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Background: The completeness of cytoreduction (CC) score, which quantifies residual tumor, is a major prognostic factor when treating appendiceal carcinomatosis with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC). Both CC-0 and CC-1 are considered complete cytoreductions (CC-0/1) and associated with the best outcomes. We analyzed if the CC-0/1 definition is reliable across appendiceal histopathologic subtypes.
Methods: A prospective database of CRS/HIPEC patients with appendiceal carcinomatosis from 1998 to 2019 was reviewed to identify patients with CC-0/1. Kaplan-Meier overall survival (OS) and progression-free survival (PFS) by CC-score for each histopathology were calculated.
Results: Overall, 297 patients had CC-0/1. Mean age was 54 ± 12 years with 67% females. Histopathologic subtypes were 45% low-grade mucinous carcinoma peritonei (LGMCP), 27% high-grade MCP (HGMCP), 20% HGMCP with signet ring cells (HGMCP-S), and 8% goblet cell adenocarcinoma (GCAC). CC-0 and CC-1 occurred in 57% and 43% of LGMCP, 65% and 35% of HGMCP, 68% and 32% of HGMCP-S, and 79% and 21% of GCAC, respectively. OS and PFS were statistically longer for CC-0 versus CC-1 in HGMCP-S (p = 0.001 and p = 0.005, respectively) and GCAC (p < 0.001 and p < 0.001), but not in LGMCP (p = 0.098 and p = 0.398) or HGMCP (p = 0.167 and p = 0.356).
Conclusions: Survival outcomes for CC-0 and CC-1 after CRS/HIPEC are different for HGMCP-S and GCAC but not for LGMCP and HGMCP. In HGCMP-S and GCAC, only CC-0 should be considered a complete cytoreduction and analyzed separately from CC-1. This distinction is key to understand disease behavior, accurately address patient prognosis, and explore new treatment strategies.
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http://dx.doi.org/10.1245/s10434-020-08844-5 | DOI Listing |
World J Radiol
August 2025
Department of General Practice, Baiyang Community Health Service Center, Hangzhou 310000, Zhejiang Province, China.
Background: Signet ring cell carcinoma originating from the appendix is extremely rare, and the lack of specific clinical symptoms and imaging features makes preoperative diagnosis particularly challenging.
Case Summary: We report a case of a 49-year-old woman who presented with irregular vaginal bleeding lasting more than five months. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed an enlarged appendix with a thickened wall and progressive enhancement after contrast administration.
Ann Med Surg (Lond)
September 2025
Department of Pathology and Laboratory Medicine, Taoyuan Armed Forces General Hospital, Taoyuan City, Taiwan.
Introduction And Clinical Importance: Primary signet ring cell carcinoma (SRCC) of the appendix is an exceedingly rare malignancy with a non-specific clinical manifestations, and it often masquerades as acute appendicitis. Case presentation A 76-year-old man presented with acute abdominal pain and peritonitis. Abdominal computed tomography revealed relative acute appendicitis with peritonitis, and emergency laparoscopic partial cecectomy with appendectomy revealed gangrenous appendicitis with perforation.
View Article and Find Full Text PDFBMJ Open Gastroenterol
August 2025
Institute of Pathology, TUM School of Medicine and Health, Technical University of Munich, Munich, Bavaria, Germany
Objective: Appendiceal adenocarcinoma is a rare cancer with very limited therapeutic options. We aimed to determine whether molecular profiling of advanced appendiceal adenocancer can identify actionable therapeutic alterations.
Methods: We retrospectively analysed cohorts from two large German precision oncology programmes.
Gan To Kagaku Ryoho
August 2025
First Dept. of Surgery, University of Fukui.
Pseudomyxoma peritonei(PMP)is rare disease, but in other countries, cytoreductive surgery(CRS)plus hyperthermic intraperitoneal chemotherapy(HIPEC)is the standard treatment, but in Japan it is not covered by insurance and is only being treated at a few institutions. In 2021, Japanese Society of Peritoneal Malignancy issued Clinical Practice Guideline for Peritoneal Malignancy, in which CRS+HIPEC was weakly recommended, but few new centres have started this treatment as it is not covered by insurance. We have been performing CRS+HIPEC for PMP and peritoneal dissemination of colorectal cancer since 1990, and hope to expand this treatment by reporting on our methods and outcomes.
View Article and Find Full Text PDFGan To Kagaku Ryoho
August 2025
Dept. of Surgery, National Center for Global Health and Medicine.
Pseudomyxoma peritonei(PMP)is a rare clinical entity that typically arises from the rupture of a low-grade appendiceal mucinous neoplasm(LAMN), resulting in accumulation of gelatinous mucinous material throughout the peritoneal cavity. Despite its low-grade histological features, PMP can be clinically aggressive due to continuous mucin production, progressive peritoneal dissemination, and organ compression. Patients often present with abdominal distension, palpable mass, or gastrointestinal symptoms, and the disease may occasionally be discovered during surgery for presumed appendicitis or ovarian tumors.
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