Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
Annals of Surgical Oncology
Published in: Annals of Surgical Oncology 13/2020

01-12-2020 | Surgery | Peritoneal Surface Malignancy

Impact of Mucin Cellularity and Distribution on Survival in Newly Diagnosed Patients with Low-Grade Appendiceal Mucinous Neoplasm Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy

Authors: Andrei Nikiforchin, MD, Mary Caitlin King, BS, Ekaterina Baron, MD, Ryan MacDonald, PhD, Michelle Sittig, RN, Carol Nieroda, MD, Vadim Gushchin, MD, FACS, Armando Sardi, MD, FACS

Published in: Annals of Surgical Oncology | Issue 13/2020

Login to get access

Abstract

Background

Low-grade appendiceal mucinous neoplasms (LAMN) are tumors that frequently present with peritoneal spread of either acellular mucin (AM) or cellular mucin (CM). We aim to determine how mucin types and distribution affect survival.

Patients and Methods

A retrospective cohort study was conducted using a prospective database. Newly diagnosed LAMN patients with AM versus CM treated with cytoreductive surgery/hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) were compared. Postoperative pathology reports were reviewed to assess each involved abdominal zone. Survival was analyzed using the Kaplan–Meier method.

Results

Of 121 identified patients, 50 (41%) had peritoneal lesions with AM and 71 (59%) with CM. Peritoneal cancer index was lower in AM versus CM (mean: 19 ± 13 vs 28 ± 10, p = 0.004), but complete cytoreduction (CC) rates were similar (98% vs 96%, p = 0.642). The 5-year progression-free survival (PFS) was higher in AM versus CM (96% vs 69.8%, p = 0.002). CM patients had zones with both types of lesions: with and without cells. The CM subgroup analysis showed significant differences in 5-year progression-free survival (PFS) among patients with 1–3, 4–7, and 8–10 zones with cells (95.2%, 68.4%, and 35.7%, respectively, p < 0.001), but PFS was not affected by the number of zones with any lesion type. There was no difference in overall survival (OS) between groups.

