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Background: Appendiceal tumors comprise a heterogeneous group of tumors that frequently disseminate to the peritoneum. Management of appendiceal tumors is lacking high quality data given their rarity and heterogeneity. In general, appendiceal tumor treatment is extrapolated in part from colorectal cancer or pooled studies, without definitive evidence of disease-specific benefit. Many practices are controversial and vary widely between institutions. A national consensus update of best management practices for appendiceal malignancies was performed to better standardize care. Herein the authors present recommendations for the management of appendiceal tumors with peritoneal involvement.
Methods: As previously described, modified Delphi consensus was performed to update the previous 2018 Chicago consensus guideline. Recommendations were supported by using rapid systematic reviews of key issues in surgical and systemic therapy. Key pathology concepts and recommendations were synthesized in collaboration with content experts.
Results: A consensus-based pathway was generated for any type of non-neuroendocrine appendiceal tumor with peritoneal involvement. The first round of Delphi consensus included 138 participants, of whom 133 (96%) participated in the second round, and greater than 90% consensus was achieved for all pathway blocks. Key items include recommending evaluation for cytoreduction to most patients with low-grade peritoneal disease who are surgical candidates, and to many patients with high-grade disease, as well as timing of systemic chemotherapy and surveillance protocols. Common pitfalls in pathologic classification and their clinical implications are also presented.
Conclusion: These consensus recommendations provide guidance regarding the management of appendiceal tumors with peritoneal involvement, including a review of current evidence in the management of recurrent and unresectable disease.
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http://dx.doi.org/10.1245/s10434-025-17364-z | DOI Listing |
BJS Open
September 2025
Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden.
Background: Appendiceal adenocarcinomas and low-grade appendiceal mucinous neoplasms (LAMNs) are rare tumours. Much of the existing knowledge is derived from registry-based studies, particularly the Surveillance, Epidemiology, and End Results database in the USA.
Methods: This retrospective cohort study used data from the Swedish Cancer Registry, Swedish Cause of Death Registry, and the National Patient Registry to analyse demographic characteristics and outcomes of patients diagnosed with appendiceal adenocarcinoma or LAMN between 2005 and 2019.
Am J Surg
August 2025
Division of Surgical Oncology and Endocrine Surgery, Mayo Clinic, Arizona, 5777 E. Mayo Blvd. Phoenix, AZ, 85054, USA. Electronic address:
Background: Signet Ring Cell Adenocarcinoma (SRCA) of the appendix is a rare tumor with a poor prognosis and limited information to help guide treatment.
Methods: We reviewed patients diagnosed with SRCA between 1998 and 2024 at all Mayo Clinic sites.
Results: Among 84 patients, the most common presentation was non-specific abdominal pain (31 %).
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.
World J Clin Oncol
August 2025
Division of Gastroenterologic Surgery, Department of Surgery, Tohoku Medical and Pharmaceutical University, Sendai 983-8536, Miyagi, Japan.
Appendiceal mucinous neoplasms (AMNs) are rare tumors originating from mucin-producing epithelial cells of the appendix. They can exhibit both benign and malignant behavior. They are often incidentally discovered during appendectomy.
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August 2025
Paediatrics, All India Institute of Medical Sciences, Mangalagiri, Mangalagiri, IND.
Pseudomyxoma peritonei (PMP) is a rare clinical entity characterized by the accumulation of mucinous ascites and peritoneal implants, most commonly originating from appendiceal or ovarian neoplasms. Its diagnosis is often delayed due to vague and nonspecific symptoms. We report the case of a 75-year-old female who presented with diffuse abdominal pain, melena, and significant weight loss.
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