Clear cell rhabdomyosarcoma of the nasal cavity and paranasal sinuses

Abstract

This report describes two cases of alveolar rhabdomyosarcoma of the nasal cavity with unusual histological appearances mimicking clear cell carcinoma. The closely packed tumour cells were polygonal and arranged in sheets and packets. They had an appreciable amount of clear cytoplasm due to accumulation of glycogen. The diagnosis of rhabdomyosarcoma was confirmed by positive staining for desmin and myoglobin. Rhabdomyosarcoma should be included in the differential diagnosis of nasal clear cell tumours, particularly in young adults. A correct diagnosis is important, because chemotherapy is indicated even for apparently localized disease.

Clear Cell (Glycogen-Rich) Rhabdomyosarcoma Presenting as Cervical Lymphadenopathy

“…Cytoplasmic staining for desmin, muscle-specific actin and sarcomeric actin together with the presence of thick and thin cytoplasmic filaments provides evidence of myogenic differentiation. Unlike some of the earlier cases described, we did not find any cross-striations on light microscopy [1,5].…”

“…They are classified into embryonal, botryoid, alveolar and pleomorphic types. Other rare variants include spindle cell, mixed and clear cell rhabdomyosarcomas [1][2][3][4][5]7]. They may arise at various sites; however, the three principal sites are the head and neck, genitourinary tract and The resulting clear cell morphology leads to problems in diagnosis.…”

“…Four cases of glycogen-rich clear cell rhabdomyosarcomas [1,3,5] and 4 cases of lipid-rich clear cell rhabdomyosarcomas [2,4] have been reported in the English language literature. Chan et al [1] reported 2 cases of clear cell rhabdomyosarcoma of the nasal cavity and paranasal sinuses occurring in a 27-year-old female and 31-year-old male.…”

“…Chan et al [1] reported 2 cases of clear cell rhabdomyosarcoma of the nasal cavity and paranasal sinuses occurring in a 27-year-old female and 31-year-old male. Bégin et al [3] added a third case of clear cell rhabdomyosarcoma of the anterior mediastinum occurring in a 63-year-old man seropositive for human T-cell lymphoma virus type I.…”

“…Clear cell rhabdomyosarcoma is a rare neoplasm posing diagnostic problems. There may be either glycogen-rich or lipid-rich variants [1][2][3][4][5]. We describe a glycogen-rich clear cell rhabdomyosarcoma, which presented with cervical lymphadenopathy in a 15-year-old patient, and review the literature.…”