The Current Management of Pancreatic Neuroendocrine Tumors

Department of Surgery, The Johns Hopkins Hospital, 1550 Orleans Street, CRB II, Room 506, Baltimore, MD 21287, USA.
Advances in Surgery 09/2012; 46(1):283-96. DOI: 10.1016/j.yasu.2012.04.002
Source: PubMed


PanNETs constitute a rare and heterogeneous group of pancreatic neoplasms whose overall prognosis is better than the more common pancreatic adenocarcinoma. Although surgery is the only treatment that provides a cure, many adjuvant therapies have been explored with some new, exciting, targeted therapies just approved for PanNETs. With growing interest in this type of neoplasm, an increasing number of clinical trials and natural history studies should shed light on the best management for these patients.

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    ABSTRACT: Pancreatic neuroendocrine tumors (PanNETs) have increased in incidence in the USA over the last 20 years. Although PanNETs are often misconceived as being indolent tumors as they have a far more favorable prognosis over pancreatic adenocarcinoma, roughly 60-70% of patients have metastatic disease at the time of diagnosis due to presentation late in the disease process. While improvements in imaging modalities allow for early detection and better tumor localization, recent advancements in basic science, as well as surgical and medical management of PanNETs have further improved the prognosis. The mainstay of therapy for localized PanNETs is surgical intervention, which has become safer and is slowly shifting towards a more minimally invasive approach. However, the prognosis still remains relatively bleak for patients with unresectable disease. Fortunately, novel molecular targeted therapies, such as everolimus and sunitinib, have recently come into the limelight and have shown significant promise for the treatment of locally advanced and metastatic disease.
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    ABSTRACT: We present a case of newly diagnosed lung adenocarcinoma and a five-month history of diarrhea. The patient was found to have elevated chromogranin A serum levels and the tumor displayed histologic and immunohistochemical features of neuroendocrine differentiation. Upon lobectomy and tumor resection, the chromogranin A levels normalized and the diarrhea resolved completely. This is the first reported case of paraneoplastic diarrhea associated with lung adenocarcinoma.
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