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High-Grade Appendiceal Mucinous Neoplasm: Clinicopathologic Findings in 35 Cases
Raul S Gonzalez1, Norman J Carr2, Haihui Liao3
1From the Department of Pathology, Beth Israel Deaconess Medical Center, Boston, Massachusetts (Gonzalez).
Archives of Pathology & Laboratory Medicine
|April 26, 2022
Summary
High-grade appendiceal mucinous neoplasm (HAMN) is a rare appendix tumor. HAMNs can spread to the peritoneum, potentially causing grade 2 pseudomyxoma peritonei with a worse prognosis.
Area of Science:
- Gastroenterology
- Surgical Pathology
- Oncology
Background:
- High-grade appendiceal mucinous neoplasm (HAMN) is a rare appendix neoplasm characterized by pushing invasion and high-grade cytologic atypia.
- HAMN remains understudied, necessitating further research into its clinicopathologic features.
Purpose of the Study:
- To elucidate the clinicopathologic characteristics of high-grade appendiceal mucinous neoplasms (HAMNs).
Main Methods:
- A multi-institutional retrospective study identified 35 HAMNs.
- Clinicopathologic and molecular features were analyzed.
- Histologic review included architectural patterns and serosal invasion.
Main Results:
- Average patient age was 57, with abdominal/pelvic pain as the common presentation.
- 57% of tumors exhibited high-grade features; 22 cases perforated the serosa.
- Peritoneal spread included acellular mucin or grade 2 pseudomyxoma peritonei.
- KRAS and TP53 mutations were observed in molecular analyses.
- No appendix-confined tumors recurred; 2 patients developed pseudomyxoma on follow-up.
Conclusions:
- HAMNs share presentation and prognosis with low-grade appendiceal mucinous neoplasms.
- Peritoneal spread typically results in grade 2 pseudomyxoma peritonei, associated with a poorer prognosis than grade 1.
- Pseudomyxoma peritonei in HAMN cases can carry a significant mortality rate.
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