Clear cell (glycogen-rich) rhabdomyosarcoma presenting as cervical lymphadenopathy

A 15-year-old patient is described who presented with cervical lymphadenopathy. A lymph node biopsy confirmed a metastatic neoplasm composed of islands and aggregates of round to polygonal-shaped cells with surrounding hyalinised fibrous stroma. The tumour cells contained a clear glycogen-rich cytoplasm and stained for desmin, muscle-specific actin and sarcomeric actin. Electron microscopy revealed paranuclear thick and thin filaments. These findings confirm striated muscle differentiation and a diagnosis of glycogen-rich clear cell rhabdomyosarcoma. The tumour recurred in a cervical lymph node after 2 years. This is yet another tumour that must be considered in the differential diagnosis of clear cell tumours of the head and neck region.