Metastatic renal cell carcinoma simulating glomus jugulare tumor.

A 59-year-old man presented with jugular foramen syndrome caused by a mass with roentgenographic and histologic features highly suggestive of a glomus jugulare tumor. However, electron microscopic examination of the surgical specimen revealed features diagnostic of a previously unsuspected renal cell carcinoma. Because primary tumors of the glomus jugulare and metastatic renal cell carcinoma may present with the same clinical and roentgenographic findings and look similar histologically, careful electron microscopic examination of the tumor and urologic screening should be performed in suspected cases of glomus jugulare tumors.

Central neural antigens: detection and diagnostic application.

During the last few years, methods have been developed which permit practical use of biochemical research on the nervous system. In the central nervous system, proteins have been identified for astrocytes (glial fibrillary acidic protein and vimentin) and oligodendroglia (myelin basic protein and other glycoproteins). For certain classes of nerve cells, the neurofilament proteins and neuron-specific enolase (a glycolytic isoenzyme) have been identified. Detection of some of these substances in body fluids is possible via radioimmunoassays (RIA) and in tissue sections using the peroxidase-antiperoxidase immunohistochemical method.

High dose Ara-C related leukoencephalopathy.

Two patients with acute myelomonocytic leukemia in central nervous system relapse developed clinical signs and computerized tomographic evidence of leukoencephalopathy five to seven days after intravenous high dose Ara-C therapy. The first patient had received 30 gm of intravenous Ara-C with cranial irradiation (1680 rad in 2 fractions) and intrathecal Ara-C (100 mg X twice) for an intracerebral chloroma and leptomeningeal leukemia. In this patient the leukoencephatlopathy was probably a result of a synergistic effect of the concomitant triple therapy. The second patient had received intrathecal administration of Ara-C and methotrexate for five and one-half months prior to intravenous Ara-C therapy. He developed altered mental status after 24 gm of intravenous Ara-C infusion. CT scan showed changes in the white matter compatible with leukoencephalopathy. In this patient the intravenous Ara-C probably was the precipitating factor of the development of leukoencephalopathy. The possible mechanism of the Ara-C induced leukoencephalopathy is discussed.

The endocrine glands in Pompe's disease. Report of two cases.

Pompe's disease (type II glycogenosis), an infantile form of generalized glycogenosis, is characterized biochemically by deficiency of lysosomal acid alpha-1,4-glucosidase and morphologically by intralysosomal glycogen storage in multiple organs, notably the central nervous system, heart, liver, and skeletal muscles. The endocrine system has not been described in detail in the literature. In two infants with Pompe's disease, intralysosomal glycogen was identified in the adrenal cortex and medulla, thyroid gland, parathyroid glands, pancreatic islets, and pituitary gland. Of special interest is the severe glycogen accumulation in the zona fasciculata of the adrenal glands.

Vimentin and glial fibrillary acidic protein in human brain tumors.

Using the peroxidase-antiperoxidase (PAP) technique, we examined 35 primary brain tumors for expression of vimentin and GFAP. Both low-grade and high-grade astrocytomas contained vimentin-positive and GFAP-positive cells. Ependymomas also stained for both markers. In gliosarcomas, the glioblastomatous portions stained like astrocytomas, while only vimentin stain was seen in the fibrosarcomatous portions. Medulloblastomas and oligodendrogliomas were negative for both vimentin and GFAP, while meningiomas contained scattered areas of vimentin-positive cells. These results suggest that the expression of vimentin and GFAP is mostly confined to glial-derived tumors, and that vimentin can potentially be a useful marker for distinguishing undifferentiated GFAP-negative glial tumors and mesenchymal tumors from primitive neuroectodermal tumors.

A rabbit model of intracerebral hematoma.

The epiphenomena that seem to cause deterioration and death after spontaneous interacerebral hematoma (SICH) might best be studied in an animal model. Therefore, the principles for developing such a model and techniques to study these phenomena were evaluated. Animals will tolerate injection of 3%-5% of their brain volume with a high proportion of clots. Fluorescein can be used to study the blood-brain barrier, and gravimetry to study edema. Others have found that injection of a paraffin/oil mixture can be employed for a control model. Refinement of the fluorescein technique, development of a primate model, and directions for future research are suggested.

Clinicopathological correlations of disseminated intravascular coagulation in patients with head injury.

To try to define the significance of disseminated intravascular coagulation (DIC) in head-injured patients, we correlated clinical, laboratory, and pathological findings in 16 patients with head injury as their main problem who had DIC, who died within 4 days of injury, and who were examined postmortem. Patients were ranked according to the number of abnormal laboratory screening tests for DIC and the severity of these abnormalities. The most frequently abnormal laboratory tests were the fibrinogen degradation products and fibrinogen, followed in order by the activated partial thromboplastin time, prothrombin time, and thrombin time. The platelet count was the least abnormal value. The patients with the fewest abnormalities had the least abnormal computed tomographic scans. Autopsy reports revealed necrosis and bleeding in the brain and in a number of other organs, particularly the lungs. Microthrombi were not reported in the original autopsy reports. However, when these cases were reevaluated and their slides were stained with an immunoperoxidase technique using rabbit anti-human fibrinogen antiserum, microthrombi were seen frequently. Large microthrombi were more common in patients who had died within less than 24 hours, suggesting a relationship to death or to less time for lysis. In order of frequency, the brain/spinal cord, liver, lungs, kidneys, and pancreas were most commonly affected, and the liver, pituitary gland, pancreas, thymus, brain/spinal cord, large intestine, kidneys, and lungs had the greatest density of microthrombi. Pulmonary dysfunction had been a frequent problem in these patients, which may have been related to the high incidence of microthrombi and bleeding found in the lungs.(ABSTRACT TRUNCATED AT 250 WORDS)

Hemiballism and metastatic brain tumor.

