Intrasellar rhabdomyosarcoma: case report.

OBJECTIVE AND IMPORTANCE: There has been only one reported case of an intrasellar rhabdomyosarcoma, the origin of which was in the para-nasal sinus. The authors encountered a patient with an intrasellar rhabdomyosarcoma with no evidence of tumor at any additional sites. CLINICAL PRESENTATION: A 28-year-old otherwise healthy man complaining of headache exhibited left abducent nerve palsy and left visual disturbance. The patient was diagnosed as having a sellar tumor invading the left cavernous sinus. INTERVENTION: Near total removal of the tumor was achieved via a trans-sphenoidal approach. Histologically, the tumor was composed of small, round-to-elongated undifferentiated cells and large spindle cells with myoblastic features. Immunohistochemically, tumor cells were positive for antibodies against desmin, myoglobin, and alpha-smooth muscle actin. The tumor was identified as an embryonal rhabdomyosarcoma on the basis of the above pathological findings. Systemic investigation, including the nasal and para-nasal regions, failed to detect any additional tumors. Postoperative local radiation therapy and chemotherapy with the use of ifosfamide, etoposide, and vincristine brought about complete initial remission. CONCLUSION: Rhabdomyosarcoma should be considered in the differential diagnosis of a primary intrasellar neoplasm.