[Tumours of the central nervous system in biopsy and autopsy material. 1st contribution: frequency as well as age and sex distribution (author's transl)].
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The Role of prenatal age, sex, and the maternal background upon the incidence, multiplicity, and spectrum of tumors induced by ethylnitrosourea has been studied in the offspring of reciprocal hybrids of the same genotype. The first generation (F1) offspring of C57BL/6J X C3HeB/FeJ and the reciprocal hybrids were observed throughout their life-span for tumor development following single i.p. injections of ethylnitrosourea (60 microng/g) given to pregnant mothers at 12, 14, 16, or 18 days of gestation. Animals exposed to ethylnitrosourea survived on the average for only 90 weeks. They developed by that age tumors in lungs, livers, ovaries, nervous system, and forestomach. Control mice killed at 90 weeks were essentially free of tumors. The fetal age at the time of administration of the carcinogen was one of the most significant modulators of tumor development in lung, liver, ovaries, and nervous system. The sex of the animals influenced the rate of development of liver tumors, whereas maternal background affected the multiplicity of lung tumors.
A 45-year-old man, afflicted with von Recklinghausen's disease, with symptomatic schwannomas and plexiform neurofibromas involving the right maxillary antrum, a rare site for peripheral nerve tumors, is presented. After a literature review, 15 cases were considered adequate for a clinicopathologic study. Twelve schwannomas, 2 plexiform neurofibromas, 2 neurofibromas, and 1 probable malignant schwannoma were included. Epistaxis was common in tumors of the ethmoid sinus and nasal fossae, while pain was related to lesions of the maxillary sinus. Two benign schwannomas recurred and were cured by surgery alone. The importance of early diagnosis and thorough surgical excisions is emphasized, especially for those tumors associated with von Recklinghausen's disease. The difficulties involved in the diagnosis of primary malignant peripheral nerve tumors are discussed.
A patient with multiple cutaneous hemangiomas and skeletal dyschondroplasia (the Maffucci syndrome) was found to have a pituitary chromophobe adenoma, a parathyroid adenoma and two other neoplasms. The presence of two endocrine tumors suggested the syndrome of multiple endocrine adenomatosis, and raised the issue of an etiologic relationship between this disease and the Maffucci syndrome. Dyschondroplasia, however, has no known influence on the secretion of parathyroid hormone or any of the pituitary hormones. The Maffucci syndrome is associated with a high incidence of malignancy, but it involves primarily mesodermal derivatives whereas multiple endocrine adenomatosis affects tissues of ectodermal origin. The association of the two in our patient is probably fortuitous.
A congenital malignant peripheral nerve sheath tumor contained small rhabdomyosarcomatous portions in both the retroperitoneal pelvic primary and in the subpleural lung metastasis. There was no family history of Von Recklinghausen's disease. Of the 10 patients with this tumor previously reported, the youngest is 14 yr old, and eight had familial neurofibromatosis.
This is a report of three patients who presented at the Mayo Clinic over a two-year period. All were initially diagnosed as having Bell's palsy but were later found to have a malignant neoplasm causing the paralysis. Two of the patients had breast carcinoma metastases involving the mastoid portion of the facial nerve. The third patient had an adenocarcinoma of the deep lobe of the parotid that involved the facial nerve distal to the stylomastoid foramen. The course of the facial paralysis in the two patients with the metastitic breast disease was almost identical. It consisted of episodes of pain in the mastoid area. generally in the late evening or during the night, often awakening the patient from sleep. This was then followed by peripheral facial-nerve paralysis, sometimes partial and at other times complete. These episodes lasted from 10 minutes to several hours and then resolved completely. They recurred over several months. The patients were completely asymptomatic and normal on examination in the intervals between episodes of paralysis until it became permanent. Metastatic lesions causing facial paralysis are extremely rare in the literature. In those cases that have been reported, the paralysis was progressive from the start and in the vast majority of cases was either painless or associated with other aural symptoms such as otorrhea, hearing loss, and periauricular swelling. There are two unusual features of these two cases: 1. the initial presentation of a breast metastasis as a facial paralysis; in the first case there were no other metastatic lesions present at diagnosis, whereas the second patient had other, asymptomatic, metastatic nodules; and 2. the multiple, brief, recurring episodes of facial paralysis, which have not previously been reported as a mode of presentation of metastitic disease. The third patient was diagnosed as having Bell's palsy. A facial nerve decompression was performed, and the nerve apparently looked normal. The paralysis failed to resolve. He was later found to have adenocarcinoma of the deep lobe of the parotid that involved the facial nerve distal to the stylomastoid foramen. A report of only one similar case could be found in the literature. The sequence of events in these three cases emphasizes the importance of submitting a patient suspected of having Bell's palsy to a thorough otoneurologic examination.
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With contact B-scan ultrasongraphy it is possible, to determine the topographical relations of space occupieing lesions to the main orbital structures. This enables the examiner to answer three important questions: (1) In which part of the orbit the lesion is located? (2) Is the proptosis produced by a lesion separate from the muscles and the optic nerve or (3) are these structures themselves affected? Our results are compared with those made by A-scan ultrasonography. The existing range of echographical patterns known of orbital tumors is thus widened by the topographical diagnosis elicited by contact B-scan ultrasonography.
