Neuroendocrine Tumors will be featured in the February 2016 issue of Hematology/Oncology Clinics. Dr. Jennifer Chan and Dr. Matthew Kulke, both from Dana-Farber Cancer Institute in Boston, Massachusetts are Editors of the issue, published by Elsevier.
Jennifer Chan, MD and Matthew Kulke, MD

Dr. Jennifer Chan and Dr. Matthew Kulke

Topics covered in the February 2016 issue will be:

Hematology Oncology Clinics Feb 2016
Neuroendocrine Tumors – Current and Future Clinical Advances
Jennifer Chan, MD and Matthew Kulke, MD

Abstract: “Remarkable progress has been made over the last several years in our understanding of the biology and treatment of neuroendocrine tumors. The field has been transformed from one where patients have limited treatment options to one characterized by an increasing number of clinical trials and approved therapeutic agents. Recent studies have also revealed that we can no longer view neuroendocrine tumors as a single disease entity. Biological differences based on primary site, histologic grade, and ability to secrete hormones and other peptides influence clinical presentation, prognosis, and response to treatment; these factors must be taken into account when formulating treatment plans for individual patients and when designing clinical trials.”

David S. Klimstra, MD

Dr. David Klimstra

• Pathologic Classificantion of Neuroendocrine Neoplasms
David S. Klimstra, MD

Abstract: “The pathologic classification of neuroendocrine neoplasms has evolved over the past decades, as new understanding of the biological behavior, histologic characteristics, and genetic features have emerged. Nonetheless, many aspects of the classification systems remain confusing or controversial. Despite these difficulties, much progress has been made in determining the features predicting behavior. Genetic findings have helped establish relationships among different types of neuroendocrine neoplasms and revealed potential therapeutic targets. This review summarizes the current approach to the diagnosis, classification, grading, and therapeutic stratification of neuroendocrine neoplasms, with a focus on those arising in the lung and thymus, pancreas, and intestines.”

• Clinical Presentation and Diagnosis of Neuroendocrine Tumors
Aaron A. Vinik, MD and Celine Chaya, MD

Aaron Vinik, MD and Celine Chaya, MD

Dr. Aaron Vinik and Dr. Celine Chaya

Abstract: “Neuroendocrine tumors (NETs) are slow-growing neoplasms capable of storing and secreting different peptides and neuroamines. Some of these substances cause specific symptom complexes, whereas others are silent. They usually have episodic expression, and the diagnosis is often made at a late stage. Although considered rare, the incidence of NETs is increasing. For these reasons, a high index of suspicion is needed. In this article, the different clinical syndromes and the pathophysiology of each tumor as well as the new and emerging biochemical markers and imaging techniques that should be used to facilitate an early diagnosis, follow-up, and prognosis are reviewed.”

• Surgical Treatment of Small Bowel Neuroendocrine Tumors
Heather A. Farley, MD and Rodney F. Pommier, MD

Rodney Pommier, MD

Dr. Rodney Pommier

Abstract: “Neuroendocrine tumors of the small bowel are rare, slow-growing malignancies that commonly metastasize to nodes at the root of the mesentery and the liver. Liver metastases are associated with carcinoid syndrome. Mesenteric nodal masses can cause bowel obstruction, intestinal angina, or variceal hemorrhage. Patients die of liver failure or bowel obstruction. Primary resection is associated with improved survival rates. Selected patients may benefit from liver debulking operations. Liver resection has excellent survival rates even in the event of an incomplete resection, as well as improvement in hormonal symptoms. Radiofrequency ablation can help to preserve hepatic parenchyma during resection.”

• Systemic Therapies for Advanced Gastrointestinal Carcinoid Tumors
Claire K. Mulvey, MD and Emily K. Bergsland, MD

Emily Bergsland, MD_2

Emily Bergsland, MD

Abstract: “Well-differentiated gastrointestinal neuroendocrine tumors (GINETs) tend to be slow growing, but treatment of advanced disease remains a challenge. Somatostatin analogues (SSAs) are considered standard therapy for carcinoid syndrome. SSAs delay tumor progression in advanced well-differentiated gastroenteropancreatic NETs. Cytotoxic chemotherapy and interferon play a limited role in the treatment of nonpancreatic GINETs. There is no standard approach to treatment of patients with disease progression. Identification of systemic agents with antitumor activity in advanced disease remains an unmet medical need. Enrollment to clinical trials is encouraged; potential therapeutic targets include the vascular endothelial growth factor and mammalian target of rapamycin signaling pathways.”

• Bronchial and Thymic Carcinoid Tumors

Anya Litvak, MD and M. Catherine Pietanza, MD

Abstract: “Bronchial and thymic carcinoids are rare. We present epidemiologic data and potential risk factors. The approach to bronchial and thymic carcinoid patients is discussed, from the initial diagnosis and evaluations to treatment. These malignancies follow staging systems of their site of origin. Because bronchial and thymic carcinoids are rare, we use many treatment strategies that have been demonstrated in gastrointestinal and pancreatic neuroendocrine tumors. The lack of information regarding efficacy in bronchial and thymic carcinoids, as well as the scarcity of therapeutic options available, demands the importance of clinical trials that include these patients.”

Thomas Clancy, MD

Dr. Thomas Clancy

• Surgical Management of Pancreatic Neuroendocrine Tumors
Thomas E. Clancy, MD

Abstract: “Pancreatic neuroendocrine tumors (PNETs) are uncommon tumors with a range of clinical behavior. Some PNETs are associated with symptoms of hormone secretion, with increased systemic levels of insulin, gastrin, glucagon, or other hormones. More commonly, PNETs are nonfunctional, without hormone secretion. Surgical resection is the mainstay of therapy, particularly for localized disease. Surgical therapy must be tailored to tumor and clinical characteristics. Resection may be particularly indicated in the setting of hormone hypersecretion. Small, incidental PNETs are increasingly managed nonoperatively. Surgery may also be indicated in some instances of metastatic disease, if all metastatic foci may be removed.”

