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胃・腸・膵由来のNETsの組織型・TNM Stage分類・Grade分類による推奨治療法

出典
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1: ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: towards a standardized approach to the diagnosis of gastroenteropancreatic neuroendocrine tumors and their prognostic stratification.
Neuroendocrinology. 2009;90(2):162-6. doi: 10.1159/000182196. Epub 2009 Aug 28.

胃・十二指腸カルチノイドの内視鏡写真および超音波内視鏡像

a:胃カルチノイド(表面に陥凹を伴う粘膜下腫瘤様の形体を示す)。
b:第1層から第3層にかけて低エコーの病変を認める。
C:十二指腸カルチノイド(十二指腸球部に表面に陥凹を伴う粘膜下腫瘤様の形体を示す)。
出典
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1: 著者提供

直腸カルチノイドの内視鏡的粘膜下層剝離術(ESD)

a:直腸に黄色調の粘膜下腫瘤様のカルチノイドを認める。b:周囲を切開する。
c:粘膜下の切開。
d:切開後。
出典
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1: 著者提供

神経内分泌腫瘍(neuroendocine tumor,NETs)のWHO分類(2010年)

従来のカルチノイド腫瘍は神経内分泌腫瘍(NETs)と定義された。
出典
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1: Rindi G, Arnold R, Bosman FT, et al. Nomenclature and classification of neuroendocrine neoplasm of the digestive system. WHO Classification of Tumours of the Digestive System. Eds Bosman FT, Carnerio F, Hruban RH. IARCPress, Lyon, 2010;13-4.http://www.pubcan.org/page.php?pageid=2

NETsのGrade別生存率

Grade G1で加療したものが有意差を持ってG2、G3より予後がよい。
a:前腸NETs
b:中腸・後腸NETs 
出典
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1: TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system.
著者: G Rindi, G Klöppel, H Alhman, M Caplin, A Couvelard, W W de Herder, B Erikssson, A Falchetti, M Falconi, P Komminoth, M Körner, J M Lopes, A-M McNicol, O Nilsson, A Perren, A Scarpa, J-Y Scoazec, B Wiedenmann, all other Frascati Consensus Conference participants, European Neuroendocrine Tumor Society (ENETS)
雑誌名: Virchows Arch. 2006 Oct;449(4):395-401. doi: 10.1007/s00428-006-0250-1. Epub 2006 Sep 12.
Abstract/Text: The need for standards in the management of patients with endocrine tumors of the digestive system prompted the European Neuroendocrine Tumor Society (ENETS) to organize a first Consensus Conference, which was held in Frascati (Rome) and was based on the recently published ENETS guidelines on the diagnosis and treatment of digestive neuroendocrine tumors (NET). Here, we report the tumor-node-metastasis proposal for foregut NETs of the stomach, duodenum, and pancreas that was designed, discussed, and consensually approved at this conference. In addition, we report the proposal for a working formulation for the grading of digestive NETs based on mitotic count and Ki-67 index. This proposal, which needs to be validated, is meant to help clinicians in the stratification, treatment, and follow-up of patients.
Virchows Arch. 2006 Oct;449(4):395-401. doi: 10.1007/s00428-006-0250-1...
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2: Neuroendocrine tumors of midgut and hindgut origin: tumor-node-metastasis classification determines clinical outcome.
著者: Henning Jann, Stephanie Roll, Anne Couvelard, Olivia Hentic, Marianne Pavel, Jacqueline Müller-Nordhorn, Martin Koch, Christoph Röcken, Guido Rindi, Philippe Ruszniewski, Bertram Wiedenmann, Ulrich-Frank Pape
雑誌名: Cancer. 2011 Aug 1;117(15):3332-41. doi: 10.1002/cncr.25855. Epub 2011 Jan 18.
Abstract/Text: BACKGROUND: Prognostic classification of neuroendocrine tumor (NET) patients is difficult due to the complexity of current classification systems. A recent proposal for a tumor-node-metastasis (TNM) classification and a grading system based on the proliferative fraction proved valid in NETs of foregut origin. The purpose of this study was to test the efficacy of a proposal for TNM staging and grading for midgut and hindgut NETs.
METHODS: Two hundred seventy patients with histologically proven midgut and hindgut NETs were investigated. Epidemiological, clinicopathological, and tumor-specific data at initial diagnosis were recorded. Tumors were classified according to the World Health Organization (WHO) and the recent European Neuroendocrine Tumor Society-TNM staging and grading proposal. Survival analysis and statistical testing for independent prognostic factors were performed using log-rank tests and Cox regression.
RESULTS: Of 270 NETs originating in the midgut or hindgut, 7% (5-year survival rate [YSR], 100%) were stage 1, 8% (5-YSR, 100%) were stage 2, 19% (5-YSR, 89.5%) were stage 3, and 66% (5-YSR, 83.3%) were stage 4 NETs; 62% (5-YSR 95.2%) were grade 1, 32% (5-YSR 82.0%) were grade 2, and 6% (5-YSR, 51.4%) were grade 3 NETs. WHO classification significantly separated poorly from well-differentiated NET or carcinomas but did not further discriminate. TNM staging significantly separated stages 1, 2, and 3 from stage 4 NETs, as did grading according to proliferative capacity for all grades. Multivariate analysis confirmed these results, particularly for Ki67 grading.
CONCLUSIONS: The acquired data confirmed the prognostic relevance of the proposed TNM staging and grading system and demonstrated the applicability of these classification tools. The TNM system can therefore facilitate therapeutic stratification and comparison of data from different institutions.

Copyright © 2011 American Cancer Society.
Cancer. 2011 Aug 1;117(15):3332-41. doi: 10.1002/cncr.25855. Epub 2011...

