Pancreatic follicular dendritic cell sarcoma: one case report and literature review

Abstract

Pancreatic follicular dendritic cell sarcoma (FDCS) is a rare neoplasm with unclear pathological characteristics. In this study, we report one case of pancreatic FDCS and review published cases to summarize the characteristics and treatment of pancreatic FDCS. A man in his early 30 s was admitted for jaundice, abdominal fullness, and weight loss for 15 days. Computed tomography revealed a large capsule solid mass in the pancreatic head together with a dilated bile duct and enlarged retroperitoneal lymph nodes. Serum biochemistry revealed high total bilirubin levels (313.9 μmol/L) and normal tumor marker levels. Pancreatoduodenectomy was performed, but no chemotherapy was administrated at the patient's behest. The pathologic diagnosis was pancreatic FDCS infiltrating the duodenal seromuscular layer and common bile duct. The patient presented with liver metastasis 3 months after surgery and died 8 months after surgery from multiorgan failure. Pancreatic FDCS is a rare disease with high invasiveness. Our previous case exhibited paraneoplastic syndrome together with this disease, and further investigation is needed to confirm whether paraneoplastic syndrome is a typical syndrome of pancreatic FDCS.

Keywords: Follicular dendritic cell sarcoma; bile duct; diagnosis; pancreas; pancreatoduodenectomy; paraneoplastic syndrome; treatment.

Figure 1.

Computed tomography images. (a) Before surgery and (b) After surgery.

Figure 2.

Gross examination of the tumor. (a) The tumor was a well-encapsulated, soft, multilobulated mass. (b) The freshly cut section of this mass was yellow-gray in color, and the tumor featured a fish flesh-like appearance with typical hemorrhagic and necrotic areas and (c) The dissected mass was fixed in paraformaldehyde.

Figure 3.

Hematoxylin–eosin staining. (a) The nodules of the tumor were separated by fibrovascular septae, and the ovoid-to-spindle–shaped cells were typically arranged in short fascicular, whorl-like, or storiform patterns within the nodules (×200) and (b) The tumor cell borders were visible, the cytoplasm was eosinophilic, and the nuclei were ovate or long spindle-like in shape with remarkable nuclear divisive phenomena (×400).

Figure 4.

Immunohistochemical staining (×400). (a) CD21 staining. (b) CD35 staining. (c) Clusterin staining and (d) CD30 staining.