Case Report - An Uncommon Multimodal Management Approach for Low-Grade Appendiceal Mucinous Neoplasm with Pseudomyxoma Peritonei and Mucinous Borderline Ovarian Tumor

Sep 29, 2026

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An uncommon synchronous case is presented in which low-grade appendiceal mucinous neoplasm (LAMN) coexists with pseudomyxoma peritonei (PMP) and a mucinous borderline ovarian tumor (MBOT), posing diagnostic and therapeutic complexity. A 44-year-old woman with diffuse mucinous peritoneal deposits underwent cytoreductive surgery and then received systemic modified FOLFIRINOX including irinotecan prior to planned second-look HIPEC. Disease-free status is maintained at 1 year.

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Case Report

Introduction

Case presentation

Clinical discussion

Conclusion

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# Case Report An uncommon multimodal management approachfor low-grade appendicealmucinous neoplasmwith pseudomyxoma peritoneiandmucinousborderline ovarian tumor Dipesh Kumar Singh, MBBS*, Isha Dhakal, MBBS, Sanskriti Ghimire, MBBS, Nitesh Pandit, MBBS,Rajeev Sharma, MD, DM, Soniya Dulal, MD, DM Introduction and importance: Low-grade appendicealmucinous neoplasm (LAMN) is a rare epithelial tumor characterized bymucin production and potential dissemination into the peritoneal cavity, resulting in pseudomyxoma peritonei (PMP) . Thecoexistence of LAMN with a mucinous borderline ovarian tumor (MBOT) represents a complex and exceedingly rare clinicalscenario. Unlike the main introduction, this abstract focuses on the clinical and therapeutic significance rather than epidemiology.There are no standardized treatment protocols for synchronous LAMN with MBOT; hence, individualized management is essential.This report highlights an uncommon multimodal strategy combining cytoreductive surgery (CRS), modified FOLFIRINOX che -motherapy incorporating irinotecan, and planned second-look surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) .Case presentation: A 44-year-old woman presented with abdominal distension. Imaging revealed diffuse mucinous peritonealdeposits, thickened appendix, and multilocular ovarian cyst. Preoperative CA-125 was 142 U/mL, and CA 19-9 was 280 U/mL.CRS including appendectomy, right hemicolectomy, omentectomy, and bilateral salpingo-oophorectomy was performed.Histopathology confirmed LAMN with PMP and MBOT. The Peritoneal Cancer Index at primary surgery was 18. A multidisciplinaryteam recommended systemic chemotherapy with modified FOLFIRINOX including irinotecan before second-look HIPEC. Thepatient tolerated the regimen well. Clinical discussion: This case underscores an emerging role of irinotecan-based chemotherapy as partof a tailored multimodaltreatment for low-grade mucinous neoplasms, especially in resource-constrained environments. Conclusion: The patient remained disease-free at 1-year follow-up, highlighting the potential effectiveness and practicality ofirinotecan-based regimens as partof an individualized management strategy for low-grade mucinous neoplasms. Keywords: cytoreductive surgery, FOLFIRINOX, HIPEC, low-grade appendiceal neoplasm, mucinous ovarian tumor,pseudomyxoma peritonei ## Introduction Low-grade appendicealmucinous neoplasm (LAMN) is an uncom -mon epithelial tumor originating from the appendix, characterizedby mucin-producing neoplastic cells that can disseminate mucinwithin the peritoneal cavity, leading to pseudomyxoma peritonei(PMP) [1] . Although each entity is rare, the synchronous occurrenceof LAMN with PMP and a mucinous borderline ovarian tumor(MBOT) represents an exceptionally uncommon clinical presenta -tion that poses diagnostic and therapeutic challenges [2] . B.P. Koirala Institute of Health Sciences, Dharan, Nepal Sponsorships or competing interests that maybe relevant to content are disclosedatthe end of this article. *Corresponding author. Address: B.P. Koirala Institute ofHealth Sciences, Dharan,P.O. Box No. 56700, Nepal. Tel.: +977 9818040566. E-mail: Dipeshkumarsingh2222@gmail.com (D.K. Singh). Copyright © 2025 The Author(s). Published by Wolters Kluwer Health, Inc. This is anopen access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in anymedium, provided the original work is properly cited. Annals of Medicine & Surgery (2026) 88:781–784Received 24 May 2025; Accepted 24 October 2025Published online 4 November 2025http://dx.doi.org/10.1097/MS9.0000000000004235 ### HIGHLIGHTS ● This case report addresses the management of a rare com -bination of low-grade appendiceal mucinous neoplasm,pseudomyxoma peritonei (PMP), and mucinous border -line ovarian tumor. ● The use ofirinotecan as part of a modified FOLFIRINOXregimenin PMP is novel, brid

FAQs

What condition combination makes this case exceptionally rare?

The synchronous coexistence of low-grade appendiceal mucinous neoplasm with pseudomyxoma peritonei and a mucinous borderline ovarian tumor is described as exceptionally uncommon, with diagnostic and therapeutic challenges.

What treatments were included in the multimodal management approach?

Management combined cytoreductive surgery (CRS) followed by modified FOLFIRINOX chemotherapy including irinotecan, with planned second-look hyperthermic intraperitoneal chemotherapy (HIPEC).

How did the patient do at follow-up?

The patient remained disease-free at a 1-year follow-up, supporting the practicality and potential effectiveness of irinotecan-based regimens within individualized management for low-grade mucinous neoplasms.

Sep 29, 2026

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