空回腸由来のNETsのカルチノイド症候群の有無による予後の違い

有意差はないが、カルチノイド症候群を伴ったものは予後が悪い傾向にある。
出典
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1: Neuroendocrine tumors of midgut and hindgut origin: tumor-node-metastasis classification determines clinical outcome.
著者: Henning Jann, Stephanie Roll, Anne Couvelard, Olivia Hentic, Marianne Pavel, Jacqueline Müller-Nordhorn, Martin Koch, Christoph Röcken, Guido Rindi, Philippe Ruszniewski, Bertram Wiedenmann, Ulrich-Frank Pape
雑誌名: Cancer. 2011 Aug 1;117(15):3332-41. doi: 10.1002/cncr.25855. Epub 2011 Jan 18.
Abstract/Text: BACKGROUND: Prognostic classification of neuroendocrine tumor (NET) patients is difficult due to the complexity of current classification systems. A recent proposal for a tumor-node-metastasis (TNM) classification and a grading system based on the proliferative fraction proved valid in NETs of foregut origin. The purpose of this study was to test the efficacy of a proposal for TNM staging and grading for midgut and hindgut NETs.
METHODS: Two hundred seventy patients with histologically proven midgut and hindgut NETs were investigated. Epidemiological, clinicopathological, and tumor-specific data at initial diagnosis were recorded. Tumors were classified according to the World Health Organization (WHO) and the recent European Neuroendocrine Tumor Society-TNM staging and grading proposal. Survival analysis and statistical testing for independent prognostic factors were performed using log-rank tests and Cox regression.
RESULTS: Of 270 NETs originating in the midgut or hindgut, 7% (5-year survival rate [YSR], 100%) were stage 1, 8% (5-YSR, 100%) were stage 2, 19% (5-YSR, 89.5%) were stage 3, and 66% (5-YSR, 83.3%) were stage 4 NETs; 62% (5-YSR 95.2%) were grade 1, 32% (5-YSR 82.0%) were grade 2, and 6% (5-YSR, 51.4%) were grade 3 NETs. WHO classification significantly separated poorly from well-differentiated NET or carcinomas but did not further discriminate. TNM staging significantly separated stages 1, 2, and 3 from stage 4 NETs, as did grading according to proliferative capacity for all grades. Multivariate analysis confirmed these results, particularly for Ki67 grading.
CONCLUSIONS: The acquired data confirmed the prognostic relevance of the proposed TNM staging and grading system and demonstrated the applicability of these classification tools. The TNM system can therefore facilitate therapeutic stratification and comparison of data from different institutions.

Copyright © 2011 American Cancer Society.
Cancer. 2011 Aug 1;117(15):3332-41. doi: 10.1002/cncr.25855. Epub 2011...

神経内分泌腫瘍(NETs)肝転移症例の治療のアルゴリズム

出典
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1: Liver metastases of neuroendocrine carcinomas: interventional treatment via transarterial embolization, chemoembolization and thermal ablation.
著者: Thomas J Vogl, Nagy N N Naguib, Stefan Zangos, Katrin Eichler, Alborz Hedayati, Nour-Eldin A Nour-Eldin
雑誌名: Eur J Radiol. 2009 Dec;72(3):517-28. doi: 10.1016/j.ejrad.2008.08.008. Epub 2008 Oct 1.
Abstract/Text: UNLABELLED: The aim of this review article is to provide a practical clinical guideline for indication, technical aspects, protocol guideline and strategies for the interventional treatment of liver metastases from neuroendocrine tumors and focusing on the results of various protocols of management. The response to therapy, in the published articles, is calculated on the basis of the following clinical parameters; including symptomatic response (SR), biologic response (BR), morphological response (MR), progress free survival (PFS), and survival periods (SP). Transarterial chemoembolization (TACE) has been associated with SR rates of 60-95%, BR of 50-90%, MR of 33-80%, SR of 20-80 months, and a 5-year survival of between 50% and 65%. PFS was also between 18 and 24 months. In the transarterial embolization (TAE) group, SR was similar to the TACE group, MR was 32% and 82%, survival was between 18 and 88 months with a survival rate of 40-67%, and BR was between 50% and 69%. Radiofrequency ablation (RFA), either percutaneous or during surgery, has been associated with SR of 71-95% for a mean duration of 8-10 months, BR of 65%, and mean SP of 1.6 years after ablation. The mean survival following surgical resection for operable cases is 4.26 years+/-S.D.: 1.1.
CONCLUSION: The interventional protocols for the management of liver metastases from neuroendocrine tumors: for oligonodular liver metastatic deposits, local resection or RFA and/or LITT is recommended, while in multinodular diseases with higher tumor load, TACE or TAE is recommended.
Eur J Radiol. 2009 Dec;72(3):517-28. doi: 10.1016/j.ejrad.2008.08.008....

胃・腸・膵由来のNETsの組織型・TNM Stage分類・Grade分類による推奨治療法

出典
imgimg
1: ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: towards a standardized approach to the diagnosis of gastroenteropancreatic neuroendocrine tumors and their prognostic stratification.
Neuroendocrinology. 2009;90(2):162-6. doi: 10.1159/000182196. Epub 2009 Aug 28.

胃・十二指腸カルチノイドの内視鏡写真および超音波内視鏡像

a:胃カルチノイド(表面に陥凹を伴う粘膜下腫瘤様の形体を示す)。
b:第1層から第3層にかけて低エコーの病変を認める。
C:十二指腸カルチノイド(十二指腸球部に表面に陥凹を伴う粘膜下腫瘤様の形体を示す)。
出典
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1: 著者提供