Primary hepatic follicular dendritic cell sarcoma: A case report

Abstract

Background: Follicular dendritic cell sarcoma (FDCS) is an uncommon type of tumor with low incidence. To date, no standard treatment for the disease has been established. Surgery remains the main treatment. Adjuvant chemotherapy and radiotherapy are optional approaches. Metastatic cases require multidisciplinary collaborative treatments. However, the choice of chemotherapeutic drugs is controversial.

Case summary: A 66-year-old Chinese woman presented to our hospital complaining of intermittent pain of right upper quadrant. An enhanced computed tomography (CT) scan of the abdomen revealed hepatocellular carcinoma. Subsequently, the patient underwent a radical partial hepatectomy. Primary FDCS of the liver was diagnosed pathologically. Except for regular follow-up examinations, the patient did not receive adjuvant chemotherapy or radiotherapy. However, fluorine-18-fluorodeoxyglucose positron emission tomography/CT (PET/CT) confirmed lymph node metastases in the space of ligamentum hepatogastricum and pancreatic head, as well as the portacaval space. The patient was given systemic chemotherapy with gemcitabine and docetaxel for she was unsuitable for surgery. Satisfactorily, the metastatic lymph nodes were significantly reduced to clinical complete remission after eight cycles of chemotherapy. Then, strengthened radiotherapy was followed when the patient rejected the opportunity of surgery. Eventually, the carcinoma got better control and the patient was free of progression.

Conclusion: This case highlights the importance of making suitable chemotherapy regimens for the rare tumor. The combination of gemcitabine, docetaxel, and consolidated radiotherapy may offer a new promising option for the treatment of metastatic hepatic FDCS in the future.

Keywords: Case report; Chemotherapy; Docetaxel; Follicular dendritic cell sarcoma; Gemcitabine; Liver; Radiotherapy.

Figure 1

Computed tomography or fluorine-18-Fluorodeoxyglucose positron emission tomography/computed tomography images of metastatic lymph nodes. A: Baseline image at progression after surgery; B: After eight cycles of chemotherapy with gemcitabine and docetaxel; C: Computed tomography image one month after radiotherapy. MLN.1: Metastatic lymph nodes in the hepatogastric ligament; MLN.2: Metastatic lymph nodes in the portacaval space in the abdomen are showed in chronological order (arrow).

Figure 2

Hematoxylin-eosin staining for primary tumor (magnification, ×400). The tumor was composed of spindle cells, abundant small lymphocytes, and mature plasmocytes.

Figure 3

Immunohistochemical staining for cluster of differentiation 21 (CD21) and cluster of differentiation 23 (CD23) is positive (magnification, ×200).