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Pseudomyxoma peritonei: cytomorphologic findings and clinicopathologic correlates
Irem Onur1, Momin T Siddiqui2, Paul E Wakely3
1Division of Cytopathology, Department of Pathology, The Johns Hopkins Medical Institutions, Rm PATH 406, 600 N. Wolfe Street, Baltimore, Maryland 21287.
Journal of the American Society of Cytopathology
|May 3, 2019
Summary
Pseudomyxoma peritonei (PP) is rare in cytopathology. Diagnosis relies on identifying extracellular mucin and neoplastic cells in fluid specimens, with appendix as the most common primary site.
Area of Science:
- Cytopathology
- Gastroenterology
- Oncology
Background:
- Pseudomyxoma peritonei (PP) is a rare, often fatal condition characterized by gelatinous ascites due to mucin accumulation.
- Neoplastic glandular epithelium is the source of mucin in PP.
Purpose of the Study:
- To analyze the cytomorphology of pseudomyxoma peritonei (PP).
- To correlate cytomorphologic findings with clinical data.
- To evaluate diagnostic accuracy in fluid specimens.
Main Methods:
- Retrospective review of 41 cases of PP from 32 patients across three medical institutions.
- Analysis of available abdominal fluid cytologic specimens (15 cases).
- Histopathologic correlation of cytopathologic diagnoses.
Main Results:
- The appendix was the primary tumor site in 78.1% of patients.
- Extracellular mucin was present in all reviewed cytologic specimens (100%).
- Neoplastic epithelial cells and muciphages were observed in a majority of cases, with 93.8% diagnostic accuracy.
Conclusions:
- Pseudomyxoma peritonei (PP) is infrequently seen in cytopathology.
- Key diagnostic features include abundant extracellular mucin and distinguishing neoplastic cells from reactive cells.
- Accurate diagnosis requires careful evaluation of fluid cytology, especially in cases with scant mucin.
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