as the characteristics and needs of the individual patient.

Frequently Asked Questions

The main difference lies in the biological behaviour of the disease. Well-differentiated neuroendocrine tumours (NETs) may follow a relatively slow course, although their behaviour varies according to grade and site of origin. In contrast, neuroendocrine carcinomas (NECs), such as LCNEC and small cell lung cancer, are poorly differentiated neoplasms that usually grow more rapidly, follow a more aggressive course and have a greater tendency to spread early. The treatment options are therefore substantially different. Depending on the individual case, NETs may be treated with somatostatin analogues, PRRT, targeted therapies or chemotherapy, whereas treatment of NECs more often relies on systemic chemotherapy and is tailored to the site of origin and the specific characteristics of the disease.

Ki-67 is a marker of the rate at which tumour cells are proliferating, but it does not by itself determine treatment choice. It is interpreted together with tumour morphology and differentiation, mitotic count, site and extent of disease, and the other clinical and biological features. In pulmonary carcinoid tumours, Ki-67 has a more limited role in classification: the distinction between typical and atypical carcinoid is based mainly on mitotic count and the presence of necrosis.

Symptoms depend both on where the disease is located and on its biological function. In some cases, well-differentiated neuroendocrine tumours (NETs) produce hormones or other bioactive substances and cause characteristic functional syndromes. These include carcinoid syndrome, Zollinger–Ellison syndrome, insulinoma, VIPoma, glucagonoma and, less commonly, Cushing syndrome due to ectopic ACTH production. Neuroendocrine carcinomas (NECs) behave differently. In small cell lung cancer in particular, paraneoplastic syndromes may occur, including SIADH, Cushing syndrome due to ectopic ACTH production, and neurological syndromes such as Lambert–Eaton syndrome. Both NETs and NECs can also cause symptoms related to the location of the primary tumour or its metastases.

A second opinion can be particularly useful when the diagnosis is rare or complex, when there is uncertainty about the exact classification of the disease, or before an important treatment decision. In neuroendocrine neoplasms, where different subtypes can have very different biological behaviour and require different treatment strategies, reassessment of the pathological diagnosis, imaging findings and available treatment options can be especially helpful.

Arrange an Appointment at the Oncology Clinic

If you have a new diagnosis, require a review of the treatment plan or would like a structured second opinion, you can send your medical records and arrange an assessment at the oncology clinic in Athens.

Contact & Arrange an Appointment

Neuroendocrine Tumours and Carcinomas

NETs • NECs • Lung • Gastrointestinal Tract & Pancreas

Why a specialist approach matters

Main Types of Neuroendocrine Neoplasms

Pulmonary Neuroendocrine Neoplasms

Gastrointestinal & Pancreatic NETs (GEP-NETs)

Treatment Options for Neuroendocrine Tumours and Carcinomas

Frequently Asked Questions

What is the difference between a NET and a neuroendocrine carcinoma (NEC)?

What does Ki-67 mean, and why is it important?

What symptoms can a neuroendocrine neoplasm cause?

What is the expected survival?

How is the most appropriate treatment selected?

When can a second medical opinion be valuable?

Arrange an Appointment at the Oncology Clinic