Surgical Treatment of Patients with Poorly Differentiated Pancreatic Neuroendocrine Carcinoma: An NCDB Analysis

Kaslow, Sarah R.; Vitiello, Gerardo A.; Prendergast, Katherine M.; Hani, Leena; Cohen, Steven M.; Wolfgang, Christopher L.; Berman, Russell S.; Lee, Ann Y.; Correa‐Gallego, Camilo

doi:10.1245/s10434-022-11477-5

Cited by 11 publications

(15 citation statements)

References 27 publications

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“…A Japanese nationwide survey showed that distant metastasis during diagnosis was observed in 46% of PanNEC cases ( 29 ). Although the prognosis of PanNECs is poor regardless of the treatment, a recent analysis using the American National Cancer Database has found that surgical resection was strongly and independently associated with improved overall survival of PanNECs ( 30 ). In that study, PanNECs had a survival advantage when treated with surgery (median overall survival of 29 months with surgery vs .…”

Section: Discussionmentioning

confidence: 99%

“…In that study, PanNECs had a survival advantage when treated with surgery (median overall survival of 29 months with surgery vs . 7 months without surgery) ( 30 ). In recent years, the efficacy of EUS-guided radiofrequency ablation or ethanol injection therapy has been reported as an alternative to surgery for local treatment of PanNENs ( 31 , 32 ).…”

Section: Discussionmentioning

confidence: 99%

“…Although the prognosis of PanNECs is poor regardless of the treatment, a recent analysis using the American National Cancer Database has found that surgical resection was strongly and independently associated with improved overall survival of PanNECs (30). In that study, PanNECs had a survival advantage when treated with surgery (median overall survival of 29 months with surgery vs. 7 months without surgery) (30). In recent years, the efficacy of EUS-guided radiofrequency ablation or ethanol injection therapy has been reported as an alternative to surgery for local treatment of PanNENs (31,32).…”

Section: Discussionmentioning

confidence: 99%

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Pancreatic neuroendocrine carcinoma with unique morphological features mimicking intraductal papillary mucinous carcinoma: A case report

et al. 2022

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BackgroundPancreatic neuroendocrine carcinoma (PanNEC) is a rare disease entity with rapid progression and poor prognosis. Here, we report a PanNEC case with unique morphological features mimicking intraductal papillary mucinous carcinoma.Case presentationA 69-year-old Japanese man was referred to our hospital for further evaluation of weight loss and deterioration of diabetes mellitus. Contrast-enhanced computed tomography showed a solid and cystic mass with hypo-enhancement at the tail of the pancreas. The main pancreatic duct (MPD) was diffusely dilated without obstruction, accompanied by marked parenchymal atrophy. Multiple peritoneal and omental nodules were observed, suggesting tumor dissemination. Endoscopic retrograde cholangiopancreatography revealed that the mass correlated with the dilated MPD. During pancreatography, a large amount of mucus was extruded from the pancreatic orifice of the ampulla. Based on these imaging findings, intraductal papillary mucinous carcinoma was suspected. Per-oral pancreatoscopy (POPS)-guided tumor biopsies were conducted for the lesion's solid components. Histopathological examination of the biopsied material confirmed small-cell-type PanNEC with a Ki-67 labeling index of 90%. Due to his condition's rapid decline, the patient was given the best supportive care and died 28 days after diagnosis.ConclusionAlthough rare, PanNEC, which correlates with the MPD and is accompanied by marked dilation of the MPD, does exist as one phenotype. In such cases, POPS-guided biopsy could be a useful diagnostic modality.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pancreatic neuroendocrine carcinoma with unique morphological features mimicking intraductal papillary mucinous carcinoma: A case report

et al. 2022

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“…For localized esophageal NEC, initial surgery seems inferior to initial chemoradiation, especially for stage III 46,47 . In a cohort of 630 pancreatic NEC cases, surgery was, after propensity score matching, significantly and independently associated with improved survival (36 vs. 8 months) 48 . In a large colorectal NEC cohort, surgery was a beneficial prognostic factor for OS in a multivariable analysis 49 .…”

