Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes are often the presenting features of the POEMS syndrome. Approximately 50% of these are associated with osteosclerotic myeloma, a rare variant of multiple myeloma and some with Castleman's disease, an unusual lympho-proliferative disorder. The underlying pathogenetic mechanisms have not been elucidated but amelioration of symptoms with the disappearance of the M-component suggests possible etiopathogenic mechanisms. We present a 40-year-old woman with manifestations of all three disorders.
Currently, of less than 50 cases of head and neck follicular dendritic cell (FDC) sarcoma reported in the literature, 5 have been found to occur in the background of Castleman disease. We report another case of head and neck FDC sarcoma with emphasise on its associated lesions and review the outcome of treatment from the existing cases in the literature.