|
1
|
Shah MH, Goldner WS, Benson AB, Bergsland E, Blaszkowsky LS, Brock P, Chan J, Das S, Dickson PV, Fanta P, Giordano T, Halfdanarson TR, Halperin D, He J, Heaney A, Heslin MJ, Kandeel F, Kardan A, Khan SA, Kuvshinoff BW, Lieu C, Miller K, Pillarisetty VG, Reidy D, Salgado SA, Shaheen S, Soares HP, Soulen MC, Strosberg JR, Sussman CR, Trikalinos NA, Uboha NA, Vijayvergia N, Wong T, Lynn B, Hochstetler C. Neuroendocrine and Adrenal Tumors, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw 2021; 19:839-868. [PMID: 34340212 DOI: 10.6004/jnccn.2021.0032] [Citation(s) in RCA: 317] [Impact Index Per Article: 79.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Neuroendocrine and Adrenal Gland Tumors focus on the diagnosis, treatment, and management of patients with neuroendocrine tumors (NETs), adrenal tumors, pheochromocytomas, paragangliomas, and multiple endocrine neoplasia. NETs are generally subclassified by site of origin, stage, and histologic characteristics. Appropriate diagnosis and treatment of NETs often involves collaboration between specialists in multiple disciplines, using specific biochemical, radiologic, and surgical methods. Specialists include pathologists, endocrinologists, radiologists (including nuclear medicine specialists), and medical, radiation, and surgical oncologists. These guidelines discuss the diagnosis and management of both sporadic and hereditary neuroendocrine and adrenal tumors and are intended to assist with clinical decision-making. This article is focused on the 2021 NCCN Guidelines principles of genetic risk assessment and counseling and recommendations for well-differentiated grade 3 NETs, poorly differentiated neuroendocrine carcinomas, adrenal tumors, pheochromocytomas, and paragangliomas.
Collapse
Affiliation(s)
- Manisha H Shah
- The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute
| | | | - Al B Benson
- Robert H. Lurie Comprehensive Cancer Center of Northwestern University
| | | | | | - Pamela Brock
- The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute
| | | | | | - Paxton V Dickson
- St. Jude Children's Research Hospital/The University of Tennessee Health Science Center
| | | | | | | | | | - Jin He
- The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins
| | | | | | | | - Arash Kardan
- Case Comprehensive Cancer Center/University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute
| | | | | | | | | | | | | | | | | | | | | | | | | | - Nikolaos A Trikalinos
- Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine
| | | | | | | | - Beth Lynn
- National Comprehensive Cancer Network
| | | |
Collapse
|
|
2
|
Lin J, Zhao Y, Zhou Y, Hao H, He Q, Tian Y, Zou B, Zhao G, Qiu X, Jiang L, Li Z, Xu Y, Xue F, Fu W, Xu Z, Li Y, Li S, Chen J, Zhou X, Zhu Z, Li H, Li Y, Li E, Cai L, Jia G, Xie JW, Li P, Zheng CH, Huang CM. Which Staging System Is More Suitable for Gastric Neuroendocrine Cancer and Mixed Adenoneuroendocrine Carcinomas? A Multicenter Cohort Study. Neuroendocrinology 2020; 111:1130-1140. [PMID: 31940636 DOI: 10.1159/000505924] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2019] [Accepted: 01/11/2020] [Indexed: 11/19/2022]
Abstract
PURPOSE To evaluate whether the European Neuroendocrine Tumor Society (ENETS) system or the 8th American Joint Committee on Cancer (AJCC) staging manual are suitable for gastric neuroendocrine carcinomas and/or mixed adenoneuroendocrine carcinomas (G-NECs/MANECs). METHODS Patients in a multicentric series with G-NEC/MANEC who underwent curative-intent surgical resection for a primary tumor were included. An optimal staging system was proposed base on analysis of the T and N status and validated by the SEER database. RESULTS Compared with the ENETS system, the survival curves of the T category and N category in the 8th AJCC system were better separated and distributed in a more balanced way, but the survival curves of T2 vs. T3, N0 vs. N1, and N3a vs. N3b overlapped. For the T category, the 8th AJCC T category was modified by combining T2 and T3, which was consistent with the T category in the 6th AJCC manual for GC. For the N category, the optimal cut-off values of metastatic lymph nodes using X-tile were also similar to those of the N category in the 6th AJCC system. The Kaplan-Meier plots of the 6th AJCC system showed statistically significant differences between individual substages. Compared with the other 2 classifications, the 6th AJCC system also showed superior prognostic stratification. Similar results were obtained in both multicentric and SEER validation sets. CONCLUSIONS Compared to the 8th AJCC and ENETS systems, the 6th AJCC staging system for GC is more suitable for G-NEC/MANEC and can be adopted in clinical practice.
