Intraventricular cerebral neuroblastoma. Analysis of subtypes and comparison with hemispheric neuroblastoma.

Two cases of intraventricular neuroblastomas were compared with cases of intraventricular and hemispheric neuroblastomas that have been reported in the published literature. The following order of tumor subtypes was found in patients with increasing age: hemispheric neuroblastoma, intraventricular undifferentiated neuroblastoma, intraventricular differentiated neuroblastoma, and intraventricular neurocytoma; for patients with intraventricular neuroblastomas, this was also the order of increasing cellular maturation and survival. Neuronal morphologic or epitope differentiation was associated with a longer survival time than lack of differentiation by Kaplan-Meier product-limit estimates and with a better survival rate (chi 2) for intraventricular tumors but not for hemispheric tumors. Pathologic distinction of a neurocytoma was confirmed with immunostaining or ultrastructural studies that suggested that a neurocytoma is a matured neuroblastoma of a granule-cell (interneuron) phenotype. Differences among neuroblastoma groups bolster previous suggestions that intraventricular tumors arise differently than do cases of hemispheric tumors and follow a more benign course when neuronal differentiation is present.

In utero brain damage: relationship of gestational age to pathological consequences.

The authors examined the brains of two infants in whom episodes of fetal compromise could be accurately correlated with gestational age. A mother who had sustained hypotensive shock at 16 weeks gestation gave birth at 20 weeks to a stillborn infant whose brain showed cystic necrosis of the lenticular nuclei and multifocal polymicrogyria. The brain of a seven-month-old infant born at 33 weeks gestation to a mother who had experienced respiratory arrest during her 28th week showed thalamic and brainstem necrosis accompanied by diffuse white matter gliosis. Review of the literature yielded 10 similar cases. When the insults occurred before 24 weeks of gestation, bilateral pallidal necrosis was a constant feature. Between 26 and 34 weeks, the common finding was thalamic necrosis, often accompanied by brainstem necrosis. These observations should be of assistance in determining the timing of gestational insults, and therefore possible clinical correlations, in prenatal brain damage.

Intracranial lipomas with teratomatous elements.

We report two cases of patients with intracranial tumors that share features with lipomas and teratomas. Although rare reports of lipomas with "hypertrophic nerves" and "teratoid tumors" have been recorded, these two cases are unusual because they contain mature neuroectoderm (choroid plexus, peripheral nerve) and mesoderm (skeletal muscle). The findings are discussed and modern classification schemes are presented. We believe that the cases are examples of a transition between lipoma and teratoma.

Amyloidoma of the CNS. I. Clinical and pathologic study.

We report a 32-year-old man with a 4-year history of headaches, seizures, and dementia secondary to multifocal amyloidomas in the white matter. Immunohistochemical and electron microscopic analyses suggest that the amyloidomas resulted from processing of plasma-cell-derived amyloidogenic protein by microglial cells.

Immunohistochemical properties of human optic nerve glioma. Evidence of type 1 astrocyte origin.

The peroxidase-antiperoxidase method was used to study ten surgically obtained human optic nerve gliomas (pilocytic astrocytomas). All tissues were formalin fixed and paraffin embedded. Primary antisera included glial fibrillary acidic protein (GFAP), HNK-1 (type 1 astrocyte precursor marker), A2B5 (type 2 astrocyte precursor marker), S-100, vimentin, myelin basic protein (MBP), laminin, keratin, cytokeratin, epithelial membrane antigen (EMA), and neuron-specific enolase (NSE). Neoplastic astrocytes in optic nerve gliomas stained with GFAP, HNK-1, S-100, and vimentin. Oligodendrocytes and myelin sheaths stained for MBP, and NSE stained surviving axons in the tumors. Neoplastic astrocytes did not stain for A2B5, keratin, cytokeratin, EMA, or laminin. These results suggest that human optic nerve gliomas (pilocytic astrocytomas) arise from type 1 astrocytes.

The erythrocyte-type glucose transporter in blood vessels of primary and metastatic brain tumors.

We previously demonstrated that brain microvessels have a high density of the erythrocyte-type glucose transporter and suggested this could be used as a marker of cells with "occluding" junctions. Now, we have studied by immunocytochemistry the tissue distribution of the glucose transporter in a variety of primary and metastatic human brain tumors with a monoclonal antibody to the glucose transporter of human erythrocytes. Microvessels in normal brain tissue distant from the tumor, and in brain surrounding the tumor, immunostained strongly for the glucose transporter. Microvessels in primary and metastatic tumors, however, lacked glucose transporter immunoreactivity. Only in the relatively benign juvenile cerebellar astrocytomas was glucose transporter immunoreactivity retained in some of the tumor microvessels. Because a high glucose transporter density is a marker of vessels with barrier properties, our results indicate that the vast majority of brain tumor microvessels do nc, have an intact blood-brain barrier.

