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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The cutaneous nodules obtained from seven patients with von Recklinghausen's neurofibromatosis were investigated by electron microscopy, and ultrastructural localization of acetylcholinesterase activity was demonstrated in the nerve fibers of this tumor for the first time using Karnovsky's thiocholine method. The enzymatic activity was mainly found in unmyelinated fibers, exactly associated with their axonal membranes, the interspace between the apposing axonal and Schwann cell membrane, and some different mesaxons, which indicated their cholinergic nature. Almost all myelinated fibers and some unmyelinated fibers did not possess the activity. The relationship between axon and Schwann cell was quite similar to that of normal peripheral nervous system, but two striking alterations of the nerves existed: One is the dissociation of unmyelinated fibers, and the other is the degenerative changes of the axon and the myelin sheath. As the evidence of schwannian proliferation, onion bulb formations and collagen pockets were observed. Some signs of fibroblastic proliferation were also found. From the present study and the review of the literature, the probable histogenesis of this disease was discussed.
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PMID:Study on the ultrastructure and acetylcholinesterase activity in von Recklinghausen's neurofibromatosis. 9 62

Von Recklinghausen's Disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. Case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "Elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.
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PMID:E.N.T. manifestations of Von Recklinghausen's disease. 10 Jun 61

Although an association between optic glioma and neurofibromatosis is well recognized, there has been no previous analysis of cases relating the locus of tumor to the presence or absence of von Recklinghausen's disease. This paper presents the results of such an analysis as well as a description of the comparative histology of optic gliomas in patients with and without neurofibromatosis. We conclude that optic gliomas in patients with neurofibromatosis present preferentially as multicentric lesions or as lesions affecting the optic nerve alone without invasion of the chiasm, that these tumors frequently differ morphologically, and that patients with neurofibromatosis and optic glioma may have a clinical course different from that of patients with optic glioma unassociated with neurofibromatosis. Previously published reports are reviewed in light of these observations.
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PMID:Neurofibromatosis and optic glioma: clinical and morphological correlations. 11 90

A series of 46 malignant schwannomas occurring in soft parts of patients having von Recklinghausen's neurofibromatosis was analyzed. The diagnosis of malignant schwannoma was based upon the occurrence of malignant spindled cells closely resembling Schwann cells in the neoplasm and the close association or origin of the malignant schwannoma in a neurofibroma (27 tumors), or a large peripheral nerve (31 tumors). Additional histologic features useful in making the diagnosis of malignant schwannoma included the arrangement of the spindled tumor cells in a whorled pattern about thin-walled, gaping blood vessels, perivascular cellular proliferation and the presence of prominent myxoid stroma containing abundant hyaluronidase-sensitive acid mucopolysaccharides. Nuclear palisading was present in only one case. Eight tumors containing both neoplastic Schwann cells and rhabdomyoblasts and five containing both neoplastic Schwann cells and rhabdomyoblasts (malignant "Triton" tumors) and five containing foci of malignant cartilage cells were included in the series. The neoplasms occurred principally in adults (median age, 34 years) and were most common in the lower extremity (18 cases) and retroperitoneum (11 cases). A mass with or without pain was the most common presenting symptom (28 cases). The median size of excised tumors was 11 cm. The malignant schwannomas were highly malignant neoplasms, causing the death of 39 patients within five years and two patients within 6--10 years after diagnosis. Only four patients were alive and free of tumor 5--15 years after diagnosis.
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PMID:Malignant Schwannoma associated with von Recklinghausen's neurofibromatosis. 15 12

Case reports of neuroblastoma revealed that some individuals are genetically predisposed and that this genetic predisposition may have other consequences. According to a mutation model, two classes of individuals could acquire neuroblastoma. One (prezygotic) was a rare class that carried a dominant gene imparting high risk of the tumor. The other (postzygotic) comprised all other individuals, each at low risk. The model related tumor incidence to germinal and somatic mutation rates and thereby carried implications for environmental modification of tumorigenesis and demographic variation in incidence. Case reports also revealed associations of neuroblastoma with congenital defects and a susceptibility to second tumors. Analogy with retinoblastoma and Wilms' tumor of the kidney suggested that these associations could result from action of a neuroblastoma gene or from chromosomal aberration. One known dominantly inherited condition, von Recklinghausen's disease, could dispose to neuroblastoma and create some associations. According to the two-mutation model, neuroblastoma may have been a single recessive gene disorder at the level of the cell. The phenomena of aganglionosis, neuroblastoma in situ, maturation of neuroblastoma to ganglioneuroma, and spontaneous regression suggested that such a neuroblastoma gene interfered with normal developmental processes. The specificities of this gene and of those for von Recklinghausen's disease and pheochromocytoma suggested that the functiof a membrane macromolecule.
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PMID:Developmental genetics of neuroblastoma. 18 2

