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PubMed1986

Oncogene expression in neurofibromatosis.

To investigate the role of oncogenes in malignancies characteristic of neurofibromatosis, oncogene transcripts were quantitated in a neurofibrosarcoma and in control tissue from a patient with hereditary neurofibromatosis. Sis and N-ras were moderately hyperexpressed, raf, Blym, and erbA were slightly hyperexpressed, and abl, erbB, fes/fps, fgr, fos, mos, myb, myc, N-myc, rasHarvey, rasKirsten, ros, src, and yes were not hyperexpressed in the tumor compared to the control tissue. Although additional tumors will be assayed before conclusions are possible, it may be significant that the two oncogenes most hyperexpressed are prior suspects for a pathogenetic role in tumors of the nervous system.

Gene Expression Regulation↗

PubMed2005

Of all the nerve! A subcutaneous forelimb mass on a cat.

A 16-year-old, male, neutered cat had a 2.5 X 1.5 cm mass on the medial aspect of the right carpus. Cytologic examination of a fine-needle aspirate of the mass indicated a markedly pleomorphic population of plasmacytoid to histiocytic-appearing cells. The cytologic diagnosis was malignant neoplasia of probable mesenchymal or round cell origin. The right forelimb was surgically removed and the scapular, axillary, and prescapular lymph nodes were excised. Malignant fibrous histiocytoma was tentatively diagnosed histologically; however, the tumor cells subsequently were found to be negative for histiocytic (MAC 387, antitrypsin), T-cell (CD3), and B-lymphocyte (immunoglobulin light chains, Ly 5/CD45R) markers, and positive for glial fibrillary acidic protein, vimentin, and S-100. Based on the immunohistochemical results, the diagnosis was modified to malignant peripheral nerve sheath tumor (PNST). Six months after surgery, the cat was reported to be well and had no evidence of metastasis. PNSTs are rare tumors in cats, and are considered as synonymous with schwannomas, neurofibrosarcomas, and hemangiopericytomas. In this cat, the plasmacytoid and pleomorphic appearance of the PNSTcells in cytologic and histologic specimens was unusual, and made it difficult to reach an accurate diagnosis without immunocytochemistry.

Animals↗

PubMedDate not supplied

Clinical signs of tumors affecting the rostral cerebrum in 43 dogs.

The clinical and pathologic features of 43 dogs with neoplasia of the rostral cerebrum were reviewed. Primary brain tumors included meningioma, astrocytoma, neuroblastoma, oligodendroglioma, and ependymoma. Other tumors that secondarily affected these areas included solitary hemangiosarcoma, nasal neuroendocrine carcinoma, nasal cell adenocarcinoma, nasal squamous cell carcinoma, and nasal neurofibrosarcoma. Older dogs were usually affected (mean, 10 years), and meningioma was the most frequent tumor type. Thirty-one dogs (72% of total) had a late-onset (greater than 5 years of age) of either generalized seizures or behavior abnormalities, or both, with an initially normal neurologic examination. In these 31 dogs, a mean time of 78 days (range, 2 to 400 days) elapsed from the onset of seizures or behavior change to the detection of a persistently abnormal neurologic examination. In all 43 dogs, the time from the detection of neurologic deficits to death or euthanasia and necropsy ranged from 1 to 63 days (mean, 13 days). On the basis of this review, it appears that dogs with late-onset seizures or behavior change, or both, should be suspected of having tumors involving the rostral cerebrum, despite the absence of persistent neurologic deficits commonly associated with cerebral tumors. Further, the onset of abnormalities in the neurologic examination and the time of death seem to occur within predictable time periods.

Adenocarcinoma↗

PubMed1989

Lymphoid and mesenchymal tumors in transgenic mice expressing the v-fps protein-tyrosine kinase.

