[Leiomyosarcoma metastatizing to the sphenoid bone presenting exophthalmos: a case report]

A rare subcutaneous leiomyosarcoma metastatizing to the sphenoid bone and presenting exophthalmos is reported. A 56-year-old female presented with protrusion of the right eye and a slowly growing lump on the right temporal region. Six years previously, she had undergone removal of a subcutaneous mass in the back, which was histologically diagnosed in another hospital as leiomyosarcoma. She had undergone four other operations, including removal of local recurrences and a right renal metastasis. On admission, physical examination showed no neurological deficits. Craniogram revealed an osteolytic lesion without marginal sclerosis in the right sphenoid bone. CT showed an inhomogeneously enhanced mass with irregular expansion of the diploic space, which was partly invading the right orbit. MRI demonstrated an extradural mass in the right sphenoid region, which was slightly low-intense in T1-weighted image, high-intense in T2-weighted image, and inhomogeneously enhanced by Gd-DTPA. Right external carotid angiogram showed a highly vascular stain fed by meningeal arteries. Radionuclide bone scintigram showed multiple high-uptake areas in the left femoral head, the ribs, and the sphenoid bone. Preoperative embolization of the tumor vessels fed by the external carotid artery was performed. Following this procedure, the tumor stain disappeared completely. The tumor was totally excised with minimal bleeding through an orbitozygomatic approach. The tumor was loosely adherent to the dura and periorbit. The bone defect was covered with a methylmethacrylate resin plate. the histological examination demonstrated fascicular arrangement of the spindle shaped cells with mitotic figures. Immunohistochemical studies showed that most tumor cells were positive for actin and myosin, but negative for desmin.(ABSTRACT TRUNCATED AT 250 WORDS)