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Long-term outcomes for patients with peritoneal acellular mucinosis secondary to low grade appendiceal mucinous
T Evans1, O Aziz2, B Chakrabarty1
1Colorectal and Peritoneal Oncology Centre, The Christie NHS Trust, Wilmslow Rd, Manchester, M204BX, UK.
Introduction:
Low grade appendiceal mucinous neoplasms (LAMN) are known to metastasise to the peritoneum resulting in pseudomyxoma peritonei (PMP). Literature suggests that the long-term outcome is dependent on the cellular grade of the peritoneal histology, less is known about the risk to patients with acellular mucinosis (AM) alone. This study aims to review long-term outcomes in patients with PMP treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC), whose peritoneal histology was AM secondary to LAMN.
Methods:
Pathological and treatment outcomes were collected from a prospectively maintained database between 2005 and 2019. Data was collected on patients with LAMN and AM diagnosed following CRS/HIPEC. A single institution performed the surgery and pathology reporting, samples reported by three different pathologists.
Results:
Of the 2079 patients with any appendiceal neoplasm referred between 2005 and 2019, 809 underwent CRS/HIPEC, 67 (8%) of those had PMP with purely AM secondary to a LAMN. In the AM group the median age was 59, 37 (55%) were female, follow up was for a median 39 (2-145) months. Inpatient mortality occurred in 1 patient (1.5%), disease specific mortality in 2 (3%), recurrence in 2 (3%) and disease progression in 1 (1.5%).
Conclusion:
This study has identified AM secondary to LAMN as a low risk group for recurrence following CRS/HIPEC compared with epithelial pathology. Given such a low rate of recurrence we would recommend low intensity surveillance post CRS/HIPEC. Agreed standardised pathological assessment is required to exclude cellular material in specimens and diagnose AM.
Insights
Patients with acellular mucinosis (AM) secondary to low-grade appendiceal neoplasms (LAMN) have a low risk of recurrence after Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC). This suggests a need for less intensive surveillance following treatment for this specific condition.
Area of Science:
- Oncology
- Gastroenterology
- Surgical Pathology
Background:
- Low-grade appendiceal mucinous neoplasms (LAMN) can metastasize to the peritoneum, causing pseudomyxoma peritonei (PMP).
- The prognostic significance of acellular mucinosis (AM) alone in PMP, compared to cellular grades, is not well-established.
- Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC) is a standard treatment for PMP.
Purpose of the Study:
- To evaluate the long-term outcomes of patients with PMP and purely acellular mucinosis (AM) secondary to LAMN who underwent CRS/HIPEC.
- To determine the risk of recurrence and disease progression in this specific patient cohort.
Main Methods:
- A retrospective analysis of a prospectively maintained database from 2005-2019.
- Inclusion criteria: patients diagnosed with LAMN and PMP with purely AM following CRS/HIPEC.
- Pathological assessment by three pathologists at a single institution.
Main Results:
- Out of 809 patients who underwent CRS/HIPEC for appendiceal neoplasms, 67 (8%) had PMP with purely AM secondary to LAMN.
- The median follow-up was 39 months.
- Low rates of inpatient mortality (1.5%), disease-specific mortality (3%), recurrence (3%), and disease progression (1.5%) were observed.
Conclusions:
- Acellular mucinosis (AM) secondary to LAMN represents a low-risk group for recurrence after CRS/HIPEC.
- Low-intensity surveillance is recommended post-treatment for patients with AM secondary to LAMN.
- Standardized pathological assessment is crucial to accurately diagnose AM and exclude cellular material.
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