Section: Treatmentmentioning

confidence: 95%

“…8 months). 48 In a large colorectal NEC cohort, surgery was a beneficial prognostic factor for OS in a multivariable analysis. 49 In another study of 502 NEC stage I-III 3-year survival after surgery was 40% versus 18% in cases without surgery.…”

Section: Prognostic Factorsmentioning

confidence: 98%

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma

Sørbye

Grande

Pavel

et al. 2023

J Neuroendocrinology

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This ENETS guidance paper, developed by a multidisciplinary working group, provides up‐to‐date and practical advice on the diagnosis and management of digestive neuroendocrine carcinoma, based on recent developments and study results. These recommendations aim to pave the road for more standardized care for our patients resulting in improved outcomes. Prognosis is generally poor for digestive NEC, most are advanced at diagnosis and median survival in metastatic disease is 11–12 months. Surgery can be of benefit for localized disease after extensive preoperative imaging. Carboplatin in combination with etoposide is recommended as first‐line treatment for metastatic disease. Irinotecan with fluoropyrimidines has the best evidence as second‐line treatment. Immunotherapy plays a minor role in biomarker‐unselected patients. Molecular profiling if available is encouraged to identify new targets. More prospective clinical trials are highly needed to fulfil the unmet needs in this field, especially on new predictive and prognostic biomarkers and to improve survival of patients with advanced disease.

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Outcomes after primary tumor resection of metastatic pancreatic neuroendocrine tumors: An analysis of the National Cancer Database

Kaslow

Hani

Cohen

et al. 2023

Journal of Surgical Oncology

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Introduction: There is no consensus regarding the role of primary tumor resection for patients with metastatic pancreatic neuroendocrine tumors (panNET). We assessed surgical treatment patterns and evaluated the survival impact of primary tumor resection in patients with metastatic panNET.Methods: Patients with synchronous metastatic nonfunctional panNET in the National Cancer Database (2004Database ( −2016 were categorized based on whether they underwent primary tumor resection. We used logistic regressions to assess associations with primary tumor resection. We performed survival analyses with Kaplan−Meier survival functions, log-rank test, and Cox proportional hazard regression within a propensity score matched cohort.Results: In the overall cohort of 2613 patients, 68% (n = 839) underwent primary tumor resection. The proportion of patients who underwent primary tumor resection decreased over time from 36% (2004) to 16% (2016, p < 0.001).After propensity score matching on age at diagnosis, median income quartile, tumor grade, size, liver metastasis, and hospital type, primary tumor resection was associated with longer median overall survival (OS) (65 vs. 24 months; p < 0.001) and was associated with lower hazard of mortality (HR: 0.39, p < 0.001). Conclusion:Primary tumor resection was significantly associated with improved OS, suggesting that, if feasible, surgical resection can be considered for well-selected patients with panNET and synchronous metastasis. | INTRODUCTIONPancreatic neuroendocrine tumors (panNETs) are a rare, but increasingly prevalent, set of neoplasms that may be associated with functional hormonal syndromes, multiple endocrine neoplasia type I, and von-Hippel−Lindau syndrome, among others. 1,2 Among pan-NETs, nonfunctional tumors are not only more prevalent but also diagnosed at more advanced stage, with more than half of patients presenting with metastasis at diagnosis. 3 Patients with distant metastases at diagnosis experience significantly shorter overall

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Surgical Treatment of Patients with Poorly Differentiated Pancreatic Neuroendocrine Carcinoma: An NCDB Analysis

Cited by 11 publications

References 27 publications

Pancreatic neuroendocrine carcinoma with unique morphological features mimicking intraductal papillary mucinous carcinoma: A case report

Pancreatic neuroendocrine carcinoma with unique morphological features mimicking intraductal papillary mucinous carcinoma: A case report

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma

Outcomes after primary tumor resection of metastatic pancreatic neuroendocrine tumors: An analysis of the National Cancer Database

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