Pancreatic neuroendocrine tumors: Nosography, management and treatment

Orditura, Michele; Petrillo, Angelica; Ventriglia, Jole; Diana, Anna; Laterza, Maria Maddalena; Fabozzi, Alessio; Savastano, Beatrice; Franzese, E.; Conzo, Giovanni; Santini, Luigi; Ciardiello, Fortunato; Vita, Ferdinando De

doi:10.1016/j.ijsu.2015.12.052

Cited by 46 publications

(42 citation statements)

References 78 publications

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“…Surgical resection with regional lymph node dissection is the only curative treatment option, and is recommended to all patients with early-stage well-differentiated pNENs [41][42][43]. Although cancer patients with metastatic diseases are generally unsuitable for surgical resection in the field of oncology, partial hepatectomy is often performed in pNENs patients with liver metastases, depending upon the number, size and location of the lesions, the extent of the primary tumor and the patient's performance status [43].…”

Section: Well-differentiated Pnens (Net G1/g2)mentioning

confidence: 99%

Pancreatic Neuroendocrine Neoplasms: Basic Biology, Current Treatment Strategies and Prospects for the Future

Ohmoto

Rokutan

Yachida

2016

Preprint

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Pancreatic neuroendocrine neoplasms (pNENs) are rare tumors accounting for only 1%-2% of all pancreatic tumors. pNENs are pathologically heterogeneous and are categorized into three groups (neuroendocrine tumor: NET G1, NET G2; and neuroendocrine carcinoma: NEC) on the basis of the Ki-67 proliferation index and the mitotic count according to the 2010 World Health Organization (WHO) classification of gastroenteropancreatic NENs. NEC in this classification includes both histologically well-differentiated and poorly differentiated subtypes, and modification of the WHO 2010 classification is under discussion based on genetic and clinical data. Genomic analysis has revealed NETs G1/G2 have genetic alterations in chromatin remodeling genes such as MEN1, DAXX and ATRX, whereas NECs have an inactivation of TP53 and RB1, and these data suggest that different treatment approaches would be required for NET G1/G2 and NEC. While there are promising molecular targeted drugs, such as everolimus or sunitinib, for advanced NET G1/G2, treatment stratification based on appropriate predictive and prognostic biomarkers is becoming an important issue. The clinical outcome of NEC is still dismal, and a more detailed understanding of the genetic background together with preclinical studies to develop new agents, including those already under investigation for small cell lung cancer (SCLC), will be needed to improve the prognosis.

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Section: Well-differentiated Pnens (Net G1/g2)mentioning

confidence: 99%

Pancreatic Neuroendocrine Neoplasms: Basic Biology, Current Treatment Strategies and Prospects for the Future

Ohmoto

Rokutan

Yachida

2016

Preprint

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“…7 Arising from the endocrine pancreas, they can be sporadic or part of a genetically inherited syndrome, such as multiple endocrine neoplasia-1 (MEN-1); the majority of PNETs are non-functional, although some do secrete hormones. 8 Different types of pancreatic malignancies have distinct etiologies and prognosis. 2,9 With a mean age at diagnosis of 71 for PDAC and 58.7 for PNET, few cases of pancreatic cancer are diagnosed in younger adults.…”

Section: Introductionmentioning

confidence: 99%

Early onset pancreatic malignancies: Clinical characteristics and survival associations

