Abstract
Background
In order for peritoneal metastases from a primary appendiceal mucinous neoplasm to
occur, the wall of the appendix must perforate to allow mucus with tumor cells access
to the peritoneal spaces. With progression the peritoneal metastases show a broad
spectrum of tumor biology varying from indolent to aggressive activity.
Methods
The histopathology of peritoneal tumor masses was determined from the clinical material
resected at the time of cytoreductive surgery (CRS). All groups of patients were treated
by a uniform strategy that involved complete CRS and perioperative intraperitoneal
chemotherapy. Overall survival was determined.
Results
From a database of 685 patients, four histologic subtypes were identified and long-term
survival determined. Four hundred and fifty patients (66.0%) had low-grade appendiceal
mucinous neoplasm (LAMN), 37 patients (5.4%) had mucinous appendiceal adenocarcinoma
of intermediate subtype (MACA-Int), 159 patients (23.2%) had mucinous appendiceal
adenocarcinoma (MACA), and 39 patients (5.4%) had a mucinous appendiceal adenocarcinoma
with positive lymph nodes (MACA-LN). The mean survival of the four groups was 24.5,
14.8, 11.2 and 7.4 years, respectively (p < 0.0001). These four subtypes of mucinous
appendiceal neoplasms were shown to have distinct survival estimates.
Conclusions
The estimated survival of these four histologic subtypes in patients having a complete
CRS plus HIPEC is of value to the oncologist managing these patients. A mutations
and perforations hypothesis was offered in an attempt to explain the broad spectrum
of mucinous appendiceal neoplasms that exist. Inclusion of MACA-Int and MACA-LN as
standalone subtypes was thought to be necessary.
Keywords
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References
- Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis: a clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei.Am J Surg Pathol. 1995; 19: 1390-1408
- Prognostic features of 51 colorectal and 130 appendiceal cancer patients with peritoneal carcinomatosis treated by cytoreductive surgery and intraperitoneal chemotherapy.Ann Surg. 1995; 221: 124-132
- Appendiceal mucinous neoplasms. A clinicopathologic analysis of 107 cases.Am J Surg Pathol. 2003; 27: 1089-1103
- Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review.Am J Surg Pathol. 2006; 30: 551-559
- A consensus for classification and pathologic reporting of pseudomyxoma peritonei and appendiceal neoplasia. The results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process.Am J Surg Pathol. 2016; 40: 14-26
- Pathology and prognosis in pseudomyxoma peritonei: a review of 274 cases.J Clin Pathol. 2012; 65: 919-923
- An update on the diagnosis, grading, and staging of appendiceal mucinous neoplasms.Adv Anat Pathol. 2018; 25: 38-60
- Analysis of morbidity and mortality in 60 patients with peritoneal carcinomatosis treated by cytoreductive surgery and heated intraoperative intraperitoneal chemotherapy.Cancer. 1996; 77: 2622-2629
- Histopathologic analysis in 46 patients with pseudomyxoma peritonei syndrome: failure vs. success with a second-look operation.Mod Pathol. 2001; 14: 164-171
- Cancer chemotherapy for peritoneal metastases. Pharmacology and treatment.in: Sugarbaker P.H. Cytoreductive surgery & perioperative chemotherapy for peritoneal surface malignancy. Textbook and video atlas. second ed. Cine-Med Publishing, Woodbury, CT2017: 47-82
- A simplified approach to hyperthermic intraoperative intraperitoneal chemotherapy (HIIC) using a self retaining retractor.in: Sugarbaker P.H. Peritoneal carcinomatosis: principles of management. Kluwer, Boston1996: 415-421
- Similar survival of all subtypes of mucinous appendiceal adenocarcinoma except the intermediate subtype which shows an improved survival.Ann Surg Oncol. 2022 Dec 21; https://doi.org/10.1245/s10434-022-12864-8
- Genetic alterations during colorectal-tumor development.N Engl J Med. 1988; 319: 525-532
- The consensus coding sequences of human breast and colorectal cancers.Science. 2006; 314: 268-274
- Prevention of colorectal cancer by colonoscopic polypectomy.N Engl J Med. 1993; 329: 1977-1981
- Pathology of colorectal cancer.in: Bleiberg H. Kemeny N. Rougier P. Wilke H. Colorectal cancer: a clinical guide to therapy. Martin Dunitz Publishers, London2002: 55-72
- Intraperitoneal cancer dissemination. Mechanisms of the patterns of spread.Cancer Metastasis Rev. 2003; 22: 465-472
- Clinically aggressive pseudomyxoma peritonei. a variant of a histologically indolent process.J Surg Oncol. 2004; 86: 10-15
- Discordant histology of primary appendiceal adenocarcinoid neoplasms and peritoneal dissemination.Ann Surg Oncol. 2008; 15: 1440-1446
- Pathology and prognosis in pseudomyxoma peritonei: a review of 274 cases.J Clin Pathol. 2012; 65: 919-923
- Analyzing the patterns of treatment failure.J Clin Oncol. 1986; 6: 825-826
Article info
Publication history
Published online: February 26, 2023
Accepted:
February 23,
2023
Received in revised form:
January 18,
2023
Received:
October 26,
2022
Publication stage
In Press Journal Pre-ProofIdentification
Copyright
© 2023 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.
