National Comprehensive Cancer Network

Clinical Recommendations

JNCCN – The Journal of the National Comprehensive Cancer Network

Table of Contents - Volume 7, Number 7 July 2009

• NCCN Clinical Practice Guidelines in Oncology™
• Neuroendocrine Tumors
• Special Feature
• Practice Patterns and Guideline Adherence of Medical Oncologists in Managing Patients with Early Breast Cancer
• Featured Articles
• Management of Adrenocortical Carcinoma
• Treatment of Metastatic Carcinoid Tumors With Radiolabeled Biologic Molecules
• Role of Interventional Radiology in the Treatment of Patients with Neuroendocrine Metastases in the Liver

NCCN Clinical Practice Guidelines in Oncology™

Neuroendocrine Tumors

Neuroendocrine tumors affect cells throughout the nervous and endocrine systems that produce and secrete regulatory hormones. Common sites of origin include the endocrine pancreas; parathyroid, adrenal, and pituitary glands; calcitonin‑producing cells of the thyroid (causing medullary thyroid carcinoma); and argentaffin cells of the gut (causing carcinoid tumors). Neuroendocrine tumors are rare and can be broadly subdivided into those with and those without a clinical syndrome, termed functional or nonfunctional, respectively. Neuroendocrine tumors can also arise sporadically (nonhereditary tumors) or as a result of genetic predisposition (hereditary tumors). Functionally active neuroendocrine tumors present with clinical symptoms of excessive hormone release from the tumor cells. Most neuroendocrine tumors (with the exception of insulinomas, which are often benign) are malignant, and metastasize commonly to lymph nodes and the liver or less commonly to bone, lung, brain, and other organs. Despite the widespread metastasis, these tumors are typically slow‑growing with a low mitotic activity, and often have an insidious presentation. These guidelines discuss the diagnosis and management of neuroendocrine tumors and special considerations relating to these tumors.

Special Feature

Practice Patterns and Guideline Adherence of Medical Oncologists in Managing Patients with Early Breast Cancer
Jill A Foster, MD, MPH; Maziar Abdolrasulnia, PhD; Hamidreza Doroodchi, MD; Joan McClure, MS; and Linda Casebeer, PhD

Studies of adherence to breast cancer guidelines have often focused on primary therapies, but concordance with other guideline recommendations has not been examined as extensively. Oncologists often make guideline-consistent choices, but discordant clinical decisions may occur in important aspects of care for early breast cancer. Broadening the diffusion and adoption of guidelines recommendations is an important mechanism for addressing these gaps and may substantially improve the quality of breast cancer care. This study assesses the knowledge and practice patterns of medical oncologists in the United States to inform education and quality improvement initiatives that can improve breast cancer care.

Featured Articles

Management of Adrenocortical Carcinoma
Jonathan R. Strosberg, MD; Gary D. Hammer, MD; and Gerard M. Doherty, MD

Adrenocortical carcinomas (ACCs) are rare tumors that arise from the adrenal gland cortex with an incidence of 1 to 2 per million. The rarity of this tumor translates into a paucity of experience in managing patients in most medical centers. Because clinical series are small and prospective evaluation of treatment strategies is limited, the current state of knowledge is strongly influenced by expert consensus opinion from a few medical centers specializing in ACCs. This article describes the basic diagnostic and prognostic issues in adrenal cancer management, and presents detailed rationales for therapeutic management.

Treatment of Metastatic Carcinoid Tumors With Radiolabeled Biologic Molecules
David Bushnell, MD

Treatment options in advanced-stage neuroendocrine tumors are limited. A promising new category of therapy was recently introduced for these tumors in which radioactive atoms are attached to molecules that target and bind to neuroendocrine cancer cells. ⁹⁰Yttrium-DOTA-Phe1-Tyr3-octreotides are radioactive drugs that target cells by binding to somatostatin receptors. ¹³¹Iodine-metaiodobenzylguanidine also targets neuroendocrine tumors using the amine transporter system. Both agents, along with the somatostatin analogue ¹⁷⁷Lutetium-DOTATATE, have shown objective response rates in approximately 30% of patients with progressive metastatic disease. Symptomatic improvement is observed in most patients receiving these drugs and evidence of survival benefit is also mounting. Targeted radionuclide therapy using the radiopharmaceuticals discussed in this article may be considered in any patient with an advanced-stage carcinoid tumor, particularly in the presence of progressive disease or significant tumor-related symptoms.

Role of Interventional Radiology in the Treatment of Patients with Neuroendocrine Metastases in the Liver
Larry K. Kvols, MD; Kiran K. Turaga, MD, MPH; Jonathan Strosberg, MD; and Junsung Choi, MD

Management of liver metastasis from neuroendocrine tumors routinely includes an interventional radiologist. Hepatic artery embolization, either bland (transarterial embolization) or with chemotherapy (transarterial chemoembolization), has been shown to improve response rates in neuroendocrine metastases to the liver. Radiofrequency ablation, cryotherapy, and percutaneous alcohol ablation are important adjuncts or alternative treatments to surgical debulking. This article examines important techniques in the armamentarium of the interventional radiologist.