Search

A patient is reported who developed multiple basal-cell carcinomas of the skin, a breast carcinoma, an astrocytoma of the spinal cord, and a bronchial carcinoid tumor following multiple fluoroscopies during artificial pneumothorax treatment for pulmonary tuberculosis. A review of the literature revealed no previously documented cases of radiation-induced gliomas of the spinal cord.

Three cases are reported of raised intracranial pressure associated with spinal tumours. The theories which have been advanced to explain this complication are discussed and it is suggested that in many of the reported cases the underlying mechanism may have been intracranial spread of the tumour and not a blockage of the cerebrospinal fluid pathways with proteinaceous deposits, as has been widely maintained. Autopsy revealed the former process to have operated in 2 of the 3 cases and it may have done so in the third. Two cases had unusual features in common: they were malignant schwannomas (a very rare spinal tumour) and they had both developed within the field of irradiation given for benign conditions many years previously.

The aim of this investigation is to evaluate the accuracy of the diagnostic parameters of the radiological features, especially in conventional lumbar myelography, since these will be used in the future as a gold standard for new developing diagnostic methods like ultrasonography (US) and magnetic resonance imaging (MRI). A short review of the clinical symptoms and diagnostic radiology of the tethered spinal cord (TSC) is given. The radiological features of the TSC syndrome are compared with the peroperative findings in 30 patients investigated and operated in our hospital. All patients showed lumbar or sacral osseous malformations on the plain X-ray films. This means a high diagnostic sensitivity although the specificity for TSC is low. Conventional myelography had a sensitivity of 0.82 and positive predictive value of 0.94, while the specificity and negative predictive value are respectively 0.96 and 0.85. The positive predictive value of the total diagnostic procedure of plain X-ray films and lumbar myelography for TSC may be estimated at 1.

The Authors report 4 cases of spinal lipomas: 2 intradural, 2 completely intrarachidian extradural. The intradural lipomas observed were 2 cases in the lumbo-sacral area, both operated on by the partial removal of the lesion with very good clinical results; histologically they were found to be 2 fibrolipomas. The 2 cases of extradural lipomas, completely intrarachidian, were situated in the thoracic and lumbar areas. Both were operated on with very good results and it was possible to remove all the lesion. They were found to be an angiolipoma and a mature lipoma. On the basis of the literature, the Authors maintain that, for these cases of intradural lipoma, the disembryogenetic hypothesis (such as embryological ectopia) should be accepted as etiopathogenesis. They point out the nature and origin of extradural lipomas, arising in a site where adipose tissue is abundant, have never aroused interest on their origin, but they too can be considered malformative lesions of the hamartomatous type and not neoplastic lesions. These data are confirmed by the remarkable frequency in these cases of the mixed variety of lipoangiomato or angiomiolipomatosis. The Authors thus underline the importance of the relationship between the presence of a subcutaneous lipoma in cases of completely intrarachidian extradural lipomas to support the hypothesis of malformative origin.

Two children with achondroplasia and neurologic sequelae secondary to foramen magnum compression were found to have syringomyelia. In one patient, the cyst was associated with a subependymal glioma. These findings suggest that foramen magnum stenosis may lead to irreversible spinal cord damage.

This case report describes the presence of numerous intradural spinal and cranial epidermoids and lipomata, as an incidental finding, 14 years after a spinal gunshot injury. The aetiological factors operating in the development of such tumours are discussed with reference to the general literature and to this case.

It is well known that congenital intraspinal epidermoid tumors, while rare, do occur. That they may also be related to spinal puncture is less well understood. In the present article, four such cases are described, including one in which an earlier myelogram performed because of lumbar disk disease showed that no tumor was present prior to the spinal puncture. The pertinent literature is reviewed, and the probable mechanism of formation of such tumors and the means for avoiding it are discussed.

Four cases are documented where a glioma was histologically verified in the irradiation field of a previously treated malignancy of a different cell line. Radiation-induced neoplasia in the central nervous system now has been established in the induction of meningioma and sarcoma. The association between therapeutic irradiation and glioma in the reported cases lends to the evidence that a causal relation does exist. This incidence is small and does not detract from the overall benefit of irradiation as a therapeutic modality.

Although complication occasionally occur in the use of ventriculoperitoneal shunts (VP-shunt), the occurrence of intractable ascites and extracranial metastasis through the shunt are uncommon. Recently intractable ascites occurred in a two years old girl in whom a left VP-shunt had been placed before four month for obstructive hydrocephalus secondary to medulloblastoma. Paracentesis was performed with recovery of clear, straw-colored fluid with a specific gravity of 1012, a protein content of 0.3 gm/L and many of mononuclear cell on microscopic examination. Liver-scan and enzyme profile gave normal results. During 3 weeks, 6 times paracentesis were performed and total volumes of ascites were 3850 ml. At 5 and 6 times paracentesis, Carboquone 1 mg was injected into peritoneal cavity and 0.1 mg into ventricle cavity through flashing device. After these injection, intractable ascites were cured, no metastasis can be seen in peritoneal cavity when died 4 month later. No precise explanation can be offered for occurrence of this intractable ascites. It is said that chronic irritation of the visceral and parietal peritoneum or sub-clinical peritonitis may cause peritoneal malabsorption. In our case, antitumor agent was very effective, so this treatment will be recommended to try for intractable ascites.

