抄録
A 16-month-old boy was admitted with right exophthalmos and disturbance of ocular movement. CT scan disclosed a tumor occupying the supra and para-sellar regions. The tumor was partially removed. Whole brain irradiation was given at doses of 4, 600 rads. One month later, the right cervical lymph nodes became swollen. CSF cytology and bone marrow smear showed tumor cells. The patient expired 6 months after the onset of the initial symptoms. Urinary catecholamine, homovanillic acid, vanylmandelic acid, and metanephrine were within normal limits after surgery and thereafter. Autopsy excluded neuroblastoma of the adrenal glands and of the sympathetic chains. Microscopically, the primary tumor of the sellar region showed fibrous tissue intermingled with a small amount of coagulative tumor cell necrosis. Meningeal tumor cell dissemination of the spinal cord and metastases to cervical lymph nodes were observed. Microscopic tumor deposits were also found in the costal and vertebral bone marrow. Histological findings of the surgical specimen and of the metastatic tumor at autopsy were almost the same. The tumor cells had oval or slightly fusiform, hyperchromatic nuclei and small, scanty or illdefined cytoplasm. Several incomplete Homer Wright rosettes were seen. Electron microscopy disclosed a few dense core vesicles of 80-200 nm in diameter in the tumor cell processes.