Conclusions

Despite comparable CC rates after CRS/HIPEC, CM patients have shorter PFS than AM patients. In CM patients, more zones with cells, but not the total number of involved zones, negatively impact PFS. Mucin type does not impact OS. It is important to assess and report mucin cellularity in LAMN specimens.
Literature
1.
Smeenk RM, van Velthuysen ML, Verwaal VJ, Zoetmulder FA. Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol. 2008;34(2):196–201.CrossRef
2.
Carr NJ, Bibeau F, Bradley RF, et al. The histopathological classification, diagnosis and differential diagnosis of mucinous appendiceal neoplasms, appendiceal adenocarcinomas and pseudomyxoma peritonei. Histopathology. 2017;71(6):847–58.CrossRef
3.
Misdraji J. Mucinous epithelial neoplasms of the appendix and pseudomyxoma peritonei. Mod Pathol. 2015;28(Suppl 1):S67–79.CrossRef
4.
5.
Moran B, Baratti D, Yan TD, Kusamura S, Deraco M. Consensus statement on the loco-regional treatment of appendiceal mucinous neoplasms with peritoneal dissemination (pseudomyxoma peritonei). J Surg Oncol. 2008;98(4):277–82.CrossRef
6.
Carr NJ, Cecil TD, Mohamed F, et al. A consensus for classification and pathologic reporting of pseudomyxoma peritonei and associated appendiceal neoplasia: the results of the Peritoneal Surface Oncology Group International (PSOGI) modified Delphi process. Am J Surg Pathol. 2016;40(1):14–26.CrossRef
7.
Roxburgh CS, Fenig YM, Cercek A, et al. Outcomes of low-grade appendiceal mucinous neoplasms with remote acellular mucinous peritoneal deposits. Ann Surg Oncol. 2019;26(1):118–24.CrossRef
8.
Pai RK, Beck AH, Norton JA, Longacre TA. Appendiceal mucinous neoplasms: clinicopathologic study of 116 cases with analysis of factors predicting recurrence. Am J Surg Pathol. 2009;33(10):1425–39.CrossRef
9.
Guaglio M, Sinukumar S, Kusamura S, et al. Clinical surveillance after macroscopically complete surgery for low-grade appendiceal mucinous neoplasms (LAMN) with or without limited peritoneal spread: long-term results in a prospective series. Ann Surg Oncol. 2018;25(4):878–84.CrossRef
10.
Choudry HA, Pai RK. Management of mucinous appendiceal tumors. Ann Surg Oncol. 2018;25(8):2135–44.CrossRef
11.
Carr NJ, McCarthy WF, Sobin LH. Epithelial noncarcinoid tumors and tumor-like lesions of the appendix. A clinicopathologic study of 184 patients with a multivariate analysis of prognostic factors. Cancer. 1995;75(3):757–68.CrossRef
12.
van Eden WJ, Kok NFM, Snaebjornsson P, et al. Factors influencing long-term survival after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei originating from appendiceal neoplasms. BJS Open. 2019;3(3):376–86.CrossRef
13.
Reghunathan M, Kelly KJ, Valasek MA, Lowy AM, Baumgartner JM. Histologic predictors of recurrence in mucinous appendiceal tumors with peritoneal dissemination after HIPEC. Ann Surg Oncol. 2018;25(3):702–8.CrossRef
14.
McCusker ME, Cote TR, Clegg LX, Sobin LH. Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973–1998. Cancer. 2002;94(12):3307–12.CrossRef
15.
Chua TC, Moran BJ, Sugarbaker PH, et al. Early-and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Clin Oncol. 2012;30(20):2449–56.CrossRef
16.
Elias D, Gilly F, Quenet F, et al. Pseudomyxoma peritonei: a French multicentric study of 301 patients treated with cytoreductive surgery and intraperitoneal chemotherapy. Eur J Surg Oncol. 2010;36(5):456–62.CrossRef
17.
Jimenez W, Sardi A, Nieroda C, et al. Predictive and prognostic survival factors in peritoneal carcinomatosis from appendiceal cancer after cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol. 2014;21(13):4218–25.CrossRef
18.
Jacquet P, Sugarbaker PH. Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. Cancer Treat Res. 1996;82:359–74.CrossRef
19.
Choudry HA, Pai RK, Shuai Y, et al. Impact of cellularity on oncologic outcomes following cytoreductive surgery and hyperthermic intraperitoneal chemoperfusion for pseudomyxoma peritonei. Ann Surg Oncol. 2018;25(1):76–82.CrossRef
20.
Ihemelandu C, Fernandez S, Sugarbaker PH. A prognostic model for predicting overall survival in patients with peritoneal surface malignancy of an appendiceal origin treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol. 2017;24(8):2266–72.CrossRef
21.