src, abl, and fps/fes are prototypes for a family of genes encoding nonreceptor protein-tyrosine kinases. The oncogenic potential of the v-fps protein-tyrosine kinase was investigated by introduction of the gag-fps coding sequence of Fujinami sarcoma virus into the mouse germ line. Transgenic mice with v-fps under the transcriptional control of a 5' human beta-globin promoter (GF) or with both 5' and 3' beta-globin regulatory sequences (GEF) were viable. Unexpectedly, both GF and GEF transgenes were expressed in a wide variety of tissues and induced a spectrum of benign and malignant tumors. These tumors, which included lymphomas, thymomas, fibrosarcomas, angiosarcomas, hemangiomas, and neurofibrosarcomas, developed with various frequencies after latent periods of 2 to 12 months. The majority of lymphoid neoplasms appeared to be of T-cell origin and were monoclonal, as judged by rearrangements of the T-cell receptor beta or immunoglobulin genes. Some tissues that expressed the v-fps oncogene, such as heart, brain, lung, and testes, developed no malignant tumors. The v-fps protein-tyrosine kinase therefore has a broad but not unrestricted range of oncogenic activity in cells of lymphoid and mesenchymal origin. The incomplete penetrance of the neoplastic phenotype and the monoclonality of lymphoid tumors suggest that tumor formation in v-fps mice requires genetic or epigenetic events in addition to expression of the P130gag-fps protein-tyrosine kinase.

Animals↗

PubMed1981

Tumours involving the brachial plexus in seven dogs.

Seven cases with tumours localised in the brachial plexus are described. The main clinical features were a progressive lameness in one forelimb with marked muscle atrophy and very obvious but non-localizable pain. A palpable lump in the axilla was present in less than half the cases. Ancillary aids contributed little in the diagnosis except for electrophysiology which gave evidence of neural damage at an early stage of the disease and as such may be the most useful aid to an early diagnosis. Two different pathological entities were observed, the first where the tumour, primarily of neural origin (usually a neurofibrosarcoma), arose within the nerves themselves and the second where the tumours arose in adjacent tissue and involved the plexus by local infiltration. In all cases the prognosis was hopeless because of local infiltration of the tumour and metastases. In the early stages accurate diagnosis can be difficult in the absence of a mass but the possibility should be considered in any case where chronic lameness with obvious non-localizable pain is present in one forelimb.

Animals↗

PubMed1987

Spinal and paraspinal neurofibromatosis: surface coil MR imaging at 1.5 T1.

Twelve patients with neurofibromatosis affecting the spine and paraspinal regions were examined with magnetic resonance (MR) imaging performed using a 1.5-T magnet, surface coils, and spin-echo pulse sequences. In all 11 patients with neurofibromas, these tumors had slightly greater signal intensity than muscle on T1-weighted images and markedly increased signal intensity on T2-weighted images. In seven patients central areas of decreased signal intensity within the neurofibromas were demonstrated on T2-weighted images. In one patient these central areas corresponded pathologically to relatively fibrous components of the tumors surrounded by myxoid matrix. Five patients had extensive plexiform neurofibromatosis, one had lateral meningoceles, one had a neurofibrosarcoma, three had extradural spinal cord compression, and two had cystic intramedullary tumors of the spinal cord. The ability to obtain high-resolution, multiplanar images in a noninvasive fashion makes surface coil MR imaging an ideal technique for evaluating neurofibromatosis involving the spinal cord, spinal canal, and paraspinal soft tissues.

Humans↗

PubMed1992

Peripheral (extracranial) nerve tumors: correlation of MR imaging and histologic findings.

Magnetic resonance (MR) images of 16 peripheral nerve tumors (14 patients) were correlated with histopathologic appearance. Thirteen patients had surgically proved neuro-fibromatosis. There were 10 neurofibromas, four schwannomas, and two neurofibrosarcomas. Seven of the 10 neurofibromas showed a target pattern of increased peripheral signal intensity and decreased central signal intensity on T2-weighted images. This pattern appeared to correspond to a distinctive zonal histologic appearance that was found only in the neurofibromas. This pattern was not seen on MR images of the other peripheral tumors.

Adolescent↗

PubMedDate not supplied

Morphologic characterization of spontaneous nervous system tumors in mice and rats.

