Surgery in malignant pancreatic neuroendocrine tumors

Department of Surgery, Division of General Surgery, The Mount Sinai School of Medicine, New York, New York 10029-6574, USA.
Journal of Surgical Oncology (Impact Factor: 3.24). 10/2007; 96(5):397-403. DOI: 10.1002/jso.20824
Source: PubMed


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.

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    • "The aggressive approach has 4 components: surgery, locoregional therapy, systemic therapy, and complication control[71]. Surgical removal of primary, non-metastatic PNET is the only clinical cure, and surgical debulking of liver PNET metastases reduces the hormone secretion from functioning PNETs and the tumor mass effects of all PNETs[76],[77]. Surgical removal of PNETs requires considerable experience and should be performed in academic centers with an expert team for PNETs. Because surgery has intrinsic operative and anesthetic risks, patient selection is key to achieving the best outcomes. "
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    ABSTRACT: Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. The genetic causes of familial and sporadic PNETs are understood to some degree while the molecular pathogenesis of PNETs is still unclear. Most PNETs are indolent but have malignant potential. The biological behavior of an individual PNET is not readily predictable; higher tumor grade, lymph node and liver metastasis, and larger tumor size generally portend less favorable prognosis. Endocrine testing, imaging, and histological evidence are needed for accurate diagnosis of PNETs. An "aggressive" treatment approach with 4 components: surgery, locoregional therapy, systemic therapy, and complication control, has becomes a trend in academic centers throughout the world. The optimal use of the multiple modalities of systemic therapy is still being developed; efficacy, safety, availability, and cost should be considered when treating a specific patient. Clinical presentation, diagnosis, and treatment of specific types of PNETs and familial PNET syndromes, including a novel Mahvash disease, are summarized.
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    ABSTRACT: Pancreatic neuroendocrine tumors are rare neoplasms often associated with a clinical syndrome. Their rarity makes a comprehensive study difficult at any single institution, while their uniqueness makes them desirable for investigation. This review summarizes recent information and advancements concerning pancreatic neuroendocrine tumor diagnosis, imaging characteristics, treatment algorithms, and staging. Insulinomas and gastrinomas comprise the majority of functional pancreatic neuroendocrine tumors. Advances in their identification and diagnostic evaluation, imaging techniques, and treatment algorithms are presented. Furthermore, a new staging classification system has been proposed which may significantly improve the ability to conduct future multi-institutional investigations on pancreatic neuroendocrine tumors. Although rare, a thorough understanding of pancreatic neuroendocrine tumors is essential for all physicians due to the wide variety of symptoms with which patients present. Currently, patients are often misdiagnosed for extended periods of time. This review summarizes the recently published literature about diagnosis, imaging, treatment, and staging of pancreatic neuroendocrine tumors.
    Current Opinion in Oncology 02/2008; 20(1):25-33. DOI:10.1097/CCO.0b013e3282f290af · 4.47 Impact Factor
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    ABSTRACT: Pancreatic neuroendocrine tumors (PNETs) comprise a heterogeneous group of neoplasms for which treatment is variable, depending on the clinical stage. Despite this diversity, surgery remains the gold standard in the management of PNETs. This paper discusses whether aggressive surgical intervention is indicated for PNETs and investigates what prognostic factors may assist in predicting which patients with invasive disease will benefit most from surgical intervention. A review was conducted of large surgical series reported in the English literature over the last 10 years as they pertain to current surgical intervention in PNETs and of prognostic factors related to surgical outcome and survival. Improved survival can be achieved with aggressive surgical management of PNETs. The presence of hepatic metastases is not a contraindication to surgical resection of the primary PNET. Results of series that reported prognostic factors are heterogeneous. Aggressive surgical resection for selected individuals with PNETs can be performed safely and may improve both symptomatic disease and overall survival. Consideration for resection of primary PNETs should be given to patients with treatable hepatic metastases. Prognostic indices such as tumor differentiation and ability to achieve R0/R1 resection have been linked to survival outcome in PNETs and should be considered when planning aggressive surgical management for this disease.
    Cancer control: journal of the Moffitt Cancer Center 11/2008; 15(4):314-21. · 3.50 Impact Factor
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