Small intestinal neuroendocrine tumors: Prognostic factors and survival

Bergestuen, Deidi Strickland; Aabakken, Lars; Holm, Kristian; Vatn, Morten H.; Thiis‐Evensen, Espen

doi:10.1080/00365520903082432

Cited by 67 publications

(64 citation statements)

References 33 publications

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“…The role of Ki67 as a prognostic factor in general has been proposed by a number of papers with respect to PETs [18,19,20,21,22], whereas few studies have focused on jejunoileal NETs [23,24]. Recently, it has been demonstrated to be the major predictor for tumor progression in advanced PETs [14].…”

Section: Discussionmentioning

confidence: 99%

Risk Factors for Disease Progression in Advanced Jejunoileal Neuroendocrine Tumors

et al. 2011

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Background: Knowledge of clinical course in advanced jejunoileal neuroendocrine tumors (NETs) is poor. Aim: To investigate progression-free survival (PFS), overall survival (OS), and possible predictors for disease progression (DP) in advanced jejunoileal NETs. Patients and Methods: We carried out a multicenter, retrospective analysis of incoming patients with sporadic advanced jejunoileal NETs. PFS and OS were assessed by Kaplan-Meier analysis. Risk factors for progression were analyzed by the Cox proportional hazards method. Results: Of the 114 patients enrolled, 46.5% had functioning tumors, 93.9% had stage IV disease, and 57.3 and 42.7% were G1 and G2 tumors, respectively. During a median follow-up of 48 months (interquartile range 29–84 months), DP occurred in 61.4% of patients, after 19 months (interquartile range 10–41 months) from diagnosis. Median PFS was 36 months. The 2-year and 5-year PFS were 59 and 33%, respectively, while 5-year OS was 77.5%. Ki67 was the sole strong independent risk factor for unfavorable outcome according to multivariate analysis, being significantly associated with both PFS and OS. Conclusions: DP occurred in the majority of patients with advanced jejunoileal NETs, with median PFS being 36 months. Ki67 was a significant predictor of DP and should be considered in determining appropriate treatments and planning follow-up for these patients.

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Section: Discussionmentioning

confidence: 99%

Risk Factors for Disease Progression in Advanced Jejunoileal Neuroendocrine Tumors

et al. 2011

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“…In addition, increased CgA levels, after excluding causes of false-positive rises, seem to be associated with disease progression (Table 4). Although its sensitivity is low in the setting of small NETs, serial CgA measurements have proven useful in monitoring patients with resected NETs to identify early tumor recurrence (Janson et al 1997, Pirker et al 1998, Bajetta et al 1999, Arnold et al 2008, Ekeblad et al 2008, Ahmed et al 2009, Bergestuen et al 2009, Welin et al 2009, d'Herbomez et al 2010). However, other recent studies failed to demonstrate the interest of CgA for NET monitoring under treatment (Vezzosi et al 2011.…”

Section: Biological Markers Tumor Progression and Therapeutic Respomentioning

confidence: 99%

Evaluating digestive neuroendocrine tumor progression and therapeutic responses in the era of targeted therapies: state of the art

Mestier¹,

Dromain²,

d’Assignies³

et al. 2013

Endocrine-Related Cancer

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Well-differentiated neuroendocrine tumors (NETs) are a group of heterogeneous rare tumors. They are often slow-growing and patients can have very long survival, even at the metastatic stage. The evaluation of tumor progression and therapeutic responses is currently based on Response Evaluation Criteria In Solid Tumors v1.1 (RECIST) criteria. As for other malignancies, RECIST criteria are being reexamined for NETs in the era of targeted therapies because tumor response to targeted therapies is rarely associated with shrinkage, as opposed to prolonged progression-free survival. Therefore, size-based criteria no longer seem to be suitable to the assessment of NET progression and therapeutic responses, especially considering targeted therapies. New imaging criteria, combining morphological and functional techniques, have proven relevant for other malignancies treated with targeted therapies. To date, such studies have rarely been conducted on NETs. Moreover, optimizing the management of NET patients also requires considering clinical, biological, and pathological aspects of tumor evolution. Our objectives herein were to comprehensively review current knowledge on the assessment of tumor progression and early prediction of therapeutic responses and to broaden the outlook on well-differentiated NETs, in the era of targeted therapies.

