Primary Serous Cystadenocarcinoma of the Spleen

Abstract

Tumours of the spleen are uncommon, and most are metastases from primaries in other organs. Primary splenic malignancies are subdivided into two main groups: lymphoid and non-lymphoid. Primary splenic cystadenocarcinomas are extremely rare, and only reports of the mucinous variant exist. We present the case of a female in her eighth decade of life who was found to have an incidental complex splenic mass with a cystic component, which showed an interval increase in size on serial imaging. After further investigation, including positron emission tomography (PET), endoscopic ultrasound (EUS), and laparoscopy, she successfully underwent distal pancreatectomy, splenectomy, and partial gastrectomy for a suspected locally invasive pancreatic malignancy. Histology and immunohistochemical analyses were consistent with the first recorded case of primary serous cystadenocarcinoma of the spleen in the literature.

Keywords: cancer; hepatopancreatobiliary; serous cystadenocarcinoma; spleen; surgery.

Figure 1. Axial (a), sagittal (b), and coronal (c) planes of CT showing a complex mass with a cystic component arising from the splenic hilum.

Figure 2. Cross-section of the resected specimen. The tumour appears confined to the spleen with both solid and cystic components and a relatively smooth interface with the splenic parenchyma.

Figure 3. Solid-cystic tumour with papillary architecture and focal hierarchical branching. Hematoxylin and eosin (H&E) staining, original magnification ×12.

Figure 4. The neoplastic cells stained negative for CDX2 (a) but positive for WT-1 (b), ER (c), and PAX-8 (d). Immunohistochemistry, original magnification ×50.