Spontaneous rodent nervous system tumors, in comparison to those of man, are less well differentiated. Among the central nervous system (CNS) tumors, the "embryonic" forms (medulloblastoma, pineoblastoma) occur both in rodents and humans, whereas the human "adult" forms (gliomas, ependymomas, meningiomas) have fewer counterparts in rodents. In general, the incidence of spontaneous CNS tumors is higher in rats (>1%) than in mice (>0.001%). A characteristic rat CNS tumor is the granular cell tumor. Usually it is associated with the meninges, and most meningeal tumors in rats seem to be totally or at least partly composed of granular cells, which have eosinophilic granular cytoplasm, are periodic acid-Schiff reaction (PAS)-positive, and contain lysosomes. Such tumors are frequently found on the cerebellar surface or at the brain basis. Rat astrocytomas are diffuse, frequently multifocal, and they invade perivascular spaces and meninges. The neoplastic cells with round to oval nuclei and indistinct cytoplasm grow around preexisting neurons, producing satellitosis. In large tumors, there are necrotic areas surrounded by palisading cells. Extensive damage of brain tissue is associated with the presence of scavenger cells that react positively with histiocytic/macrophage markers. The neoplastic astrocytes do not stain positively for glial fibrillary acidic protein; they probably represent an immature phenotype. In contrast to neoplastic oligodendroglia, they bind the lectin RCA-1. Astrocytomas are frequently located in the brain stem, especially the basal ganglia. Rat oligodendroglial tumors are well circumscribed and frequently grow in the walls of brain ventricles. Their cells have water-clear cytoplasm and round, dark-staining nuclei. Atypical vascular endothelial proliferation occurs, especially at the tumor periphery. Occasionally in the oligodendrogliomas, primitive glial elements with large nuclei occur in the form of cell groups that form rows and circles. Primitive neuroectodermal tumors of rats, such as pineal tumors or medulloblastomas, appear to have features similar to those found in man. In mice, the meningeal tumors are mostly devoid of granular cells and the astrocytomas are similar to those occurring in rats, whereas spontaneous oligodendrogliomas are observed extremely rarely. Tumorlike lesions, such as lipomatous hamartomas or epidermoid cysts, are occasionally encountered in the mouse CNS. It is suggested that we classify rodent CNS lesions as "low grade" and "high grade" rather than as "benign" and "malignant." The size of CNS tumors is generally related to their malignancy. Tumors of the peripheral nervous system are schwannomas and neurofibromas or neurofibrosarcomas consisting of Schwann cells, fibroblasts, and perineural cells. Well-differentiated schwannomas are characterized by S-100 positivity and the presence of basement membrane. They show either Antoni A pattern with fusiform palisading cells or Antoni B pattern, which is sparsely cellular and has a clear matrix. The rat develops specific forms of schwannomas in the areas of the submandibular salivary gland, the external ear, the orbit, and the endocardium. Spontaneous ganglioneuromas occur in the rat adrenal medulla or thyroid gland. Compared to experimentally induced neoplasms, the spontaneous tumors of the rodent nervous system are poor and impractical models of human disease, although they may serve as general indicators of the carcinogenic potential of tested chemicals.

Animals↗

PubMed1976

Neoplasms of mesenchymal origin in the spinal cord and nerve roots of three dogs.

Clinicopathological findings of three different neoplastic conditions involving the spinal cord and nerve roots in three dogs are described. One sarcomatous intra-medullary tumor closely associated with the vasculature was classified as a reticulum cell sarcoma of the spinal cord. The second case had massive and widespread neoplastic proliferation of reticulohistiocytic cells around the perineurinal vessels of many spinal nerve roots. The process was classified as a primary neoplastic reticulosis of the spinal roots. Multiple highly malignant tumors, infiltrating the spinal cord were found in one thoracic and several lumbosacral spinal nerve roots in the third case. These were considered to be anaplastic neurofibrosarcomas because of high collagen content, intact nerve fibers, whorl formation, and the tendency to palisade in some areas.

Animals↗

PubMed2002

Antiangiogenesis in neurofibromatosis 1.

Antiangiogenesis therapy has become a potentially promising tool to inhibit tumor growth by targeting an essential yet untransformed tissue component. Identifying the factors involved and understanding the mechanisms required for tumor angiogenesis will facilitate efficient and specific targeting. In neurofibromas, tumor growth is facilitated by a genetically and cytologically diverse mixture of cell types, including Schwann cells, fibroblast, mast cells, and neurons where nf-/- Schwann cells are most likely the tumorigenic cell type. The matrix forming nf+/- cells may provide a permissive environment, facilitating tumor development, perhaps by providing landscaping factors such as the angiogenic molecules fibroblast growth factor-2, platelet-derived growth factor, endothelial growth factor, vascular endothelial growth factor, and midkine, which have been detected in neurofibromas. Systemic overexpression of specific factors such as midkine owing to loss of one nf allele might further lower the overall threshold for tumorigenesis and development of a tumor vasculature. Targeting these heparin-binding growth factors might inhibit not only angiogenesis but also proliferation of tumor cells because most of these factors also stimulate proliferation of neurofibroma-derived Schwann cells. We discuss the role of specific secreted molecules for angiogenesis in tumors of neurofibromatosis 1 and possible Approaches for their targeting. Furthermore, results are discussed that demonstrate the efficacy of antiangiogenesis targeting to inhibit growth of neurofibrosarcomas in experimental animal models.

