Appendiceal tumors with glandular and neuroendocrine features exhibiting peritoneal metastases - Critical evaluation of outcome following cytoreductive surgery with perioperative chemotherapy

Barrak, Dany; Desale, Sameer; Yoon, JaeHee J.; Dugan, Michelle; Kodavanti, Preeti Prasad; Sampah, Maame Efua; Sugarbaker, Paul H.

doi:10.1016/j.ejso.2021.01.010

Cited by 6 publications

(14 citation statements)

References 21 publications

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“…The use of CRS-HIPEC in patients with peritoneal spread was investigated in multiple retrospective studies ( 10 , 27 , 29 – 31 , 36 , 37 , 47 , 50 , 51 , 57 , 60 , 65 , 74 ). Most showed a median OS between 17 and 45 months ( 29 , 47 , 50 , 57 , 65 , 74 ). Disease-free survival was reported as 13–16 months ( 51 , 60 ).…”

Section: Resultsmentioning

confidence: 99%

“…In patients with peritoneal metastases who have undergone CRS-HIPEC, lower grade ( 36 , 51 ), a peritoneal cancer index of 0–20 ( 29 , 60 , 74 ), complete resection ( 29 , 35 , 51 , 60 , 65 , 74 ), and adjuvant chemotherapy ( 47 ) have all been associated with longer survival. In a series of 24 patients, OS and PFS was significantly higher in patients with a cytoreductive score of 0 (no evidence of disease after resection) compared to a score of 1 (tumour nodules ≤0.25 cm after resection), and the authors recommend only using a cytoreductive score of 0 as a definition of complete cytoreduction in GCA ( 35 ).…”

Section: Resultsmentioning

confidence: 99%

“…In a series of 24 patients, OS and PFS was significantly higher in patients with a cytoreductive score of 0 (no evidence of disease after resection) compared to a score of 1 (tumour nodules ≤0.25 cm after resection), and the authors recommend only using a cytoreductive score of 0 as a definition of complete cytoreduction in GCA ( 35 ). Neither the administration of neoadjuvant chemotherapy nor the type of perioperative chemotherapy was associated with improved survival ( 29 , 47 ); however, one study found higher in vitro drug sensitivity to docetaxel in GCA than in colonic adenocarcinoma ( p = 0.05) ( 65 ). There were relatively low reported morbidity rates associated with CRS-HIPEC, with grade III or higher morbidity ranging between 13.4% and 30.2% ( 47 , 51 ).…”

Section: Resultsmentioning

confidence: 99%

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Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry

et al. 2022

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BackgroundGoblet cell adenocarcinoma (GCA) of the appendix is a rare and aggressive tumour with varying nomenclature and classification systems. This has led to heterogeneity in published data, and there is a lack of consensus on incidence, survival, and management.MethodsWe provide an overview of GCA with a comprehensive systematic review using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology and a retrospective analysis of all cases recorded in the English National Cancer Registration and Analysis Service database between 1995 and 2018. The Kaplan–Meier estimator was used to calculate overall survival, and Cox proportional hazards regression was used to identify prognostic factors.ResultsThe systematic review demonstrated an incidence of 0.05–0.3 per 100,000 per year among North American registry studies. The 1-, 3-, and 5-year survival rate was 95.5%, 85.9%–87.6%, and 76.0%–80.6%, respectively. Age, stage, and grade were identified as prognostic factors for survival. Our analysis included 1,225 cases. Age-standardised incidence was 0.0335 per year in 1995 and gradually rose to 0.158 per year in 2018. The 1-, 3-, and 5-year survival rate was 90.0% [95% confidence interval (95% CI): 85.4–94.0], 76.0% (95% CI: 73.8–80.9), and 68.6% (95% CI: 65.9–72.2), respectively. On univariate Cox regression analyses, female sex, stage, and grade were associated with worse overall survival. On multivariate analysis, only stage remained a statistically significant prognostic factor.ConclusionsGCA of the appendix is rare, but incidence is increasing. We report a lower incidence and survival than North American registry studies. Higher stage was associated with decreased survival. Further prospective studies are required to establish optimal management.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry

et al. 2022

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“…Patients with a neuroendocrine component to their mucinous appendiceal neoplasm were excluded. 9 To be included in this study, patients had resection of an advanced pseudomyxoma peritonei, total gastrectomy, and a temporary high diverting jejunostomy. Not included in these data are patients who had a partial gastrectomy with gastroduodenal or gastrojejunal anastomosis.…”

