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Mucinous epithelial cysts of the spleen associated with pseudomyxoma peritonei
D G Du Plessis1, J A Louw, P A Wranz
1Department of Anatomical Pathology, University of Stellenbosch and Tygerberg Hospital, Tygerberg, South Africa. plessi-d@wcnn.co.uk
Aims:
We report two rare cases of neoplastic pseudomyxoma peritonei associated with splenic mucinous epithelial cysts and review previously reported cases of splenic mucinous lesions in order to investigate the extent and implications of such an association.
Methods And Results:
The majority of mucinous lesions of the spleen appear to be associated with pseudomyxoma peritonei. The clinicopathological profile of these cases conforms to that of neoplastic pseudomyxoma peritonei, showing a similar age of onset, outcome and histological features. Most of the cases were associated with a confirmed or suspected appendiceal primary. The immunophenotype (cytokeratin 7 negative; cytokeratin 20 and CEA positive) of the lesions of both our cases, including those in the ovary, was suggestive of a gastrointestinal origin.
Conclusions:
Splenomegaly due to cystic intrasplenic mucinous epithelial lesions may occasionally be the presenting feature of pseudomyxoma peritonei or herald tumour recurrence. Mucinous epithelial cysts of the spleen may also precede the development of pseudomyxoma peritonei. All cases of pseudomyxoma peritonei should be investigated for splenic involvement and, conversely, a primary mucinous neoplasm sought elsewhere in the abdomen in all cases of splenic mucinous cysts.
Insights
Splenic mucinous cysts can be an early sign of pseudomyxoma peritonei. Investigating spleen involvement in pseudomyxoma peritonei and abdominal mucinous neoplasms is crucial for diagnosis.
Area of Science:
- Gastroenterology
- Oncology
- Pathology
Background:
- Neoplastic pseudomyxoma peritonei (PMP) is a rare malignancy characterized by mucinous ascites.
- Splenic mucinous epithelial cysts are uncommon lesions with an unclear association with PMP.
Observation:
- Two rare cases of PMP associated with splenic mucinous epithelial cysts are presented.
- A review of existing literature on splenic mucinous lesions and PMP was conducted.
Findings:
- The majority of splenic mucinous lesions are associated with PMP, sharing similar clinicopathological features.
- Most cases had an appendiceal primary, and immunophenotype suggested a gastrointestinal origin.
- Splenic lesions can be the initial presentation or indicate recurrence of PMP.
Implications:
- Splenomegaly from cystic intrasplenic mucinous lesions may herald PMP or recurrence.
- Splenic involvement must be investigated in all PMP cases.
- Abdominal mucinous neoplasms should be sought in splenic mucinous cyst cases.