We describe a 78-year-old man with right hemiballism as the presenting symptom of a metastatic occult adenocarcinoma of the lung. CT demonstrated two contrast-enhancing lesions: one in the left subthalamic region and the other in the left parietal region. Metastatic tumor was confirmed at autopsy.

The biological and clinical behaviour of pilocytic astrocytomas of the optic pathways.

The clinical and pathological findings in thirty patients with pilocytic astrocytomas of the anterior visual pathways were analysed. Our cases and those in the literature did not show positive correlation between the extent of surgical resection or irradiation or the presence of residual tumour, and the length of survival or degree of residual disability. Most of these tumours may be considered hamartoma-like growths, akin to developmental malformations, among which aggressive behaviour is exceptional. Their inherently benign nature, associated with long periods of dormancy and spontaneous regression, indicate that treatment should be conservative, and that a surprisingly favourable outcome can often be anticipated.

Germ-cell tumors of the diencephalon.

Germ-cell tumors arising in the neuraxis have similar histopathological characteristics to those germ-cell tumors occurring in the gonads and other midline body sites. As a rare cause of intracranial neoplasm, germ-cell tumors affect predominantly the diencephalon and in particular the pineal region and suprasellar structures. Clinical features are due to local infiltration of these areas by tumor and hydrocephalus. Germinomas or mixed-cell tumors with a germinoma component make up the majority of these tumors and are very responsive to irradiation and chemotherapy. Early clinical recognition of a diencephalic tumor and determination of the specific tumor-cell type may result in curative treatment by surgery, irradiation and chemotherapy.

Myelin basic protein and glial fibrillary acidic protein in human fetal brain.

We demonstrated myelin basic protein (MBP) and glial fibrillary acidic protein (GFAP) in tissue sections of routinely-processes premature human brain employing the peroxidase-antiperoxidase (PAP( method. The MBP immunostain delineated oligodendroglia before the appearance of myelin sheaths. The GFAP immunostain indicated that in addition to the stellate astrocyte, bouquet-shaped glia and radial glia are astrocytic in nature. The bouquet-shaped glia may be the normal counterpart for the gemistocytic astrocyte. The glia limitans stained with GFAP beginning with our first specimen. Small numbers of ependymal cells also contained cytoplasmic GFAP.

The separation of pineocytoma from pineoblastoma.

From our study of eight pineoblastomas and five pineocytomas and a review of the literature, we have described two clinicopathologic syndromes that characterize these neoplasms. Pineoblastomas highly resemble the medulloblastoma-neuroblastoma group of tumors and occur mostly in young people. The tempo of progression of the disease is fast, the length of illness is short. These are infiltrating neoplasms that commonly spread via the cerebrospinal fluid. They are radiosensitive. Histologically they are also similar to the medulloblastoma-neuroblastoma group and are characterized by the scarcity of cytoplasmic processes and by the Homer Wright rosette. They contain giant cells. Pineocytomas are tumors of adults. The tempo of progression of the disease is slow, and the length of illness is long. They expand by compressing the surrounding tissues. Histologically they are characterized by the abundance of cytoplasmic processes and by the pineocytomatous rosette. They contain giant cells. Areas composed of neoplastic gangliocytes and astrocytes in various combinations are common variants in some of these neoplasms.

The role of macrophages and polymorphs in the levan-induced inhibition of Lewis lung carcinoma in C57BL mice.

High-mol.-wt levan injected locally inhibits the growth of Lewis lung carcinoma in C57BL mice. The inhibition is dependent on the number of tumour cells injected and on the dose of levan. The inhibition decreases tumour incidence and size as well as prolonging survival. The polysaccharide is most effective when injected daily beginning on the day of tumour-cell inoculation. Treatment begun on later dates is less effective. Treatment begun one day before tumour-cell inoculation enhances tumour growth. Histological studies showed that levan induces an intense polymorphonuclear (PMN) reaction followed by accumulation of vacuolated, levan-laden macrophages. Both PMN and activated macrophages seemed to have an inhibitory effect upon the growth of the tumour. The effector role of PMN was not explained by the histological study. Tumour cells in close contact with levan-laden macrophages appeared mostly necrotic. Administration of levan begun one day before tumour-cell inoculation produced a similar reaction, but the infiltrating cells did not appear to approach and damage the tumour cells.

Pineocytoma with astrocytomatous differentiation.

A pineocytoma with astrocytomatous differentiation in a 57-year-old man is reported. The histogenetic and oncogenetic implications of this neoplasm are briefly discussed.

Aberrant lacrimal gland and pleomorphic adenoma within the muscle cone.

Aberrant lacrimal gland tissue within the muscle cone formed a pleomorphic adenoma (benign mixed tumor). Histopathologically, the lesion was identical to similar neoplasms originating from lacrimal and other salivary glands as well as from other serous glands of the body.

Sphenoidal pituitary adenoma.

A 62 year old female died of a chromophobe pituitary adenoma of the sphenoid bone. A normal pituitary gland was present within the sella turcica. She also had a chief cell parathyroid adenoma, a papillary thyroid carcinoma, and an ovarian thecoma (multiple endocrine neoplasia syndrome).

Cerebral malakoplakia.

A newborn male developed diffuse myoclonus. Right frontal craniotomy revealed a thin hemispheric mantle and a cyst communicating with the right lateral ventricle. In the biopsy of the cyst wall there were the characteristic findings of malakoplakia, granulomatous inflammation with von Hansemann histiocytes and Michaelis-Gutmann bodies. The child died at 1 1/2 years of age.