• Systemic Therapies for Advanced Pancreatic Neuroendocrine Tumors
Nitya Raj, MD and Diane Reidy-Lagunes, MD

Abstract: “Pancreatic neuroendocrine tumors are an uncommon tumor type and compose 1% to 2% of all pancreatic neoplasms. They are rarely localized at presentation and are typically diagnosed in the presence of metastatic disease. The management poses a significant challenge because of the heterogeneous clinical presentations and varying degrees of aggressiveness. A variety of systemic therapies have been developed for the management of pancreatic neuroendocrine tumors, including somatostatin analogues, a select group of cytotoxic chemotherapy agents, and targeted or biological agents. This article reviews the available systemic therapy options for advanced pancreatic neuroendocrine tumors.”

Nitya Raj, MD and Diane Reidy-Lagunes, MD

Dr. Nitya Raj (left) and Dr. Diane Reidy-Lagues (right)


•Pheochromocytoma and Paraganglioma: Genetics, Diagnosis, and Treatment
Lauren Fishbein, MD

Abstract: “Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare but unique neuroendocrine tumors. The hypersecretion of catecholamines from the tumors can be associated with high morbidity and mortality, even when tumors are benign. Up to 40% of PCCs/PGLs are associated with germline mutations in susceptibility genes. About one-quarter are malignant, defined by the presence of distant metastases. Treatment options for unresectable metastatic disease, including chemotherapy, 131I-MIBG, and radiation, can offer limited tumor and hormone control, although none are curative. This article reviews the inherited genetics, diagnosis, and treatment of PCCs and PGLs.”

Dr. Lauren Fishbein

Dr. Lauren Fishbein


• Poorly Differentiated Neuroendocrine Tumors
Jennifer R. Eads, MD

Abstract: “Poorly differentiated neuroendocrine carcinomas of the gastrointestinal tract are rare and limited data are available to guide treatment. These tumors have been treated akin to small cell lung cancer given the histologic similarities. Their pathology is complex. Over the last decade, these tumors are shown as likely a distinct disease entity that is more heterogeneous than accounted for by the current classification system. This article discusses the epidemiology, prognosis, clinical presentation and pathologic nuances and provides a review of the existing clinical data in poorly differentiated neuroendocrine carcinomas and small cell lung cancers.”

Dr. Jennifer Eads

Dr. Jennifer Eads

• Role of Somatostatin Analogues in the Treatment of Neuroendocrine Tumors
Sujanta Narayana, MD and Pamela L. Kunz, MD

Dr. Pamela Kunz

Dr. Pamela Kunz

Abstract: “Neuroendocrine tumors (NETs) are rare epithelial neoplasms with neuroendocrine differentiation originating most commonly in the lungs and gastroenteropancreatic. Treatment includes surgery and other local therapies; treatment of inoperable disease centers around symptom management and control of tumor growth. Somatostatin analogues (SSAs) have been a mainstay of managing hormone-related symptoms. Emerging evidence suggests that they are effective therapies for r control also. Peptide receptor radionuclide therapy with radiolabeled SSAs is a new, promising treatment for inoperable or metastatic NETs. This article reviews the role of SSAs in the treatment of NETs.”

•Peptide Receptor Radionuclide Therapy in the Treatment of Neuroendocrine Tumors
Dik J. Kwekkeboom, MD and Eric P. Krenning, MD

Abstract: “Peptide receptor radionuclide therapy (PRRT) is a promising new treatment modality for inoperable or metastasized gastroenteropancreatic neuroendocrine tumors patients. Most studies report objective response rates in 15% to 35% of patients. Progression-free (PFS) and overall survival (OS) compare favorably with that for somatostatin analogues, chemotherapy, or newer, “targeted” therapies. Prospective, randomized data regarding the potential PFS and OS benefit of PRRT compared with standard therapies is anticipated.”

• Hepatic-directed Therapies in Patients with Neuroendocrine Tumors

Andrew S. Kennedy, MD

Dr. Andrew Kenndy

Dr. Andrew Kennedy

Abstract: “Neuroendocrine tumors (NETs) of the gastrointestinal (GI) tract have a propensity for producing hepatic metastases. Most GI NETs arise from the foregut or midgut, are malignant, and can cause severe debilitating symptoms adversely affecting quality of life. Aggressive treatments to reduce symptoms have an important role in therapy. Patients with GI NETs usually present with inoperable metastatic disease and severe symptoms from a variety of hormones and biogenic amines. This article describes intra-arterial hepatic-directed therapies for metastases from NETs, a group of treatments in which the therapeutic and/or embolic agents are released intra-arterially in specific hepatic vessels to target tumors.”



• Clinical Trial Design in Neuroendocrine Tumors
Daniel M. Halperin, MD and James C. Yao, MD

Abstract: “Neuroendocrine tumors (NETs) present tremendous opportunities for productive clinical investigation, but substantial challenges as well. Investigators must be aware of common pitfalls in study design, informed by an understanding of the history of trials in the field, to make the best use of available data and our patient volunteers. We believe the salient issues in clinical trial design and interpretation in the NET field are patient homogeneity, standardized response assessment, and rigorous design and execution. Whether designing or interpreting a study in patients with NET, these principles should drive assessment.”
Dr. Daniel Halperin (left) and Dr. James Yao (right)

Dr. Daniel Halperin (left) and Dr. James Yao (right)


Print Friendly, PDF & Email