Alleles↗

PubMed1988

Neoplasms of the vagus nerve.

A tumor of the vagus nerve often is discovered unexpectedly at operation. We report 36 of these rare neoplasms in 35 patients. The majority of the tumors presented as a mass in the upper cervical or parapharyngeal region. Usually the mass was asymptomatic. The following types and frequencies of neoplasms of the vagus nerve were noted: paragangliomas, 50%; neurilemmomas, 31%; neurofibromas, 14%; and neurofibrosarcomas, 6%. Surgical resection, with preservation of the vagus nerve when possible, is the treatment of choice. The clinical features, diagnosis, management, and prognosis of the tumors are presented. Special problems that occur with vagal neoplasms include postoperative dysfunction, catecholamine secretion, and intracranial or skull-base extension.

Adolescent↗

PubMed1994

Otologic manifestations of neurofibromatosis.

A chart review of 440 patients with neurofibromatosis seen at the Children's Hospital of Philadelphia Neurofibromatosis Clinic between 1980 and 1991 was performed to look for otologic manifestations. Neurofibromatosis 1 was present in 434 patients, and neurofibromatosis 2 was present in 6. A total of 31 otologic abnormalities related to neurofibromatosis were found in 28 patients. Neurofibromas of the external ear, including the pinna and the external auditory canal, were the most common finding. Middle ear neurofibromas were found in two patients. Acoustic neuromas were seen exclusively in patients believed to have neurofibromatosis 2. One cerebellopontine angle neurofibrosarcoma was found in a patient with neurofibromatosis 1.

Adolescent↗

PubMed1983

CT of sarcomatous degeneration in neurofibromatosis.

Neurofibromatosis is a relatively common disorder that often involves many organ systems. One of the least understood aspects of this malady is a well documented potential for sarcomatous degeneration of neurofibromas. The inability to identify patients at risk and the lack of noninvasive screening methods for symptomatic patients often leads to late diagnosis. In six of seven subsequently proven neurofibrosarcomas, CT demonstrated low-density areas that histopathologically appeared to be due to necrosis, hemorrhage, and/or cystic degeneration. The density differences within these sarcomas were enhanced by the intravenous administration of iodinated contrast agents.

Adolescent↗

PubMed2006

Forelimb amputation for treatment of a peripheral nerve sheath tumor in an African pygmy hedgehog.

CASE DESCRIPTION: A 6-year-old female African pygmy hedgehog was evaluated because of a mass of unknown duration on the lateral aspect of the right shoulder region. CLINICAL FINDINGS: A fine-needle aspirate of the mass was collected for cytologic examination; findings were consistent with a spindle cell tumor. A CBC, plasma biochemical analyses, and whole-body radiography revealed no other abnormalities. TREATMENT AND OUTCOME: An initial surgery performed in an attempt to remove the mass with preservation of the limb failed in that tumor-free surgical margins were not obtained. Histologically, the mass was identified as a peripheral nerve sheath tumor (neurofibrosarcoma). A second surgery to amputate the forelimb was successful. After 1 year, there had been no further development of peripheral nerve sheath tumor at this or other sites. CLINICAL RELEVANCE: In African pygmy hedgehogs, potential differential diagnoses for a subcutaneous mass should include peripheral nerve sheath tumor. If necessary, forelimb amputation can be performed successfully in this species with procedures modified from those used in dogs. Information gathered during the treatment and recovery of the hedgehog of this report may assist practicing veterinarians in counseling owners of hedgehogs that are undergoing forelimb amputation with regard to the course of recovery that may be expected following this procedure.

Amputation, Surgical↗

PubMed1986

Mediastinal neurogenic tumours. Early and late results of surgical treatment.

