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Neoplasms of the peripheral nervous system.

Neoplasms of the peripheral nervous system arise from the cellular sheath surrounding the nerve trunks, that is, the pluripotential Schwann cells and related cells, and rarely affect the feet. When present, they are most frequently associated with the autosomal dominantly inherited neurofibromatosis 1. This condition has been related to chromosome 17, and it appears, from in vitro experiments, to involve defects in tyrosine metabolism. Hence, the most common neoplasm is the neurofibroma. Distinct criteria have been established for a diagnosis of neurofibromatosis 1, so that a single pedal neurofibroma may not represent this complex. However, if the complex is present, it is necessary to consider the possibility of malignant transformation to a neurofibrosarcoma. Although malignant peripheral nerve neoplasms are extremely rare in the feet, they may arise in the context of neurofibromatosis 1, or independently. Other benign or malignant schwannomas may (rarely) also arise in the feet. Surgical excision of benign lesions, according to established standards for tumor surgery, is usually curative, but a detailed personal and familial history, along with adjunctive radiologic procedures and biopsy, is necessary to determine the nature of the lesion. Since (endocrine and) other abnormalities may complicate neurofibromatosis 1, surgical procedures must not be undertaken until the patient has been medically cleared and is carefully monitored. A high mortality rate is associated with malignant peripheral neurogenic tumors, especially those arising in the context of neurofibromatosis 1. It should be recalled that neurologic manifestations in the foot may represent non-neoplastic conditions as well as peripheral nerve tumors (or other tumors involving those nerves) that are proximal to the foot and ankle area.

Adult↗

Anti-neurofilament monoclonal antibodies: reagents for the evaluation of human neoplasms.

Sixty human nervous system neoplasms were examined by immunohistochemistry using a monoclonal antibody against neurofilament triplet proteins. Only those of neuronal origin had tumor cells with intracytoplasmic, immunoreactive neurofilament triplet proteins. However, not all such neoplasms contained labeled tumor cells. Benign or differentiated neuronal tumors more often contained labeled cells than malignant, less differentiated neoplasms of the neuron series. We conclude that anti-neurofilament monoclonal antibodies are useful reagents for the evaluation of human neoplasms.

Antibodies, Monoclonal↗

[Effect of PS-K on experimental neurogenic tumors in rats induced by ethylnitrosourea].

The effect of PS-K as immunomodulator was studied in Fischer rats bearing neurogenic tumors induced transplacentally by ENU. 114 rats were divided into control and PS-K treated groups. all rats were subjected to an autopsy at death and all of central nervous system were examined macroscopically. Final neurogenic tumor incidence in PS-K treated groups was significantly lower than that in the control group, but distribution of these neurogenic tumors and number of neurogenic tumors in a rat remained unchanged between the control and the each PS-K treated groups. Survival time of PS-K treated groups did not increase than the control group. From these experiments, it might be that induction of neurogenic tumors may be suppressed by administration of PS-K.

Adjuvants, Immunologic↗

CNS involvement in leukaemia. An autopsy study of 100 consecutive patients.

The central nervous system has been examined in a consecutive autopsy material of 100 adult patients with acute (N = 67) or chronic (N = 33) leukaemia. In all patients the disease was active at the time of death, infiltrating several organs, and in 45% of the cases CNS was involved. 81% of the patients with ALL had leukaemic infiltrates in CNS, and in the total AML group they were seen in 46% (P less than 0.05). A comparison between the subtypes of the AML group revealed CNS involvement in 39% of M1 + M2 and in 69% of M4 + M5 (P less than 0.05). Only in a single case of CML was the central nervous system affected, whereas 8 of 16 patients with CLL has CNS involvement. Furthermore, other pathological findings such as haemorrhages and infarcts were registered at the time of death in 33% of all patients. Terminal neurological symptoms could be ascribed either to leukaemia, other CNS pathology or a combination of both. The rate of CNS involvement is higher than reported in similar studies, and it is supposed that this may partly be ascribed to the inclusion of the spinal cord in this investigation.