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“…Nejbenignější skupina má 5leté přežívání 100 %, nejhorší subtyp s vysokým proliferačním indexem vykazuje 3leté přežívání pouze 17 % [9].…”

Section: úVodunclassified

A Case Report: Patient with Metastatic Goblet Cell Carcinoid

Bencsiková

Jurečková²,

Lžičařová³

et al. 2013

Klin Onkol

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¹ Klinika komplexní onkologické péče, Masarykův onkologický ústav, Brno ² Oddělení onkologické patologie, Masarykův onkologický ústav, Brno SouhrnVýchodiska: Karcinoid z pohárkových buněk (goblet cell carcinoid) představuje zvláštní podskupinu apendikálních neoplazií. Jejich patologické charakteristiky a klinické projevy jsou jiné než u klasických karcinoidů a primárních adenokarcinomů apendixu. Správná histopatologická klasifi kace a diagnostika jsou základem pro léčbu a prognózu. Morfologická transformace typického karcinoidu z pohárkových buněk na morfologii adenokarcinomu je pravděpodobně spojena s přídatnými genetickými změnami, proto je nutná subklasifi kace této skupiny nádorů. Výzkum molekulárně genetických změn vede k lepšímu chápání této exotické, ale klinicky dů-ležité skupiny nádorů a jejich správné léčbě. Kazuistika: Uvádíme případ pacientky s metastatickým karcinoidem z pohárkových buněk s postižením terminálního ilea, colon ascendens, ovaria, omenta a masivní peritoneální diseminací, po debulking operaci a paliativní chemoterapii režimem FOLFOX4 s velmi dobrým léčebným efektem, s regresí onemocnění na CT a PET kompletní remisí. Kombinovaná paliativní léčba vedla ke zmírnění klinických symptomů a zlepšení kvality i délky života. Závěr: Správná diagnostika a léčebné úsilí i u rozsáhlého metastatického onemocnění přinášejí prospěch pacientovi. Poznání vzácných nádorů a porozumění jejich bio logii vede ke zlepšení léčebných přístupů. Klíčová slova karcinoid z pohárkových buněk -nádory apendixu -chemoterapie SummaryBackground: Goblet cell carcinoid represents a unique entity of appendiceal neoplasia. Its pathological features and clinical behavior are distinct from the classic carcinoid tumor as well as primary adenocarcinoma of the appendix. Correct histopathological classifi cation and diagnosis provide guidelines for treatment and prognosis. Morphological transformation of the Goblet cell carcinoid from typical Goblet cell carcinoid to adenocarcinoma morphology is likely associated with accumulation of additional genetic changes that is why subclassifi cation of this group of tumors is needed. Investigation of molecular genetic changes could increase our understanding of this exotic but clinically important group of tumors. Case: We present the case of a patient with metastatic goblet cell carcinoid involving terminal ileum, ascendent colon, ovary, omentum and peritoneal spreading, treated with debulking surgery and chemotherapy (FOLFOX4 regimen) with good response, reduction of disease on CT and PET complete remission. Improvement of clinical symptoms as well as quality of life was reached by combined palliative treatment. Conclusion: Correct diagnostics and therapeutic eff orts bring patient benefi t even in metastatic setting. Better knowledge of rare tumors and understanding of their bio logy help improve therapeutic approaches.

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Small intestinal neuroendocrine tumors: Prognostic factors and survival

Abstract: Survival did not improve over the study period. Overall and relative 5-year survival compared favorably with that in population-based studies. Distant metastases, elevated chromogranin A levels, and advanced age were the only independent predictors of poor survival.

Cited by 67 publications

References 33 publications

Risk Factors for Disease Progression in Advanced Jejunoileal Neuroendocrine Tumors

Risk Factors for Disease Progression in Advanced Jejunoileal Neuroendocrine Tumors

Evaluating digestive neuroendocrine tumor progression and therapeutic responses in the era of targeted therapies: state of the art

A Case Report: Patient with Metastatic Goblet Cell Carcinoid

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