Collapse
Affiliation(s)
- Jianxian Lin
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, China
- Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, China
- Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, China
| | - YaJun Zhao
- Department of Gastrointestinal Surgery, West District of The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, China
| | - Yanbing Zhou
- Department of Gastrointestinal Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Hankun Hao
- Department of General Surgery, Huashan Hospital, Fudan University, Shanghai, China
| | - Qingliang He
- Department of Gastrointestinal Surgery, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China
| | - Yantao Tian
- Department of Pancreatic and Gastric Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Bingbing Zou
- Department of Gastrointestinal Surgery, The First Affiliated Hospital of Anhui Medical University, Hefei, China
| | - Gang Zhao
- Department of Gastrointestinal Surgery, Renji Hospital, Shanghai Jiaotong University, Shanghai, China
| | - Xiantu Qiu
- Department of Gastrointestinal Surgery and Gastrointestinal Surgery Research Institute, The Affiliated Hospital of Putian University, Putian, China
| | - Linxin Jiang
- Department of Gastrointestinal Surgery, Yantai Yuhuangding Hospital, Yantai, China
| | - Zhi Li
- Department of General Surgery, Henan Cancer Hospital, Zhengzhou, China
| | - Yanchang Xu
- Department of Gastrointestinal Surgery, Fujian Medicine University Teaching Hospital, The First Hospital of PuTian, Putian, China
| | - Fangqin Xue
- Department of Gastrointestinal Surgery, Provincial Clinical Medical College of Fujian Medical University, Fujian Provincial Hospital, Fuzhou, China
| | - Weihua Fu
- Department of General Surgery, Tianjin Medical University General Hospital, Tianjin, China
| | - Zekuan Xu
- Department of General Surgery, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China
| | - Yongxiang Li
- Department of Gastrointestinal Surgery, The First Affiliated Hospital of Anhui Medical University, Hefei, China
| | - Shuliang Li
- Department of Gastrointestinal Surgery, The Second People's Hospital of Liaocheng, Liaocheng, China
| | - Jinping Chen
- Department of Gastrointestinal Surgery, Quanzhou First Hospital Affiliated to Fujian Medical University, Quanzhou, China
| | - Xiaojun Zhou
- Department of Gastroenterology Surgery, The First Affiliated Hospital of Soochow University, Suzhou, China
| | - Zhenggang Zhu
- Department of Gastrointestinal Surgery, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Honglang Li
- Department of Gastrointestinal Surgery, Second Affiliated Hospital, Nanchang University, Nanchang, China
| | - Yong Li
- Department of General Surgery, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China
| | - En Li
- Department of Gastrointestinal Surgery, Meizhou People's Hospital, Meizhou, China
| | - Lisheng Cai
- Department of General Surgery, Zhangzhou Affiliated Hospital of Fujian Medical University, Zhangzhou, China
| | - Guiqing Jia
- Department of Gastrointestinal Surgery, Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital, Chengdu, China
| | - Jian-Wei Xie
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, China
- Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, China
- Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, China
| | - Ping Li
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, China
- Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, China
- Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, China
| | - Chao-Hui Zheng
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, China
- Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, China
- Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, China
| | - Chang-Ming Huang
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, China,
- Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, China,
- Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, China,
| |
Collapse
|
|
3
|
Oronsky B, Ma PC, Morgensztern D, Carter CA. Nothing But NET: A Review of Neuroendocrine Tumors and Carcinomas. Neoplasia 2017; 19:991-1002. [PMID: 29091800 PMCID: PMC5678742 DOI: 10.1016/j.neo.2017.09.002] [Citation(s) in RCA: 466] [Impact Index Per Article: 58.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/09/2017] [Revised: 09/06/2017] [Accepted: 09/07/2017] [Indexed: 02/07/2023]
Abstract
This review covers the diverse topic of neuroendocrine neoplasms (NENs), a relatively rare and heterogeneous tumor type, comprising ~2% of all malignancies, with a prevalence of <200,000 in the United States, which makes it an orphan disease (Basu et al., 2010).1 For functional purposes, NENs are divided into two groups on the basis of clinical behavior, histology, and proliferation rate: well differentiated (low grade to intermediate grade) neuroendocrine tumors and poorly differentiated (high grade) neuroendocrine carcinoma (Bosman et al., 2010)2; this histological categorization/dichotomization is highly clinically relevant with respect to impact on treatment and prognosis even though it is not absolute since a subset of tumors with a low-grade appearance behaves similarly to high-grade lesions. Given the relative dearth of evidenced-based literature about this orphan disease as a whole (Modlin et al., 2008),3 since the focus of most articles is on particular anatomic subtypes of NENs (i.e., gastroenteropancreatic or pulmonary), the purpose of this review is to summarize the presentation, pathophysiology, staging, current standard of care treatments, and active areas of current research.
Collapse
Affiliation(s)
- Bryan Oronsky
- EpicentRx Inc, 4445 Eastgate Mall, Suite 200, San Diego, CA 92121, USA.
| | - Patrick C Ma
- West Virginia University, Mary Babb Randolph Cancer Center, 8901 Wisconsin Ave., PO Box 9162, Morgantown, WV 26506, USA
| | - Daniel Morgensztern
- Washington University School of Medicine, Division of Oncology, 660 S. Euclid, Box 8056, St. Louis, MO 63110, USA
| | - Corey A Carter
- Walter Reed National Military Medical Center, 8901 Wisconsin Ave., Bethesda, MD 20889, USA
| |
Collapse
|
|
7
|
Yang Y, Li LQ, Zheng JT, Chen Q, Wang M. Diagnosis, treatment and prognosis of gastric neuroendocrine carcinoma. Shijie Huaren Xiaohua Zazhi 2014; 22:3831-3834. [DOI: 10.11569/wcjd.v22.i25.3831] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To evaluate the therapeutic effects, treatment strategies, clinicopathologic features, and prognosis of gastric-neuroendocrine carcinoma (G-NEC).
METHODS: According to the 2010 World Health Organization (WHO) classification of NEC, clinical data for 44 patients with G-NEC were analyzed retrospectively, including treatment methods, pathological features and prognosis.
RESULTS: All the 44 patients were followed. The median survival of these patients was 23.5 mo, and the overall 1-, 3- and 5-year survival rates were 70.8%, 25.3% and 12.0%, respectively. Univariate analysis showed that age, gender, tumor size, and tumor location were not correlated with prognosis, but surgical procedure, invasive depth, lymph node and distant metastases were significantly correlated with prognosis.
CONCLUSION: G-NEC has a high recurrence rate and poor prognosis. The prognosis depends on multiple factors such as carcinoma infiltration and metastasis and surgical treatment. Multidisciplinary treatment should be given in these cases.