Two brothers developed multiple primary neoplasms in childhood; one had glioblastoma and non-Hodgkin's lymphoma at age 11 years, and the other brain tumor and acute leukemia at six years. A third brother died with myelogenous leukemia at thre years, and a fourth with cyanotic congenital heart disease at 11 weeks. Each child also had at least one hamartomatous lesion of the skin. The clinical features suggested von Recklinghausen's neurofibromatosis or other inherited cancer syndrome, but laboratory studies identified no markers of susceptibility to familial neoplasia.
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PMID:Double primary cancers in 2 young sibs, leukemia in another, and dextrocardia in a fourth. 19 73

Peripheral nerve tumors comprise less than 5% of all tumors of the hand. The most common solitary nerve tumor is the neurilemmoma, which arises from the neural sheath, is well encapsulated, minimally symptomatic, and may be surgically enucleated without producing a neurological deficit. Neurofibromas may be solitary, multiple, or associated with von Recklinghausen's disease. They are usually centrally placed with nerve fibers traversing the tumor mass making it more difficult to remove the tumor without producing permanent neurological damage. Malignant tumors include neurofibrosarcomas which often are very aggressive, requiring wide excision or amputation, and the rare neuroepitheliomas. Reported nerve tumors, intraneural in location but nonneural in origin, include fibrofatty infiltration of the median and digital nerves, intraneural lipoma, hemangioma, and ganglion cysts. These lesions may be treated by decompression or excision, depending on the nature of the tumor. Four unusual cases are described.
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PMID:Nerve tumors of the hand and forearm. 19 48

Cells with spheroidal filamentous cytoplasmic bodies, distinctive by both light and electron microscopy, were found in a neoplasm arising from the sciatic nerve of a patient with von Recklinghausen's disease. Tissue fixed with formalin and embedded in paraffin for three years was deparaffinized, reprocessed, and examined with the electron microscope. The morphology of the spheroidal body cells, the close resemblance to erythrophagocytosis, and the possible significance of the changes are discussed.
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PMID:Spheroidal filamentous inclusion body cells in von Recklinghausen's disease. 20 9

Thirty of 1720 temporal bones from the Wittmaack's Collection contain nonoperated acoustic neuromas, 22 of them large tumors. Histologically, each tumor has to be considered as an individual. Most of the tumors show a mixture of Antoni type A and B. Tumor vascularization is pronounced in half the cases. Vessels of the internal acoustic meatus are found in all cases. Protein contents of the perilymphatic spaces is medium to pronounced in 23 of 30 cases in comparison to the small protein contents of the healthy side. Ganglion geniculi is invaded in 11 cases (9 von Recklinghausen's disease), Ganglion Scarpae in 26 cases (9 von Recklinghausen's disease), and Ganglion spirale in 9 cases (7 von Recklinghausen's disease). Cochlear and vestibular nerve fibers within the internal auditory meatus were affected by the tumor in 28 of 30 cases, the facial nerve only in 11 cases, among them 9 cases of von Recklinghausen's disease. Twenty-six of 30 schwannomas have a portion within the cribriform area of the cochlea fundus, which explains the limitation to radical tumor surgery without damage of the cochlear nerve. These histological findings explain the site of damage of hearing to expand between the cochlea to the auditory brain stem nuclei, and support the audiological experience that a correlation of "acoustic neuroma" and "retrocochlear lesion" is often not correct.
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PMID:Histopathology of 30 non-operated acoustic schwannomas. 42 17

Two cases of solitary neurogenic tumors of the brachial plexus unassociated with von Recklinghausen's disease are presented. One patient had a malignant schwannoma. The lesion of the other patient was benign and was diagnosed pathologically as a plexiform neurofibroma. These uncommon neurogenic tumors of the brachial plexus unassociated with von Recklinghausen's disease pose diagnostic and surgical problems. The initial clinical presentation is usually that of a painless supraclavicular mass. At the time of surgical exploration, the exact site or nerve of origin cannot always be identified. If motor loss is caused by such a tumor of the brachial plexus, it usually indicates a malignant lesion and a poor prognosis. Although wide radical excision of a malignant neurogenic tumor is indicated surgically, one of our patients had an early malignant recurrence that necessitated immediate amputation.
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PMID:Neurogenic tumors of the brachial plexus: report of two cases. 45 Feb 20


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