Abstract
Pancreatic neuroendocrine neoplasms (panNENs) are relatively rare but their incidence has increased almost sevenfold over the last four decades. Neuroendocrine neoplasms are classified according to their histologic differentiation and their grade. Their grade is based on their Ki-67 proliferation index and mitotic index. Their prognosis is highly variable according to these elements and treatments also vary according to their classification. Surgery is the only curative treatment for localized and advanced panNENs and offers a better prognosis than non-surgical treatments. In the case of an advanced panNEN without the possibility of resection and/or ablation, medical treatment remains the cornerstone for improving survival and preserving quality-of-life. PanNENs are considered as chemosensitive tumors, unlike midgut neuroendocrine tumors. Thus, panNENs can be treated with chemotherapy, but targeted therapies and somatostatin analogs are also treatment options. The scarcity and heterogeneity of NENs make their management difficult. The present review aims to clarify the medical treatments currently available for advanced panNENs, based on their characteristics, and to propose a treatment algorithm.
Highlights
Pancreatic neuroendocrine neoplasms (PanNENs) are considered as relatively rare neoplasms
This review focuses on the medical treatment of metastatic Pancreatic neuroendocrine neoplasms (panNENs), excluding surgical, locoregional, and ablative therapies
Grade 3 poorly differentiated panNECs are rare in the gastrointestinal tract and account for less than 5% of GEP NENs, whereas they are common in the form of small cell carcinoma (SCLC) in the lung
Summary
Pancreatic neuroendocrine neoplasms (PanNENs) are considered as relatively rare neoplasms. Octreotide significantly improved the median time to tumor progression (mTTP) compared to placebo: 14.3 versus 6 months, respectively (hazard ratio (HR) 0.34, p < 0.001) [6]. This trial demonstrated the efficacy of SSAs for midgut NETs but excluded panNETs. Later, the phase III CLARINET trial randomized 204 patients with advanced well-differentiated and non-functional G1 or G2 panNETs to receive lanreotide autogel. In case of SSTR avidity, SSAs (octreotide LAR 30 mg and lanreotide autogel 120 mg every 28 days) can be used as first-line treatment of advanced panNETs with stable or slowly progressing disease, or in patients with unknown tumor behavior with Ki-67 less than 5–10%. The most common side effects are injection site pain, abdominal pain with diarrhea, nausea and vomiting
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