Paul BK, Ihemelandu C, Sugarbaker PH. Prior surgical score: an analysis of the prognostic significance of an initial nondefinitive surgical intervention in patients with peritoneal carcinomatosis of a colorectal origin undergoing cytoreductive surgery and perioperative intraperitoneal chemotherapy. Dis Colon Rectum. 2018;61(3):347–54.CrossRef
22.
Clavien PA, Barkun J, de Oliveira ML, et al. The Clavien–Dindo classification of surgical complications: five-year experience. Ann Surg. 2009;250(2):187–96.CrossRef
23.
Shankar S, Ledakis P, El Halabi H, Gushchin V, Sardi A. Neoplasms of the appendix: current treatment guidelines. Hematol Oncol Clin North Am. 2012;26(6):1261–90.CrossRef
24.
Enblad M, Birgisson H, Wanders A, Skoldberg F, Ghanipour L, Graf W. Importance of absent neoplastic epithelium in patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol. 2016;23(4):1149–56.CrossRef
25.
Yantiss RK, Shia J, Klimstra DS, Hahn HP, Odze RD, Misdraji J. Prognostic significance of localized extra-appendiceal mucin deposition in appendiceal mucinous neoplasms. Am J Surg Pathol. 2009;33(2):248–55.CrossRef
26.
Huang Y, Alzahrani NA, Chua TC, Morris DL. Histological subtype remains a significant prognostic factor for survival outcomes in patients with appendiceal mucinous neoplasm with peritoneal dissemination. Dis Colon Rectum. 2017;60(4):360–7.CrossRef
27.
Swellengrebel HA, Zoetmulder FA, Smeenk RM, Antonini N, Verwaal VJ. Quantitative intra-operative assessment of peritoneal carcinomatosis—a comparison of three prognostic tools. Eur J Surg Oncol. 2009;35(10):1078–84.CrossRef
28.
Fallis SA, Moran BJ. Management of pseudomyxoma peritonei. J BUON. 2015;20(Suppl 1):S47–55.PubMed
29.
Glehen O, Gilly FN, Boutitie F, et al. Toward curative treatment of peritoneal carcinomatosis from nonovarian origin by cytoreductive surgery combined with perioperative intraperitoneal chemotherapy: a multi-institutional study of 1,290 patients. Cancer. 2010;116(24):5608–18.CrossRef
30.
Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol. 2006;7(1):69–76.CrossRef
31.
McDonald JR, O’Dwyer ST, Rout S, et al. Classification of and cytoreductive surgery for low-grade appendiceal mucinous neoplasms. Br J Surg. 2012;99(7):987–92.CrossRef
32.
Foster JM, Sleightholm RL, Wahlmeier S, Loggie B, Sharma P, Patel A. Early identification of DPAM in at-risk low-grade appendiceal mucinous neoplasm patients: a new approach to surveillance for peritoneal metastasis. World J Surg Oncol. 2016;14(1):243.CrossRef
33.
34.
Levine EA, Votanopoulos KI, Qasem SA, et al. Prognostic molecular subtypes of low-grade cancer of the appendix. J Am Coll Surg. 2016;222(4):493–503.CrossRef
35.
Tokunaga R, Xiu J, Johnston C, et al. Molecular profiling of appendiceal adenocarcinoma and comparison with right-sided and left-sided colorectal cancer. Clin Cancer Res. 2019;25(10):3096–103.CrossRef
Metadata
Title
Impact of Mucin Cellularity and Distribution on Survival in Newly Diagnosed Patients with Low-Grade Appendiceal Mucinous Neoplasm Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy
Authors
Andrei Nikiforchin, MD
Mary Caitlin King, BS
Ekaterina Baron, MD
Ryan MacDonald, PhD
Michelle Sittig, RN
Carol Nieroda, MD
Vadim Gushchin, MD, FACS
Armando Sardi, MD, FACS
Publication date
01-12-2020
Publisher
Springer International Publishing
Published in
Annals of Surgical Oncology / Issue 13/2020
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-020-08535-1

Other articles of this Issue 13/2020

Annals of Surgical Oncology 13/2020 Go to the issue

ASO Author Reflections

ASO Author Reflections: Prognostic Impact of Budding Grade on Patients with Residual Liver Recurrence of Colorectal Cancer After Initial Hepatectomy

Peritoneal Surface Malignancy

Benchmarking Perioperative Outcomes of Cytoreductive Surgery for Cancer: Implications for Quality Measurement

ASO Author Reflections

ASO Author Reflections: To Redo or Not to Redo, That is the Question: Assessing the Slings and Arrows of Repeat CRS/HIPEC in Patients with Recurrent or Progressive Peritoneal Carcinomatosis

ASO Author Reflections

ASO Author Reflections: Laparoscopic Surgery of Perihilar Cholangiocarcinoma Between Oncologic Adequacy and Technical Challenges

ASO Author Reflections

ASO Author Reflections: Preoperative Nutritional Care: The ‘Cinderella’ of Surgical Management in Patients with Pancreatic Cancer

Breast Oncology

Radioactive Seed Versus Wire-Guided Localization for Ductal Carcinoma in Situ of the Breast: Comparable Resection Margins