Early and late results of surgery for neurogenic mediastinal tumour were evaluated in 66 cases with a mean follow-up of 12 years. The series comprised 48 neurilemmomas, 8 neurofibromas, 7 ganglioneuromas, and neurinoma, neurofibrosarcoma and ganglioneuroblastoma each in one case. There were two early deaths (3%), one due to peroperative bleeding from the left subclavian artery and the other to acute myocardial infarction. Operative complications arose in 12 cases (18%), the most common being wound infection (3 cases). There were 17 late deaths (26.6%). In the neurilemmoma group, 3 of the 13 late deaths were related to the tumour or its treatment, and both deaths in the neurofibroma group were related to malignant transformation. Recurrence of tumour appeared in 3 of the 48 patients with neurilemmoma and also in the single patient with neurinoma as tumour classification. One neurilemmoma was a dumb-bell tumour, and operation in this case resulted in paraplegia. Malignant transformation appeared in 2 of the 8 neurofibromas 5 and 13 years postoperatively. Because of the risks of malignant degeneration and of recurrence, patients operated on for neurogenic mediastinal tumour should be carefully followed up for many years.

Adolescent↗

PubMed1982

Definitive radiation therapy for chordoma and chondrosarcoma of base of skull and cervical spine.

Proton-beam radiation therapy has been developed for the treatment of chordomas or sarcomas of bone or soft tissue that abut the central nervous system. The authors report the results of treatment of 10 patients, six with chordoma, three with chondrosarcoma, and one with a neurofibrosarcoma. Local control has been achieved for all patients (with, however, one marginal failure) with a follow-up period ranging from 2 months to 6 years. High doses of radiation, up to 76 Cobalt Gray Equivalents (CGE), have been delivered without significant morbidity. In particular, no neurological sequelae have been observed.

Adult↗

PubMed1991

Enhanced in vitro uptake and retention of 3H-tetraphenylphosphonium by nervous system tumor cells.

Photodynamic therapy is a promising treatment for human brain tumors because of the selective retention of certain compounds by tumor cells. Certain lipophilic cationic compounds, such as tetraphenylphosphonium (TPP), are selectively taken up by a variety of carcinomas. Although preferential retention of TPP has been demonstrated for the breast carcinoma cell line MCF-7, this compound had not been tested previously on cells derived from nervous system tumors. In the present study, tritiated-TPP (3H-TPP) uptake and retention for eight different cell cultures of three histologically different types of nervous system tumors was measured and the data were compared to a positive control (MCF-7) and negative controls (normal African Green monkey kidney epithelium (CV-1) and the normal human fibroblast (WI-38) cell lines). Uptake and retention characteristics could be grouped by specific pathological tumor types, but individual tumor variability was notable. Malignant astrocytoma (grade III/III glioblastoma) and malignant neurofibrosarcoma cells showed preferential uptake and retention of 3H-TPP relative to meningioma cells and normal controls. A clonogenic assay utilizing the cytotoxic lipophilic cationic compound dequalinium showed strong retainers of 3H-TPP to be more susceptible to the effects of dequalinium than weak retainers. These data demonstrate that certain human and experimental animal nervous system tumor cell lines retain lipophilic compounds possessing a delocalized positive charge. Lipophilic cationic compounds may be useful in the intraoperative delineation of tumor margins and in the photodynamic therapy of certain nervous system tumors.

Animals↗

PubMedDate not supplied

Tumors of the peripheral nervous system.

Most tumors (benign and malignant) do not arise from the nerves per se, but from the supporting cells; tumors arising from the cells of Schwann are termed schwannoma or neurilemoma--benign or malignant. Surgical extirpation is the only way of treating these tumors. Radiation therapy can offer significant palliation and prolongation of life, but no cures have been observed. Other forms of therapy (chemotherapy, immunotherapy, etc.) have little to offer at this time. Benign tumors can be treated by local surgical extirpation; malignant tumors must be radically resected, including major amputations where indicated. Certain dermatomes have a proclivity to produce tumors, and new growths (benign and malignant) proximal to the one clinically apparent can be expected in certain patients. Neurofibromatosis (von Recklinghausen's disease) is a genetic error of metabolism with a proclivity to produce multiple neurofibrosarcomas, and in about 10 percent of the patients, malignant neurilemomas. Of 100 patients with malignant neurilemomas treated by the author, 74 were considered determinate; among them, the 10-year "cure" rate was 32 percent. Patients with von Recklinghausen's disease had almost as good a 10-year survival rate as those with solitary malignant schwannoma (30 percent vs 39 percent).

Female↗