Beeghly–Fadiel

Luu

et al. 2016

Intl Journal of Cancer

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Diagnosed before age 50, early onset pancreatic malignancy (EOPM), is hypothesized to be a distinct subset of disease, although research is limited. To better characterize EOPM, and the effect of age at diagnosis on pancreatic cancer survival, we examined clinical characteristics and survival in EOPM and typical age-at-onset pancreatic malignancy (TOPM) cases. Vanderbilt University Medical Center (VUMC) Cancer Registry confirmed pancreatic adenocarcinomas (PDACs) and malignant pancreatic neuroendocrine tumors (PNETs) were evaluated. Clinical characteristics were compared using χ(2) tests. Overall survival was visualized with Kaplan-Meier functions; Cox proportional hazards regression was used to evaluate hazard ratios (HRs) and 95% confidence intervals (CIs). A total of 1,697 pancreatic malignancies were diagnosed at the VUMC between 1988 and 2013. Of 1,407 PDACs, 118 (8.4%) were EOPM, which was associated with significantly better survival (adjusted HR: 0.82, 95% CI: 0.67-1.00). EOPM and TOPM PDACs significantly differed with regard to having multiple malignancies; survival associations significantly differed by race, stage of disease, treatment and multiple malignancies. Of 190 PNETs, 63 (33.1%) were EOPM, which was not significantly associated with survival (adjusted HR: 0.80, 95% CI: 0.46-1.40). Malignant neuroendocrine EOPM and TOPM cases significantly differed by stage of disease and tumor location; survival associations significantly differed by family history of pancreatic cancer, stage of disease and multiple malignancies. Differences in clinical characteristics and associations with survival were identified, indicating that EOPM is distinct from TOPM, and exists among both pancreatic adenocarcinomas and malignant pancreatic neuroendocrine tumors.

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“…Pancreatic neuroendocrine tumors (pNETs) represent approximately 7% of all NETs. Their prevalence has considerably increased over the last three decades, which is primarily due to improvements in imaging and diagnostic techniques (1,2). Despite the increased diagnosis of incidental lesions, 40-95% of patients present with a metastatic disease at the first diagnosis (2,3).…”

Section: Introductionmentioning

confidence: 99%

Liver transplantation for unresectable pancreatic neuroendocrine tumors with liver metastases in an era of transplant oncology

2018

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Patients with pancreatic neuroendocrine tumors (pNETs) very often present with a metastatic disease at the first diagnosis. Liver transplantation (LT) for unresectable pNET with liver metastases (pNETLM) has been described to prolong survival in highly selected patients, although outcomes were worse than those of patients who underwent LT for gastrointestinal NETLM (GI-NETLM). In this review, several proposed criteria are described with their rationale and controversies. Most of the data used to establish these criteria do not reflect the recent improvements of non-surgical treatments that has changed the landscape of treatment for pNETs, including the development of peptide receptor radionuclide therapy and molecular-targeted agents (sunitinib and everolimus). Properly designed studies are necessary to define the role of down-staging and bridging therapy prior to LT incorporating systemic chemotherapy using these molecular-targeted agents. Also, given the indolent nature of low or intermediate grade pNETs, the best endpoint to compare the efficacy of each treatment option for patients with pNETLM has yet to be determined. Lastly, the definition of "unresectable" remains ambiguous. The indication of the conventional technique of two-staged liver resection with portal vein embolization or the new technique of associating liver partition and portal vein ligation for staged hepatectomy to expand the resectability of wide-spread metastatic liver tumors has been controversial. In an era of transplant oncology, LT should be the last resort for patients who are considered unresectable and otherwise untreatable after an exhaustive multidisciplinary team discussion by all experts in the field. In conclusion, although its long-term outcomes have been promising, the role of LT for unresectable pNETLM as a curative or palliative treatment remains unclear. A well-designed randomized control study is required to elucidate the clinical impact of LT for pNETLM.

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Pancreatic neuroendocrine tumors: Nosography, management and treatment

Cited by 46 publications

References 78 publications

Pancreatic Neuroendocrine Neoplasms: Basic Biology, Current Treatment Strategies and Prospects for the Future

Pancreatic Neuroendocrine Neoplasms: Basic Biology, Current Treatment Strategies and Prospects for the Future

Early onset pancreatic malignancies: Clinical characteristics and survival associations

Liver transplantation for unresectable pancreatic neuroendocrine tumors with liver metastases in an era of transplant oncology

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