Collapse
|
|
8
|
Sorbye H, Strosberg J, Baudin E, Klimstra DS, Yao JC. Gastroenteropancreatic high-grade neuroendocrine carcinoma. Cancer 2014; 120:2814-23. [PMID: 24771552 DOI: 10.1002/cncr.28721] [Citation(s) in RCA: 250] [Impact Index Per Article: 22.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2014] [Revised: 03/17/2014] [Accepted: 03/19/2014] [Indexed: 12/15/2022]
Abstract
Gastroenteropancreatic (GEP) neuroendocrine neoplasms are classified as low-grade, intermediate-grade, and high-grade tumors based on morphologic criteria and the proliferation rate. Most studies have been conducted in patients with well differentiated (low-grade to intermediate-grade) neuroendocrine tumors. Data are substantially scarcer on poorly differentiated, high-grade neuroendocrine carcinoma (NEC), which includes the entities of small cell carcinoma and large cell NEC. A literature search of GEP-NEC was performed. Long-term survival was poor even among patients who presented with localized disease. Several studies highlighted heterogeneity within the high-grade NEC category and a need for the further identification of discreet prognostic and predictive groups. Tumors with a Ki-67 proliferation index <55% were less responsive to platinum-based chemotherapy, and patients with such tumors or with well differentiated morphology had better survival than patients who had tumors with poorly differentiated morphology or a higher Ki-67 index. Treatment options beyond platinum-based chemotherapy are emerging. A revision of the World Health Organization high-grade NEC classification seems to be necessary based on recent data. Platinum-based chemotherapy may not be the optimal treatment for patients who have GEP-NEC with a moderately high proliferation rate. Adequate diagnostic and prognostic stratifications constitute the basis for future progress.
Collapse
Affiliation(s)
- Halfdan Sorbye
- Department of Oncology, Haukeland University Hospital, Bergen, Norway
| | | | | | | | | |
Collapse
|
|
9
|
Kamiya-Matsuoka C, Blas-Boria D, Williams MD, Garciarena P, Tummala S, Tremont-Lukats IW. N-type calcium channel antibody-mediated paraneoplastic limbic encephalitis: a diagnostic challenge. J Neurol Sci 2014; 338:188-90. [PMID: 24462122 DOI: 10.1016/j.jns.2014.01.004] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2013] [Revised: 12/26/2013] [Accepted: 01/02/2014] [Indexed: 11/17/2022]
Abstract
BACKGROUND The etiology of encephalitis presents a diagnostic challenge and often remains a mystery. However, current technological advances using antibodies can enable a definitive diagnosis in cases that would previously have been suspected to be idiopathic or viral encephalitis. Our objective is to show that tonsil neuroendocrine carcinoma can present initially as limbic encephalitis mediated by N-type calcium channel antibodies and to highlight the diagnostic confusion before cancer detection. METHODS We report a rare case of neuroendocrine cancer presenting as limbic encephalopathy, Lambert-Eaton myasthenic syndrome and neuropathy. The patient was diagnosed and treated at The University of Texas MD Anderson Cancer Center in November 2011. RESULTS Paraneoplastic limbic encephalitis was diagnosed based on clinical presentation of seizures, short-term memory loss, retrograde amnesia, disorientation, distractibility, and abulia; on the exclusion of brain metastases, CNS infection, stroke, metabolic or nutritional deficits, or medication-related events; and on CSF results with inflammatory findings and an abnormal electroencephalography study that showed seizure activity in the left temporal lobe. Serum paraneoplastic panel was positive for P/Q-type calcium channel antibody and N-type calcium channel antibody. Magnetic resonance imaging of brain was unremarkable. CONCLUSION This case highlights limbic encephalitis as an atypical presentation of neuroendocrine cancer. It also illustrates how treatment of the underlying cancer can reverse limbic encephalitis and Lambert-Eaton myasthenic syndrome in a neuroendocrine carcinoma patient even before the paraneoplastic panel becomes negative.
Collapse
Affiliation(s)
- Carlos Kamiya-Matsuoka
- Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States.
| | - David Blas-Boria
- Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States
| | - Michelle D Williams
- Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States
| | - Pedro Garciarena
- Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States
| | - Sudhakar Tummala
- Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States
| | - Ivo W Tremont-Lukats
- Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States
| |
Collapse
|