[Intracranial fibrous xanthoma (xanthofibroma) in an infant: a case report].

A case of intracranial fibrous xanthoma (xanthofibroma) is reported. Intracranial fibrous xanthoma in infancy under the age of 1 year is extremely rare. This patient was a 8-month-old boy with a history of convulsive seizure. He had a previously known chest wall tumor which was diagnosed as fibrous xanthoma of the skin. Plain CT scan revealed a well defined high density area in the left temporal lobe. The area was well enhanced with contrast media. At operation, it was found that the tumor did not attach to dura mater and was almost well demarcated. Total removal of the tumor was performed. The patient has been doing well for these 6 months following craniotomy, with no sings of recurrence and no neurological deficits. Histologically, the tumor was composed of fibroblastic cells and foamy phagocytic cells in storiform pattern. Some multinucleated giant cells were found. Immunohistochemistry technique revealed that the tumor cells were negative for GFAP, positive for Vimentin, positive for S-100 protein and negative for EMA. Our studies support the diagnosis of intracranial fibrous xanthoma coexistent with the same tumor found in the subcutaneous space of the chest wall of a boy under 1 year of age. We regard it as a rare incidence. Differential diagnosis and the characteristics of fibrous xanthoma were discussed.

[A case of pleomorphic xanthoastrocytoma].

A case of pleomorphic xanthoastrocytoma (Kepes) is reported. This patient was a 12-year-old boy with a history of convulsive seizure. Neurological examination on admission showed no abnormality. Plain CT scan revealed a well defined low density area with calcification in the right frontal lobe. A part of peripheral portion of low density area were well enhanced with contrast media. At operation, there was a cyst containing xanthochromic fluid in the right frontal lobe. A part of cyst well near the cerebral surface was reddish hard. Total removal of nodular tumor and subtotal removal of the cyst wall were performed. He has been doing well for these 3 years following craniotomy and has no deficit without CT evidence of recurrent tumor. Histologically the tumor cells displayed marked pleomorohism. However either necrosis or mitosis were not seen. Frequently these cells had vacuolated or foamy cytoplasm. There were many of the giant cells and multinucleated cells. In some area, these tumor cells were surrounded by a fine network of reticulin fibers. Electron microscopically the tumor cells were occasionally filled with glial filament and lipid granules were seen. Immunoperoxidase technique revealed GFAP in the cytoplasm of the tumor cells. This case was considered to be pleomorphic xanthoastrocytoma first proposed by Kepes.

[A case of cervical intramedullary neurenteric cyst].

A case of cervical intramedullary neurenteric cyst was reported. A 12-year-old girl was admitted with severe pain over the nape and shoulders, and weakness of all extremities. At the age of 4 years, she had suffered from the nape pain and paraparesis which, however, cleared later spontaneously. Neurological examination revealed evidences of presumptive cervical intramedullary lesion, and myelography showed a complete block at the third cervical level accordingly. Surgical exploration through C3-C5 laminectomy disclosed an intramedullary cyst situated within the right half of the cord. The cyst was removed except for its upper and lower apices. Excellent clinical results followed the operation. The cyst was composed of collagen fibers with an inner epithelial lining, which consisted of single or pseudostratified layer of columnar, cuboidal or squamous cells. Cells were ciliated at some parts. The base of the epithelial cells rested upon the basement membrane. Nuclei were positioned near the base of the cells, to present a row. The cytoplasm in the majority of cells contained abundant mucin positive to PAS staining. Pathological diagnosis of neurenteric cyst was made on the basis of these histological findings. Usually intraspinal neurenteric cyst is located in the subarachnoid space and ventrally to the spinal cord. Neurenteric cyst appears histologically similar to ependymal cyst, though, in the latter the epithelial cells seldom contain mucin, and only in scanty amount, if any present. Embryogenesis during the third week of embryonic life was discussed in relation to the development of neurenteric cyst.