Medical Treatment of Advanced Pancreatic Neuroendocrine Neoplasms

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.

A Case of Pancreatic Neuroendocrine Tumor G3 Which Responded Remarkably to Chemotherapy

“…It has been reported that platinum-based chemotherapy in NET G3 demonstrates a lower response rate than in NEC [12], but there are no previous reports describing the use of molecular targeted therapy and STZ for NET G3. In general, STZ and molecular targeted therapy are used for unresectable NENs [13], but an optimal treatment for NET G3 has not been established. Therefore, while attempting to treat NET G3 according to the conventional treatment for NET G1/G2, collecting the treatment results of NET G3 cases is important in attempting to establish the optimal treatment.…”

Interventional Radiological Management and Prevention of Complications after Pancreatic Surgery: Drainage, Embolization and Islet Auto-Transplantation

“…Surgical resection can be performed in about 15–20% of the patients at the time of diagnosis [ 2 ]. In the remaining patients with locally advanced or metastatic disease, palliative treatments are required [ 3 , 4 , 5 , 6 , 7 , 8 , 9 ]. Moreover, pancreatic surgery is one of the most complex surgical abdominal procedures, which is due to anatomical location of the pancreatic gland, close to vascular structures and the biliary and gastrointestinal system.…”

Well-Differentiated Grade 3 Neuroendocrine Tumors

“…The NORDIC study reported a low response rate to platinum-based chemotherapy for high-grade NENs with a Ki-67 < 55%, and this was subsequently confirmed in multiple small cohort studies 1,5–7,12,13 . On the other hand, alkylator-based chemotherapy is an established treatment option even for low-grade pancreatic NETs, which are regarded as chemosensitive tumors, unlike midgut NETs 16,17 . The role of surgical management and other local therapies for G3 NETs is also controversial, as it is often indicated in G1–2 NETs but contraindicated for NECs 4,6,10,18 …”

“…1,[5][6][7]12,13 On the other hand, alkylator-based chemotherapy is an established treatment option even for low-grade pancreatic NETs, which are regarded as chemosensitive tumors, unlike midgut NETs. 16,17 The role of surgical management and other local therapies for G3 NETs is also controversial, as it is often indicated in G1-2 NETs but contraindicated for NECs. 4,6,10,18 Clinical decision making for this rare disease is complex and based on limited data.…”