Acute Disease↗

Human immunodeficiency virus-related lymphoreticular malignancies and peripheral neurologic disease. A report of four cases.

The most common human immunodeficiency virus-related (HIV) malignancies to date include Kaposi's sarcoma and the high-grade non-Hodgkin's lymphomas. There also appears to be an association between HIV and an aggressive form of Hodgkin's disease. In addition, there is a spectrum of HIV-related central and peripheral neurologic syndromes. This article documents four patients with HIV-associated lymphoma who presented with peripheral neurologic syndromes as part of their neoplastic process. Autopsy results obtained from two of these patients showed direct nerve infiltration by lymphoma. All patients had an elevated serum lactate dehydrogenase (LDH). It is recommended that HIV-related lymphoma be considered in a high-risk patient who presents with a peripheral neurologic syndrome especially if there is an elevated serum LDH.

Acquired Immunodeficiency Syndrome↗

Neurologic disorders associated with AIDS retroviral infection.

Neurologic disease occurs frequently in patients infected with the human immunodeficiency virus (HIV), and a large body of literature now exists detailing the various infections, neoplasms, and other conditions that can affect the central nervous system (CNS) or the peripheral nervous system in children and adults with AIDS, persistent generalized lymphadenopathy, or (in some cases) only serologic evidence of retroviral infection. Although certain opportunistic infections (toxoplasmosis, cryptococcosis, progressive multifocal leukoencephalopathy, and herpesviral infections) and CNS lymphomas often produce CNS disease in patients with AIDS, it is now clear that many cases of neurologic disease are caused by a group of disorders thought to be related to direct CNS infection by the AIDS retrovirus. Disease of the peripheral nervous system is also being increasingly recognized; some cases probably have an autoimmune basis.

Acquired Immunodeficiency Syndrome↗

AIDS and neurological disorders: an overview.

Neurological disease occurs frequently in patients infected with the human immunodeficiency virus. Disorders may affect either the central or peripheral nervous systems and may be the presenting manifestation of human immunodeficiency virus-related disease. Opportunistic infections and lymphomas are major causes of central nervous system disease. Increasingly, however, human immunodeficiency virus infection of the central nervous system is being recognized and is now associated with a syndrome of progressive dementia in adults, referred to as the acquired immunodeficiency syndrome dementia complex, and an encephalopathy in infants born to human immunodeficiency virus-infected mothers. Whether brain disease related to this virus will respond to antiretroviral drugs will be a major focus of future research. Although less frequent than central nervous system disease, disorders of the peripheral nervous system are increasingly being recognized, including cases that probably have an autoimmune basis.

Acquired Immunodeficiency Syndrome↗

[Neuropathology of HIV infection].

Some references about the important neuropathologic lesions observed in patients with AIDS and about the variation on the relative incidence of the processes detected when analyzing clinic and neuropathologic tables, as well as a mention to the confusion created by terminological problems, clinicopathologic disagreement and wrong etiologic attributions, are used as an introduction to the exposition of HIV infection neuropathology. In a first section, Central Neuropathology is described, starting with the disorders considered as primarily caused by HIV. Then, infectious secondary Neuropathology, caused by different viruses, parasites, fungi or bacteria, and neoplastic secondary Neuropathology, mainly represented by localized lymphoma and by the uncommon Kaposi's disease, are analyzed. This section also includes references to cerebrovascular complications, Pediatric Neuropathology and ends with an analysis of cerebral biopsy indications. In the second part, Peripheral Neuropathology is described, splitting up peripheral nerve and muscle pathology.

Acquired Immunodeficiency Syndrome↗

Age- and dose-dependent transplacental carcinogenesis by N-nitrosoethylurea in Syrian golden hamsters.

