Surgery of the primary tumour in 201 patients with high-grade gastroenteropancreatic neuroendocrine and mixed neuroendocrine-non-neuroendocrine neoplasms,Journal of Neuroendocrinology

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Surgery of the primary tumour in 201 patients with high-grade gastroenteropancreatic neuroendocrine and mixed neuroendocrine-non-neuroendocrine neoplasms
Journal of Neuroendocrinology ( IF 3.3 ) Pub Date : 2021-03-26 , DOI: 10.1111/jne.12967
Hans-Christian Pommergaard _{1,

2} , Kirstine Nielsen _{1,

2,

3} , Halfdan Sorbye _{4,

5} , Birgitte Federspiel _{1,

6} , Elizaveta M Tabaksblat _{7,

8} , Lene W Vestermark ₉ , Eva T Janson _{10,

11} , Carsten P Hansen _{1,

2} , Morten Ladekarl _{7,

8,

12} , Herish Garresori ₁₃ , Geir O Hjortland _{14,

15} , Anna Sundlöv ₁₆ , Renate Galleberg ₄ , Pauline Knigge _{1,

3,

17} , Andreas Kjaer _{1,

17} , Seppo W Langer _{1,

18} , Ulrich Knigge _{1,

2,

3}

Affiliation

ENETS Neuroendocrine Tumor Centre of Excellence, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Department of Surgery and Transplantation, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Department of Endocrinology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Department of Oncology, Haukeland University Hospital, Bergen, Norway.
Department of Clinical Science, University of Bergen, Bergen, Norway.
Department of Pathology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
ENETS Neuroendocrine Tumor Centre of Excellence, Aarhus University Hospital, Aarhus, Denmark.
Department of Oncology, Aarhus University Hospital, Aarhus, Denmark.
Department of Oncology, Odense University Hospital, Odense, Denmark.
ENETS Neuroendocrine Tumor Centre of Excellence, Uppsala University Hospital, Uppsala, Sweden.
Department of Medical Sciences, Endocrine Oncology, Uppsala University, Uppsala, Sweden.
Department of Oncology, Clinical Cancer Research Center, Aalborg University Hospital, Aalborg, Denmark.
Department of Oncology, Stavanger University Hospital, Stavanger, Norway.
ENETS Neuroendocrine Tumor Centre of Excellence, Oslo University Hospital, Oslo, Norway.
Department of Oncology, Oslo University Hospital, Oslo, Norway.
Department of Oncology, Skåne University Hospital, Lund, Sweden.
Department of Clinical Physiology, Nuclear Medicine & PET and Cluster for Molecular Imaging, University of Copenhagen and Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Department of Oncology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.

The benefit of surgery in high-grade gastroenteropancreatic neuroendocrine neoplasms (GEP NEN) and mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) is uncertain. The present study aimed to investigate outcomes after tumour surgery in patients with high-grade (Ki-67 > 20%) GEP NEN or MiNEN stage I-III or stage IV. We analysed data from patients treated in the period 2007-2015 at eight Nordic university hospitals. Overall survival (OS) and progression-free survival (PFS)/disease-free survival (DFS) were analysed by Kaplan-Meier estimates. Prognostic factors were evaluated using Cox regression. We included 201 surgically resected patients, 143 stage I-III and 58 stage IV, with 68% having neuroendocrine carcinoma, 23% MiNEN, 5% neuroendocrine tumour G3 and 4% uncertain NEN G3. Primary tumours were located in colon/rectum (52%), oesophagus/cardia (19%), pancreas (10%), stomach (7%), jejunum/ileum (5%), duodenum (4%), gallbladder (2%) and anal canal (1%). For patients with stage I-III, median DFS was 12 months (95% confidence interval [CI] = 5.5-18.5) and median OS was 32 months (95% CI = 24.0-40.0). For patients with stage I-III and an R0 resection, median DFS was 21 months (95% CI = 4.9-37.1) and median OS was 39 months (95% CI = 25.0-53.0). For patients with stage IV, median PFS/DFS was 4 months (95% CI = 1.9-6.1) and median OS was 11 months (95% CI = 4.8-17.2). For patients with stage IV and an R0 resection, median DFS was 6 months (95% CI = 0-16.4) and median OS was 32 months (95% CI = 25.5-38.5). Performance status > 1 and colorectal primary were associated with poor prognosis. There was no difference in survival between patients with high-grade GEP NEN and MiNEN. Surgery of the primary tumour in patients with loco-regional high-grade GEP NEN or MiNEN led to good long-term results and should be considered if an R0 resection is considered achievable. Highly selected patients with stage IV disease may also benefit from surgery.

中文翻译：

201例高级别胃肠胰神经内分泌及神经内分泌-非神经内分泌混合肿瘤患者的原发肿瘤手术

高级别胃肠胰腺神经内分泌肿瘤 (GEP NEN) 和混合性神经内分泌-非神经内分泌肿瘤 (MiNEN) 的手术益处尚不确定。本研究旨在调查高级别（Ki-67 > 20%）GEP NEN 或 MiNEN I-III 期或 IV 期患者肿瘤手术后的结果。我们分析了 2007-2015 年期间在北欧八家大学医院接受治疗的患者的数据。通过 Kaplan-Meier 估计分析总生存期 (OS) 和无进展生存期 (PFS)/无病生存期 (DFS)。使用 Cox 回归评估预后因素。我们纳入了 201 名手术切除的患者，143 名 I-III 期和 58 名 IV 期患者，其中 68% 患有神经内分泌癌，23% 患有 MiNEN，5% 患有神经内分泌肿瘤 G3 和 4% 不确定 NEN G3。原发性肿瘤位于结肠/直肠（52%），食道/贲门 (19%)、胰腺 (10%)、胃 (7%)、空肠/回肠 (5%)、十二指肠 (4%)、胆囊 (2%) 和肛管 (1%)。对于 I-III 期患者，中位 DFS 为 12 个月（95% 置信区间 [CI] = 5.5-18.5），中位 OS 为 32 个月（95% CI = 24.0-40.0）。对于 I-III 期和 R0 切除的患者，中位 DFS 为 21 个月（95% CI = 4.9-37.1），中位 OS 为 39 个月（95% CI = 25.0-53.0）。对于 IV 期患者，中位 PFS/DFS 为 4 个月（95% CI = 1.9-6.1），中位 OS 为 11 个月（95% CI = 4.8-17.2）。对于 IV 期和 R0 切除的患者，中位 DFS 为 6 个月（95% CI = 0-16.4），中位 OS 为 32 个月（95% CI = 25.5-38.5）。体能状态 > 1 和结直肠原发与预后不良相关。高级别 GEP NEN 和 MiNEN 患者的生存率没有差异。对局部区域高级别 GEP NEN 或 MiNEN 患者进行原发肿瘤手术可产生良好的长期结果，如果认为可以实现 R0 切除，则应考虑手术。高度选择的 IV 期疾病患者也可能从手术中受益。

更新日期：2021-05-25

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