An embryonal rhabdomyosarcoma that initially was seen as a polyp in the external auditory canal underwent uncontrollable direct extension along the facial nerve through the internal auditory meatus, with encasement of the base of the brain and entire spinal cord despite radical surgery, supervoltage irradiation, and multidrug chemotherapy. This degree of massive neuraxial spread without systemic metastases appears to be unique.
Serially transplanted neurogenic tumors of experimental origin were explanted in vitro and analyzed by scanning electron microscopy. In explants of intracerebrally transplanted gliomas, small stellate cells with branching processes were observed; in transplanted tumors of the peripheral nervous system, slender bipolar cells as well as fibroblasts emerged. The stereoscopic features were compared with the picture of conventional light microscopy. The findings are consistent with the assumption of the glial derivation of CNS-tumors induced with neurotropic alkylating carcinogens. The peripheral tumors are composed of Schwann-cell like elements and fibroblasts. The significance of the results for the cytogenetic derivation of brain tumors are discussed.
Hematogenous metastases of carcinoma to dorsal root ganglia was found in 2 of approximately 500 consecutive autopsies in which a lumbar dorsal root ganglion was routinely examined microscopically. The primary tumors were poorly differentiated colonic adenocarcinoma and oat cell carcinoma of the lung, both with widespread hematogenous metastases which spared the central nervous system. No symptoms were detected clinically. In the same series of patients the sural nerve as well as the lumbar plexus were histologically sampled but no examples of distant endoneurial metastases were found. The vascular endothelium of dorsal root ganglia is fenestrated and, presumably as a consequence, provides no blood-ganglion barrier. This microvascular difference may account for the susceptibility of the ganglia to metastases when compared to nerve trunks which posses unfenestrated endothelium and blood-nerve barrier.
A retrospective histologic study of 145 consecutive autopsy cases of systemic malignant lymphomas (including lymphatic leukemias) was performed. The classification followed the Kiel Classification (Gérard-Marchant et al., 1974). There was an overall secondary CNS involvement in 26.2% of the total or in 30.4% of the non-Hodgkin's lymphomas including ALL, with intracranial lesions in 21.4 and 26.1%, respectively, and spinal epidural spread in 5.5 (5.1%). Peripheral nerve involvement was seen in almost 40% of the examined cases. Ten further cases were isolated ("primary") intracranial lymphomas without evidence of extraneural deposits or systemic lymphatic disease. The CNS complications in non-Hodgkin's lymphomas were diffuse meningeal and/or perivascular infiltration with or without invasion of the nervous parenchyma, and did not differ from those in CNS leukemia. Isolated solid mass lesions in the brain were only present in 7% of the secondary CNS lymphomas, but were seen in all instances of "primary" cerebral lymphomas. The incidence of CNS complications was highest in lymphoblastic lymphomas including ALL (39%), CLL (31%), immunocytic lymphoma (29%), less frequent in immunoblastic (18.7%), and centrocytic lymphomas (16.6%). No intracranial lesion was observed in centroblastic-centrocytic and centroblastic lymphomas which only produced epidural spread. Bone marrow involvement was present in 92.8% of the cases with secondary CNS lesions, and in 83.2% of the epidural lymphomas. Leukemic conversion, present in 44% of the total (52% with ALL), was demonstrated in 83.3% of the cases with secondary brain lesions, but was hardly combined with epidural spread. The histologic pattern of CNS lesions in non-Hodgkin's lymphomas and their frequent association with leukemic conversion suggest the importance of hematogenous dissemination rather than of direct spread from bone marrow or local manifestation in multisystem disease. Isolated ("primary") lymphomas of the CNS which are morphologically identical with the extraneural lymphomas may represent a primary, often lethal manifestation of a multisystem disease with or without secondary generalization.
Two cases of basal cell carcinoma had diffused infiltration that included not only perineural but endoneurial invasion. One patient presented with trigeminal neuralgia, and the other patient developed localized neuralgia when the tumor recurred. Nests and indian files of carcinoma cells were in the endoneurium, a region that anatomically is devoid of lymphatics. The involved nerves displayed axonal degeneration. Such intraneural invasion by basal cell carcinoma is very rare, even in the scelerosing variety of basal cell carcinoma.
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Reported is a case of epidermoid carcinoma of the lower lip that spread along the inferior alveolar nerve to the cranium. Early clinical symptoms are correlated with radiographic and histopathologic findings, and attention is again called to the fact that carcinomas of the head and neck may occasionally spread along nerve pathways.
CT findings in 50 patients with histologically verified soft tissue sarcomas are presented. In nearly all the cases the cross-sectional view and the higher resolution of density make it possible to determine exactly location of the tumors, their size and relationship to adjacent structures. Thus CT opens a new dimension, which is of great value for staging and therapy treatment planning. The high rank of CT in the follow-up after therapy and the detection of recurrent tumor is demonstrated. The contribution of CT, however, to the anatomic characteristics and its prospective value with the regard to soft tissue tumor is less important. Further limits of the method are discussed.
A case of adenoid cystic carcinoma of the mandible in a 47-year-old woman metastasized to the Gasserian ganglion probably through the perineural space of the inferior alveolar nerve. The patient was treated by irradiation after excision of the tumor from the ganglion. She is currently an outpatient in the oral and maxillofacial surgery department; two years and seven months after the first examination, there is no sign of recurrence or metastasis of adenoid cystic carcinoma.