Objective To explore the clinicopathological features, treatments and outcomes of follicular dendritic cell sarcoma （FDCS） in abdomen. Methods One rare case of pancreatic FDCS was reported in the Second Affiliated Hospital of Shaanxi University of Chinese Medicineand the relevant case reports in both domestic and foreign literatures were reviewed. Results A 66-year-old female patient with pancreatic FDCS was hospitalized for physical examination detecting a space-occupying lesion at tail of pancreatic body. Computed tomography （CT） indicated that tumor with a size of around 9 cm had distinct boundaries with a compression of splenic vein. Tumor marker CA125 was elevated. During pancreatic-caudal splenectomy, tumors presented spindles with distinct nucleolus and obvious mitosis. With the findings of CD21, CD35, fascin （+）, EBER in situ hybridization （-） and Ki-67 around 20%, pathological diagnosis was pancreatic FDCS. No other adjuvant treatment was offered. No recurrence or metastasis occurred during a follow-up period of 6 months. And 49 cases of abdominal FDCS were retrieved from a literature review. For a total of 50 cases, the average age was 50（19-77） year and most of them had a non-specific medical history. Liver and retroperitoneal cavity are dominant and spleen, large intestine, pancreas, small intestine, ileocecal part and appendix are also common sites. No significant inter-gender difference existed. Generally patients may have no obvious symptoms or varying severity of abdominal symptoms. Some dermal and pulmonary symptoms are extra-abdominal. Preoperative laboratory tests revealed no obvious abnormalities and tumor marker CA125 was elevated in 10% of patients. Ultrasonography and CT are two major auxiliary examinations. Colonic polyps is often detected under endoscope. And 22% of patients had metastases at an initial visit. Almost all macroscopic slides showed single solid tumors with distinct boundaries and maximal diameter of tumor was （1-30） cm. A definite diagnosis is often dependent upon a combination of several specific markers such as CD21, CD35, Fascin, CD23 and fascin plus histology. Most EBER in situ hybridizations was positive. And 96% of them were operated. Most of them survived tumor-free post-operation and few had recurrence and metastasis. Adjuvant chemotherapy or radiotherapy was rare. And efficacy should be further confirmed. Conclusion As a kind of rare moderate malignant tumor, abdominal FDCS carries a high risk of local recurrence and metastasis after excision and lacks a specificity of clinical pathogenesis. Its pathogenesis may be correlated with Epstein-Barr virus infection and immune system diseases. Whether or not postoperative chemoradiotherapy improves its prognosis remains uncertain. However, if high risk factors are present, long-term follow-ups, regular reviews and aggressive interventions are recommended.
Key words
Follicular dendritic cell sarcoma /
Clinicopathological analysis /
Literature review
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