Follicular dendritic cell sarcoma of the neck with pulmonary metastases

We present a case of follicular dendritic cell sarcoma in a 48 years old Saudi female who reported with slowly progressive right sided extranodal neck mass associated with pulmonary metastasis. Clinical examination, histopathologic features including distinct immunostains combine together to make the rare diagnosis of follicular dendritic cell sarcoma. This entity is often misdiagnosed due to non-consideration in differential diagnosis of sarcoma. It carries a significant potential for regional as well as distant spread and hence categorized as intermediate risk malignancy. Clinical, histopathological and immunohistochemical aspects and therapeutic options of this unusual case are discussed.