Giant invasive intracerebral dermoid tumor with subependymoma-like reaction: case report.

A large, completely intracerebral, dermoid tumor with extensive outgrowth of appendages into brain tissue caused prominent reactive changes in the surrounding white matter, similar to those seen in a typical subependymoma. This case lends support to the notion that some subependymomas are reactive in etiology.

Septo-optic dysplasia (SOD) or DeMorsier syndrome.

The historical evolution of the DeMorsier syndrome is reviewed. As the emphasis shifted from the eye findings to endocrinological defects and as the syndrome was further expanded through more frequent and easier radiological examination, the concept appears to have expanded, raising questions as to the nature of the disease and its clinical expression. To determine pathogenesis and better define the extent of the disease, further observations are needed, including postmortem studies on patients with septo-optic dysplasia and related disorders.

Large pituitary gland adenomas evaluated with magnetic resonance imaging.

Magnetic resonance imaging (MRI), computed tomographic (CT) scanning, and angiography or digital subtraction angiography (DSA) were used preoperatively to evaluate 16 surgically proven cases of sellar tumor with extrasellar extension. There were 15 pituitary tumors and 1 schwannoma. The capabilities of MRI in evaluating these tumors were compared with CT scanning and angiography. Bone destruction and tumor calcification were better detected by CT scanning than by MRI. MRI was as effective as CT scanning in detecting a cyst or variation in tumor consistency. Neither MRI nor CT scanning was capable of distinguishing specific tumor types. In every case, MRI was superior to CT scanning for delineating spatial relationships of the tumor to the 3rd ventricle, the optic apparatus, adjacent brain, and parasellar vasculature. Vessel encasement by tumor was clearly seen on MRI when there was no direct indication of this on other studies. Cavernous sinus invasion was not demonstrated by CT scanning, but was indicated by MRI in 5 cases and was surgically confirmed in 3. MRI can provide more precise spatial information on extrasellar tumor extension. Vascular encasement and cavernous sinus invasion may be determined preoperatively. Treatment expectations and operative approaches can be guided by this information. When MRI is available, it is the test of choice for the preoperative evaluation of patients with suspected large pituitary gland (sellar region) tumors. Contrast-enhanced CT scanning and angiography can be used as supplementary studies to add information inherently unique to these techniques.

Neuropathology of "septo-optic dysplasia" (de Morsier syndrome) with immunohistochemical studies of the hypothalamus and pituitary gland.

The de Morsier syndrome, or septo-optic dysplasia, is a developmental anomaly characterized by involvement of the optic system, hypothalamic-pituitary axis and septum pellucidum. Only a few anatomical observations are recorded. We report three new cases and review the pertinent literature. The neuropathological lesions varied as did the clinical features. The hypothalamic nuclei were most commonly involved, followed by the optic system and the septum pellucidum. Other lesions were found in the cerebral cortex, corpus callosum, olfactory system and cerebellum. The hypopituitarism appeared to have been secondary to hypothalamic damage rather than to intrinsic pituitary defect. A virtually normal histology and the usual endocrine cell populations were demonstrated by immunocytochemistry in the adenohypophysis. Damage to the neurophysin-containing cells of the hypothalamus explains the various degrees of clinically observed diabetes insipidus.

Cerebro-ocular dysplasia-muscular dystrophy syndrome. Report of two cases.

Cerebro-ocular dysplasia-muscular dystrophy (COD-MD) syndrome is a rare disorder encompassing a triad of brain, eye, and muscle abnormalities. The principal central nervous system features are cerebral and cerebellar agyria-micropolygyria, cortical disorganization, glial-mesodermal proliferation within the leptomeninges, neuronal heterotopias, hypoplasia of nerve tracts, hydrocephalus, and, occasionally, encephalocele. Ocular abnormalities include microphthalmia, cataract, immature anterior chamber angle, retinal dysplasia with or without retinal detachment, persistent hyperplastic primary vitreous, optic nerve hypoplasia, and coloboma. Skeletal muscle findings include fiber splitting, variable fiber size, and endomysial fibrosis. Recent evidence has shown that COD-MD syndrome may be identical to the Walker-Warburg (also known as Warburg) syndrome. Fukuyama congenital muscular dystrophy is similar to the COD-MD and Walker-Warburg syndromes, although the ocular manifestations are less severe. We report the histopathologic findings in two siblings with multiple features of COD-MD syndrome.

