Treatment of unresectable malignant rhabdoid tumor of the orbit with tandem high-dose chemotherapy and gamma-knife radiosurgery.

Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor that primarily occurs in very young children. We report here a patient with a primary MRT of the orbit who received tandem high-dose chemotherapy and gamma-knife radiosurgery. Although the tumor was not completely removed, and the initial chemotherapy failed, the patient achieved long-term survival after this modality of treatment. This approach may be one to be further considered in patients with MRT.

Primary granulocytic sarcoma in the sphenoidal bone and orbit.

CASE REPORT: We report a case of a primary cranial chloroma in boy aged 2 years and 8 months. The symptoms were progressive bilateral exophthalmos, right abducens palsy, and bilateral papilledema. The tumor was partially calcified and was a round mass located in the bilateral sphenoidal bone extending into the orbit. Laboratory study did not show hematological abnormality. The tumor was partially removed by bilateral frontotemporal craniotomy and a diagnosis of primary granulocytic sarcoma was made from the surgical specimen. Progressive deterioration of visual acuity was seen and chemotherapy started on the 11th postoperative day followed by local cranium irradiation (24 Gy). The patient has been in complete remission for 37 months. The visual acuity recovered partially and follow-up magnetic resonance imaging showed a significant decrease in the size of the tumor. DISCUSSION: Radiological diagnosis of primary intracranial granulocytic sarcoma is difficult. Surgical removal may be an option for progressive neurological deterioration but chemotherapy is more important for both neurological stabilization and induction of remission.

Complete penetration of the optic chiasm by an unruptured aneurysm of the ophthalmic segment: case report.

BACKGROUND: It is well known that aneurysms of the ophthalmic segment sometimes elevate the optic nerve or chiasm, and in case of large or giant aneurysms, the optic apparatus can be dramatically thinned. Nonetheless, they rarely penetrate the optic pathway completely. To our knowledge, no previous reports have dealt with the complete penetration of the optic chiasm by unruptured aneurysms of the ophthalmic segment. CASE DESCRIPTION: A 70-year-old woman presented with visual dysfunction in her left eye that she had experienced for several months. Her left visual acuity had rapidly deteriorated to the level of finger counting and visual field testing demonstrated nasal hemianopsia in the left eye and upper temporal quadrant hemianopsia in the right eye. Left internal carotid angiograms and three-dimensional digital subtraction angiograms showed an aneurysm of the ophthalmic segment projecting superomedially. Intraoperative findings revealed complete penetration of the optic chiasm by the fundus of the aneurysm. The optic pathway adjacent to the dome had become remarkably thin and dark yellow. After clipping was completed, the fundus of the aneurysm was punctured to decompress the optic chiasm. Postoperatively, patient's visual acuity in the left eye gradually recovered, but the visual field deficit persisted after the operation. CONCLUSION: This rare case demonstrates the potentially aggressive behavior of unruptured aneurysms of the ophthalmic segment. Patients with unruptured aneurysms of the ophthalmic segment who present with visual symptoms should be treated with surgical clipping to decompress the optic pathway as soon as possible.