AI Summary & Key Takeaways
AI InsightIn conclusion, EBV-positive inflammatory FDCS is an uncommon splenic tumor with non-specific clinical courses and heterogeneous radiological features.
📌 3 Key Takeaways
Abstract
Follicular dendritic cells act in regulation of humoral immune responses. Epstein-Barr virus (EBV)-positive inflammatory follicular dendritic cell sarcoma (FDCS) is a rare malignancy, accounting for less than 1% of all benign and malignant splenic tumors. A 48-year-old woman presented with pain in the left upper abdomen. Ultrasonography showed a well-circumscribed, hypoechoic solid lesion in the spleen. On computed tomography, a mild swelling with ill-defined borders in the middle part of the spleen was detected. The lesion had increased 18-Fluorodeoxyglucose (18-FDG) uptake on positron emission tomography/computed tomography (PET/CT) scan. A laparoscopic splenectomy was successfully performed. On pathology, 6 cm well-circumscribed nodular lesion was detected in the splenic parenchyma, with positive staining of FDC markers including CD35, CD21, and CD23. Tumor cells were found positive for EBV-encoded RNA in situ hybridization, and was diagnosed as EBV-positive inflammatory FDCS. Splenic EBV-positive inflammatory FDCSs almost always present as single tumor. Nonspecific symptomatology makes imaging methods prominent in the diagnosis. However, definitive diagnosis requires further confirmation through histopathology and immunohistochemistry. Complete surgical resection remains the primary treatment, since the effectiveness of chemoradiotherapy has not been well demonstrated. In conclusion, EBV-positive inflammatory FDCS is an uncommon splenic tumor with non-specific clinical courses and heterogeneous radiological features. Given that complete surgical resection is the only potential curative treatment, a high clinical suspicion is of importance for accurate management.