Surgery in malignant pancreatic neuroendocrine tumors.
ABSTRACT Because of their rarity and indolent nature, optimal management of malignant pancreatic neuroendocrine tumors remains controversial. The purpose of this study is to review a series of patients with these tumors and investigate the role of surgery in the treatment.
A retrospective study of 73 patients (ages 24-86 years; 36 women) undergoing treatment at a tertiary academic medical center was performed. Patient demographics, diagnostic tests, operations, pathologic findings, adjuvant treatments, and survival were reviewed.
Seventy-four percent of patients had advanced disease with hepatic metastases and 30% had functional tumors. Fifty-seven percent of the patients underwent pancreatic resections. Two 60-day mortalities occurred and the postoperative complication rate was 27%. Overall 5-year survival rate was 44%. There was no difference in survival between patients with functional and nonfunctional tumors. Patients undergoing resection, even in metastatic disease, had better survival than patients who had no resection (60% vs. 30%, P = 0.025). Recurrence occurred in 20% of patients who underwent a curative resection.
Patients with malignant pancreatic neuroendocrine tumors commonly present with advanced disease. Although, curative resection is not frequent, survival benefit may be obtainable with aggressive surgical management even in the face of metastatic disease.
- SourceAvailable from: Haggi Mazeh[Show abstract] [Hide abstract]
ABSTRACT: Pancreatic neuroendocrine tumors (PNETs) are a rare heterogeneous group of endocrine neoplasms. Surgery remains the best curative option for this type of tumor. Over the past two decades, with the development of laparoscopic pancreatic surgery, an increasingly larger number of PNET resections are being performed by these minimally-invasive techniques. In this review article, the various laparoscopic surgical options for the excision of PNETs are discussed. In addition, a summary of the literature describing the outcome of these treatment modalities is presented.World Journal of Gastroenterology 05/2014; 20(17):4908-4916. · 2.55 Impact Factor
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ABSTRACT: Pancreatic neuroendocrine tumors (PNETs) share a unique genetic identity, functional behavior, and clinical course. Compared with tumors of the exocrine pancreas, they are rare and show a different biologic behavior and prognosis. On the basis of data from recent studies, all PNETs, outside of small insulinomas, should be considered potentially malignant and treated accordingly. Untreated tumors have a high possibility to grow locally into adjacent structures or spread to distant organs. Although surgical excision irrespective of tumor functioning or nonfunctioning state remains the cornerstone of therapy, providing the best disease-free and survival rates to date, the understanding of the genetic nature of the disease yields new 'targets' to consider in drug development. The aim of this review is to summarize all recent advances of genetic research and new drug development in terms of PNETs, especially their genetic identity and subsequent alterations leading to the development of near or total malignant activity, and the new medical treatment strategies of this potentially curable disease on the basis of therapeutical agents acting, where possible, at the genetic level.European journal of gastroenterology & hepatology. 08/2014; 26(8):826-35.
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ABSTRACT: Background Pancreatic neuroendocrine tumors (PNETs) present in more than 50% of cases with liver metastases as the only systemic localization. Liver metastases are unresectable in 80% of cases at diagnosis. In the context of a metastatic disease the benefit of primary tumor removal in terms of survival is controversial. Methods A single-centre series of PNETs presenting with synchronous hepatic unresectable metastases and treated within a framework of a multidisciplinary team was retrospectively analysed to assess the prognostic factors and the potential benefit of primary tumor resection on long-term survival. Results At the time of diagnosis, 12 patients out of 43 (27.9%) underwent primary tumor resection. After a median follow-up of 5 years (range 0.6-14), 22 disease related deaths were observed. The corresponding 5-year and median disease-specific survival were 58.4% and 77 months, respectively. In the operated and not operated patients the 5-year disease-specific survival was 81.8% and 49.7%, respectively (P=0.027). At multivariate analysis, patients with primary tumor removed had a statistically significant improved survival compared to patients who did not (HR: 0.18; 95% CI 0.05-0.66; P=0.010). Other significant factors associated with improved survival at multivariate analysis were lower age, lower Ki-67 index, and liver tumor burden <25%. Conclusions In the present series of patients with PNETs and unresectable liver metastases, resection of the primary tumor was associated with an improved survival. This suggests that primary tumor surgery should be part of a global therapeutic strategy and its indication and timing should be discussed within a multidisciplinary team.Surgery 01/2013; · 3.37 Impact Factor