Syrian golden hamsters have a very short period (15 days) of gestation. The implantation of the blastocyst occurs on day 5, embryogenesis proceeds very rapidly thereafter and neural tube closure is completed by day 9. In the present study the effects of two different doses of N-nitrosoethylurea (NEU) administered at various stages of gestation were quantitatively evaluated in Syrian golden hamsters. NEU at either 0.2 or 0.5 mmol/kg was administered transplacentally as a single i.p. injection to pregnant hamsters on gestation days 7, 8, 9, 10, 11, 12, 13, or 14. The incidence, latency period and multiplicity of tumors varied with the dose of NEU and the stage of development at the time of NEU administration. Although tumors of the peripheral nervous system predominated, a variety of other tumors, including melanomas and visceral tumors of epithelial and mesenchymal origin, were also observed in hamster offspring exposed transplacentally to NEU. Sensitivity to transplacental carcinogenesis was maximal during late gestation and very low before day 9.

Age Factors↗

Effect of light/dark regimen on N-nitrosoethylurea-induced transplacental carcinogenesis in rats.

Pregnant females were randomly subdivided into three groups (24 rats per group) and kept at the 12:12 h light/dark regimen (group 1), at the constant light illumination (24 h a day, group 2) or at the continuous darkness (group 3). N-nitrosoethylurea (NEU) has been injected into the tail vein of all rats (80 mg/kg) on the 18-19th day of the pregnancy. After the delivery the lacting dams and their progeny during the lactation period (1 month after delivery) were kept also at the three different light/dark regimens. Then all offspring from each group was kept at the 12:12 h light/dark regimen, males and females separately, and were observed until natural death. The exposure to constant light significantly promoted the transplacental carcinogenesis whereas the exposure to constant darkness inhibited it. The incidence of total tumors, tumors of both a peripheral nervous system and kidney was 2.6; 2.5 and 8.5 times higher, and survival significantly shorter, correspondingly, in rats from the group 2 exposed to the constant light regimen as compared to the group 1 (12:12 h light/dark regimen) (P<0.05). On the other hand, the exposure to the continuous darkness during the pregnancy and the lactation period significantly inhibited the transplacental carcinogenesis in the offspring of rats treated with NEU. The incidence of total tumors, tumors of a peripheral nervous system was by 2.4 and 2.7 times less, and survival longer, respectively, in exposed to the darkness rats from the group 3 as compared to the group 1 (12:12 h light/dark regimen) (P<0.05). Thus, our data firstly have shown the modifying effect of light-dark regimen on the realization of the transplacental carcinogenesis induced by NEU in rats.

Alkylating Agents↗

Immunohistochemical diagnosis of nervous system neoplasms.

Classic histopathologic study revealed a series of highly treasured criteria for diagnosing central and peripheral nervous system neoplasms. The advent of immunohistochemistry galvanized further improvements in the accuracy of diagnostic neurooncologic pathology, and moreover, enriched our understanding of tumor histogenesis. Now with the tremendous technical advances in immunodetection and the commercial availability of high-quality monoclonal and polyclonal antibodies, the potential for first-rate diagnostic assessment is available to practically every laboratory, regardless of how small or remotely located. Intensive immunohistochemical analysis of nervous system neoplasms has taught us that neuroectodermal cells have a limited repertoire of highly redundant antigenic phenotypes and that neoplastic cells usually mimic their non-neoplastic immature or fully differentiated counterparts. These observations led to the concept that a panel of antibodies ought to be used to identify the "immuno-gestalt" of tumors, because it is often difficult if not impossible to subcategorize the tumors on the basis of a single immunohistochemical determination. The ensuing tour through the immunodiagnostic neurooncology summarizes approximately 10 years of original work in this field. I have attempted to provide consensus opinions, and whereever appropriate, highlight dissenting arguments. The salient immunohistochemical features of most nervous system tumors also are summarized in tabular form to facilitate diagnostic considerations at your own institutions.

Antigens, Neoplasm↗