Diffuse dysplasia of cerebral hemispheres in a fetus. Possible viral cause?

The pathologic anatomy of an infant born with an occluded ventricular system and cerebral dysplasia is described. The possible role of an intrauterine viral infection as the cause is discussed, as is the possible relationship of this lesion to cerebro-ocular dysgenesis (Warburg syndrome).

Anaplastic astrocytoma mimicking metastatic carcinoma.

A case of anaplastic astrocytoma mimicking a metastatic carcinoma is presented. This rare type of astrocytoma with epithelial features is compared to cases reported in the literature, and the importance of staining brain tumor biopsies for glial fibrillary acidic protein is emphasized.

Major cerebral infarction from tumor embolus.

A major right hemispheric infarct developed in a 31-year-old man within forty-eight hours of lung resection for metastatic synovial-cell sarcoma. Post mortem exam revealed tumorous occlusion of the right internal carotid artery. Major stroke from cerebral tumor embolus should be seriously considered in patients with primary or metastatic lung cancer who have had a very recent pneumonectomy, especially when there are symptoms and signs of multi-organ or extremity ischemia.

Duplication of the pituitary gland and spinal cord.

Multiple malformations, including duplication of the pituitary gland, stalk, and infundibulum, and complex duplication of the spinal cord were observed in a female infant. The problems of classification of the spinal cord duplications are discussed, and the role of the notochord in the formation of double organs is proposed.

Ultrastructural and histochemical abnormalities of skeletal muscle in a patient with a new variant (type Homburg) of glucosephosphate isomerase (GPI) deficiency.

Ultrastructural and enzyme histochemical muscle abnormalities are described in a case with a new variant (type Homburg) of glucosephosphate isomerase (GPI) deficiency, associated with congenital nonspherocytic hemolytic anemia and muscle weakness. The enzyme is thermostable in contrast to other described variants. The muscle fibers showed decreased GPI activity, ultrastructural abnormalities, including giant mitochondria, and a diffuse increase of glycogen. The functional alteration of muscle tissue is due to a stable enzyme protein with decreased specific activity.

Extradural spinal angiolipoma with secretory activity. An ultrastructural, clinico-pathological study.

The light and ultrastructural analyses of an extradural intraspinal angiolipoma causing symptoms of spinal cord compression, are reported. The tumour showed morphological evidence of an endocrine-like secretory activity of fat cells, with an apparent mechanism of secretory function that has not previously been described for angiolipomas. The secretory granules, containing a lipid-like material, were covered with a continuous basement membrane originating from the basement membrane of the adipocyte.

Primitive neuroectodermal tumors of the cauda equina in adults with no detectable primary intracranial neoplasm--three case studies.

We studied three cases of primitive neuroectodermal tumors surgically removed from the cauda equina region of adults. There was no clinical or radiological evidence of cerebellar medulloblastoma, other intracranial tumor or paraspinal sympathetic neuroblastoma. Two patients died: autopsies revealed no primary intracranial neoplasm. One patient is alive 3 years after surgery with no detectable intracranial tumor. The tumors had the light and electron microscopic features of primitive neuroectodermal tumors as described earlier in the literature. In addition, in all three cases many tumor cells could be stained for cytoplasmic neurofilament antigen.

Fourth ventriculoceles with extracranial extension.

The radiographic features and long-term clinical outcome in three patients who presented at birth with a cystic suboccipital mass in direct communication with the fourth ventricle are reviewed. The pathological findings in a fourth infant who died are also discussed. All surviving infants were treated with cyst excision and diversion of cerebrospinal fluid. The prognosis in these children, followed from 6 to 20 years, surpasses that of the more common occipital encephalocele, for which this entity could be mistaken. The morphogenetic implications relative to more common congenital lesions in this location are discussed.

Chiasmal apoplexy: hemorrhage from a cryptic vascular malformation in the optic chiasm.

We describe three patients with acute chiasmal visual field loss caused by hemorrhage within an intrachiasmal arteriovenous malformation. One patient had five episodes; vision improved three times without treatment and twice after surgical decompression. Surgical intervention restored vision in two of the three patients. The role for surgery is indefinite, but may be indicated if the visual defect remains static for 1 week or progresses. Decompression is likely to be most effective if done early. The approach to the optic chiasm should be transcranial, not transsphenoidal. A radiologically normal sella turcica favors the diagnosis of chiasmal apoplexy in the acute chiasmal syndrome.