Section: Methodsmentioning

confidence: 99%

“…Patients with paraaortic lymph node dissemination or other sites of disease outside the abdomen and pelvis were excluded. Patients with a neuroendocrine component to their mucinous appendiceal neoplasm were excluded 9 …”

Section: Methodsmentioning

confidence: 99%

Treatment of advanced pseudomyxoma peritonei using cytoreductive surgery including total gastrectomy and perioperative chemotherapy

Sugarbaker

Chang

2021

Journal of Surgical Oncology

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Background: Pseudomyxoma peritonei is a disease caused by the widespread distribution of mucinous tumor into the peritoneal space from a perforated appendiceal neoplasm.Methods: All patients in this study had cytoreductive surgery with total gastrectomy plus perioperative intraperitoneal chemotherapy. A high diverting jejunostomy was used on all patients. Patient characteristics, adverse events, and survival were accumulated prospectively.Results: Fifty-eight patients were available for long-term follow-up. In the univariate analysis, features associated with a less favorable outcome included female gender (p = 0.0127), intestinal obstruction before treatment (p = 0.00791), and prior surgical score (PSS) (p = 0.0054). In the multivariate analysis, the two significant variables were grade (p = 0.0458) and PSS (p = 0.0041). Median survival was 12 years with a 5-, 10-, and 20-year survival of 76%, 58%, and 37%, respectively. There were two postoperative deaths (3.4%) and Grades 3 and 4 adverse events in 20 (34.5%) patients.Conclusions: A 10-year survival after cytoreductive surgery, total gastrectomy with temporary high diverting jejunostomy, and perioperative chemotherapy occurred in 58% of these patients with advanced pseudomyxoma peritonei. High-grade disease and extensive prior surgery with a high PSS were associated with reduced benefit.

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Secondary cytoreductive surgery for 86 patients with mucinous appendiceal adenocarcinoma

Sugarbaker¹,

Chang

2023

Journal of Surgical Oncology

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Background After patients have a surgical procedure for a gastrointestinal cancer, follow‐up is indicated. If cancer progression is documented in patients with mucinous appendiceal adenocarcinoma (MACA), a secondary cytoreductive surgery (SCRS) may be considered. Methods In patients who had a prior complete cytoreductive surgery (CRS), variables associated with the index CRS and SCRS were extracted. These variables were statistically assessed for their impact on survival. Results Of 198 MACA patients, 86 (43.4%) had SCRS. The median follow‐up was 5.0 years and the median survival was 7 years. Significant prognostic variables associated with the index CRS by univariant analysis was histopathologic grade of MACA‐Intermediate (MACA‐Int) as compared to other MACA histologic subtypes (p = 0.0164). Significant prognostic variables associated with the SCRS were bowel obstruction (p = 0.0149), interval of CRS to SCRS (p = 0.0059), and completeness of cytoreduction (p = 0.0014). Conclusions In the analysis of variables from SCRS, the interval from CRS to SCRS ≤24 months indicates an aggressive biology of the disease. The CC score of complete versus incomplete decreased median survival from 11 to 4 years. A composite of these two variables allowed prediction of survival of 50% when patients showed these two favorable variables and only 9.1% when these variables were unfavorable.

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Appendiceal tumors with glandular and neuroendocrine features exhibiting peritoneal metastases - Critical evaluation of outcome following cytoreductive surgery with perioperative chemotherapy

Cited by 6 publications

References 21 publications

Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry

Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry

Treatment of advanced pseudomyxoma peritonei using cytoreductive surgery including total gastrectomy and perioperative chemotherapy

Secondary cytoreductive surgery for 86 patients with mucinous appendiceal adenocarcinoma

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