Laparoscopic Distal Pancreatectomy with Splenectomy for a Multiloculated Primary Splenic Epidermoid Cyst Mimicking a Lymphangioma in an Older Woman: A Case Report
Open Access
- 1 January 2026
- journal article
- Published by Japan Surgical Society in Surgical Case Reports
- Vol. 12 (1) , cr.26-0466
Abstract
INTRODUCTION: Primary splenic epidermoid cysts typically occur in young adults as unilocular lesions and are usually distinguished from lymphangiomas on histopathology, although preoperative imaging differentiation remains challenging. A multiloculated presentation in an older patient is uncommon and can be mistaken for a lymphangioma.CASE PRESENTATION: Herein, we report a 75-year-old asymptomatic woman with a multiloculated splenic cyst that was managed conservatively for approximately 10 years. CT and MRI favored a lymphangioma, and surgical resection was performed because of interval growth. Laparoscopic distal pancreatectomy with splenectomy (DP-S) was required because the cyst was firmly adherent to the pancreas. Histopathological examination revealed a 3.7 × 3.1-cm multiloculated cyst within the splenic capsule, lined by stratified squamous epithelium and associated with foamy histiocytes and cholesterol crystal clefts. Immunohistochemistry demonstrated negativity for CD31, CD34, and D2-40, and positivity for AE1/AE3, cytokeratin (CK) 5/6, and CK7, confirming a primary splenic epidermoid cyst. Negativity for calretinin and Wilms tumor 1 (WT-1) excluded a mesothelial cyst, whereas basal p63 positivity and carbohydrate antigen 19-9 expression supported the multilayered subtype. A postoperative pancreatic fistula was managed conservatively, and the patient was discharged on POD 34.CONCLUSIONS: Integrated clinicopathological assessment, rather than any single marker, is needed to distinguish a multiloculated splenic epidermoid cyst from a lymphangioma; comprehensive immunohistochemistry, including D2-40, calretinin, WT-1, and p63, is a useful adjunct. This case also shows that a benign splenic cyst may require distal pancreatectomy: when dense pancreatic adhesion is encountered intraoperatively, en bloc DP-S can become an unavoidable step toward safe and complete resection, and postoperative pancreatic fistula remains a relevant complication to anticipate.Keywords
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