Abstract
Dendritic follicular sarcoma (DFS) is a very rare and often poorly known neoplasia. It develops mainly in peripheral lymph nodes at the expense of dendritic follicular cells. However, extra nodal locations were also found in the literature. Diagnosis is often difficult, based mainly on histopathological and immunohistochemical data. There is currently no consensual therapeutic strategy proposed by the National Understanding Cancer Network. The aim of this work is to focus on the various epidemiological, histopathological, immunohistochemical and therapeutic aspects of the follicular dendritic sarcoma. We report the case of a cervical dendritic follicular sarcoma with literature review. This is a 51-year-old patient with no particular pathological history, consulting for a right lateral cervical mass evolving for 5 years without dyspnea, dysphagia, dysphonia, nor other associated signs evolving in a context of apyrexia and general state conservation. The clinical examination had objectified a firm, fixed, painless right upper jugulocarotid mass without inflammatory signs in sight. The cervical MRI had objectified two contigüe lateral cervical tissue masses well encapsulated, well limited necrotic with mass effect on the larynx without vascular enclosing. The patient had benefited from tumor reduction. Histology was in favor of a dendritic follicular sarcoma. Cervical CT was performed 2 months postoperatively which had shown a mixed right lateral cervical mass with thickened wall and hypodense center pushing in and back the primary carotid and forward the internal jugular vein without any sign of invasion locoregional. The patient then benefited from a complete removal of the tumor mass with ligation of the internal jugular vein. The anatomopathological study had objectified a tumor proliferation made of fusiform cells with nuclei augmented of irregular volume, nucleoli presenting moderate atypies without peri nervous engainement or neoplastic vascular emboli. Immunohistochemistry had shown positive antibodies anti-CD23, anti-CD35, anti-vimentin and anti-PS100. The Ki-67 index was positive on 15% of the cells. This profile concluded in a dendritic follicular sarcoma. The post-operative follow-up was simple. The patient was then referred to oncology for complementary radiation therapy. At 6 months no sign of recurrence was noted. Dendritic follicular sarcoma (DFS) is a relatively rare entity derived from dendritic follicular cells, which are B-cell antigen-presenting cells in the germinal centres of lymphoid follicles. It mainly affects the peripheral lymph nodes especially those of the head and neck. However, extra nodal tissues are affected in about 30% of cases especially the pharynx, oral cavity, thyroid gland. The diagnosis of SFD is difficult and often poorly understood, due to its silent symptomatology, extremely varied morphology and aspecific imaging. The main differential diagnoses are inflammatory myofibroblastic tumors, pseudotumoral tumors, interdigitant dendritic cell sarcomas, lymphomas and undifferentiated carcinomas. Diagnosis is mainly based on pathological examination with immunohistochemical complement.