A 21-year-old patient, after radiation therapy to the mediastinum for Hodgkin's disease, died six years later of a spinal cord glioma, believed to be caused by irradiation therapy. That the x-ray therapy provoked neoplastic changes seems likely, although it could be coincidental.

Four children with previously repaired myelomeningoceles presented toward the end of the first decade or early in the second decade of life with deteriorating lower-extremity and bladder function. Myelography and computerized tomography scanning demonstrated irregular filling defects at the area of the myelomeningocele repair, and surgical exploration disclosed dermoid tumors that were adherent to the placode and adjacent roots. Dermoid tumors should be considered in the differential diagnosis of neurological deterioration in children with a repaired myelomeningocele.

Epidural lipomatosis usually occurs as a complication of steroid therapy. This report includes the first description of a case secondary to hypothyroidism. Adults in all age groups can be affected and present with progressive signs and symptoms of spinal cord or cauda equina compression. The dorsal aspect of the thoracic spine is most commonly involved, usually in its total extent. Spine radiographs are normal and myelography poorly delineates the nature and extent of abnormality. While computed tomography can provide information about the nature of the spinal mass, magnetic resonance imaging is the investigation of choice. Extensive decompressive laminectomy is useful but the results are variable. Correction of underlying endocrine abnormality can reverse the process. The need for early diagnosis of this disorder is therefore emphasized.

Cerebrospinal fluid seeding is a well-known mode of metastasis for intracranial neoplasms such as medulloblastoma, ependymoma, and glioblastoma; however, retinoblastoma is not usually considered. The route of spread appears to be by direct extension into the optic nerve from the retina and into the meningeal spaces by extension from the choroid, or along the central retinal vessels to the subarachnoid space. Four cases are presented.

A male aged 60 is reported who, 5 years after chordotomy at the Th 2/3 level, developed acute paraplegia of the legs and a sensory transverse lesion due to an extradural tumour of the Th 1-4 level. Neuropathology revealed transverse necrosis of the thoracic spinal cord (Th 1-4) due to an intradural tumour at the upper thoracic and lumbar spinal levels, the thoracic malignancy arising at the site of the previous chordotomy, with a suture being observed within the tumour mass. Histology, positive immunostaining of tumour cells with Factor VIII antigen, a specific marker of endothelial cells and the ultrastructural demonstration of Weibel-Palade bodies in endothelial cells of the tumour vessels suggested a malignant mesenchymal tumour of angiosarcoma type presumably arising from the spinal meninges, and broadly invading the spinal cord. The usual relation of this extremely rare intraspinal vasoformative malignancy to previous chordotomy is discussed.

Because prior work with mice had revealed remarkable inhibition of central nervous system (CNS) tumor by chronic Toxoplasma infection, the effect of immunomodulation produced by this obligate intracellular parasite was studied in rats which developed CNS tumors following transplacental exposure to the chemical carcinogen ethylnitrosourea. Groups of Fischer 344 rats which had been exposed to ethylnitrosourea were either uninfected or infected at 1 month of age with a virulent strain of Toxoplasma gondii. Rats were sacrificed when morbid symptoms from tumor growth developed, and neural tissue was prepared for light microscopy. Chronic Toxoplasma infection had no effect on the survival of rats or on the amount, location, or histological type of CNS tumor which developed. Although serum antibody to Toxoplasma was present in all infected rats for the duration of the experiment, there was no histological evidence in the brain of a cellular response to infection or to the presence of tumor. When these results are compared to prior experiments of CNS tumor in mice, they suggest that mechanisms of protection against Toxoplasma infection differ in mice and rats and that an inflammatory component produced by the Toxoplasma organism in the brain is a necessary prerequisite for tumor inhibition.

The simultaneous occurrence of neurofibromatosis and a peripheral neuropathy that has the clinical and electrophysiological features of Charcot-Marie-Tooth disease (HMSN I) has rarely been reported. A recent report described patients with HMSN I with hypertrophic lumbosacral nerve roots. We report a patient with compelling evidence for neurofibromatosis who also demonstrates clinical and electrophysiological features of Charcot-Marie-Tooth disease. Abdominal and pelvic CT scan revealed diffusely and symmetrically enlarged lumbosacral nerve roots. These nerve roots were biopsied, and the specimens revealed neurofibromas. Histology, electrophysiological studies, radiology, and clinical appearance of the abnormality in peripheral nerves and lumbosacral nerve roots will be emphasized in this paper. The simultaneous occurrence in our patient of neurofibromatosis and Charcot-Marie-Tooth disease suggests a possible genetic relationship between these two disorders.

A 5 year old boy developed an implantation dermoid tumour after a lumbar puncture in infancy. A survey of senior paediatricians has shown it to be common practice to use an unstiletted needle for lumbar puncture in small children. A stiletted needle is recommended for all such procedures.

A hypothesis is advanced concerning the relationship of poliomyelitis viruses and late developing neoplasia in the central nervous system. The literature is surveyed, and several experimental approaches based upon known evidence of viral and neoplastic behaviour are advanced. The present status of world polio incidence is discussed as it applies to this possible relationship.