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

To determine the frequency and natural history of tumors of the optic nerves and chiasm in patients with neurofibromatosis type 1, we obtained computed tomographic scans of 65 children who had no known visual or ocular abnormalities before their initial evaluation. Optic gliomas were detected in 10 children (15%). The median age of children with gliomas was 4.3 years (mean 5.8 years, range 9 months to 21 years). Three children (30%) had isolated, unilateral tumors, three (30%) had bilateral tumors, and four (40%) had involvement of the optic chiasm and of one or both nerves. Definite abnormalities of vision were found in only two children (20%). Five additional children were referred to the clinic after evaluation of ophthalmologic complaints led to the diagnosis of neurofibromatosis type 1: three had unilateral exophthalmos and two had plexiform neurofibromas of the eyelid with associated glaucoma. Ipsilateral optic gliomas were found in all five children; one child also had a contralateral tumor. Optic gliomas are commonly identified in young children with neurofibromatosis type 1 who have no ocular or visual abnormalities. Optic nerve gliomas may be associated with plexiform neurofibromas of the eyelid and glaucoma.
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PMID:Optic gliomas in children with neurofibromatosis type 1. 249 36

The authors report one case of Von Recklinghausen's disease localized to the temporomandibular joint capsule and unilaterally associated with other cephalic manifestations. Although no other study, notably a CAT-scan, showed this tumor localization, surgical exploration was imperative and led to exeresis of the lesion which proved to be the treatment of choice.
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PMID:[Temporomandibular joint dysfunction and neurofibromatosis. Apropos of a case]. 249 8

A case of intracerebral multifocal gliomas with von Recklinghausen's disease is reported. A 12-year-old boy was admitted to our hospital with an episode of convulsive attacks which were uncontrolled by anticonvulsants. CT scan and MRI revealed small well circumscribed tumors in the left frontal cortex and left parietal subcortex. Both of the tumors revealed low density in plain CT scan and low intensity in T1 weighted MRI. The vascularity of these tumors was poor in cerebral angiography. In other words these tumors were of a similar nature. The histology of the frontal tumor, which was totally removed surgically, showed typical pilocytic astrocytoma. The other tumor in the left parietal subcortex must also be included in the category of low grade glioma. In the sixth month after the operation, we could find neither recurrence of the frontal glioma nor enlargement of the parietal tumor, on CT and MRI findings. Immunohistochemically, the outer portion of the Rosenthal fiber in this tumor was positive for GFAP and S-100 protein, but the inner portion was negative, because the GFAP and S-100 protein there had degenerated. The cytoplasm of this tumor's cell was abundant with mitochondria and Golgi's bodies compared to the fibrillary astrocytoma. This case may be the first case of multifocal gliomas in the same cerebral hemisphere. We suggest that multifocal gliomas grow naturally, and over the years, tumors combine with each other and finally constitute a large type diffuse glioma.
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PMID:[Multifocal gliomas in cerebral hemisphere associated with von Recklinghausen's disease: case report]. 249 19

A case of neurofibromatosis is reported in a patient who was initially seen with obstructive jaundice caused by a carcinoid tumor originating from the ampulla of Vater. An extensive review of the literature suggests that patients with von Recklinghausen's disease are at significant risk for periampullary neoplasms of neural-crest and non-neural-crest origin. The tendency of those tumors to arise from the ampulla of Vater has diagnostic and therapeutic implications.
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PMID:Periampullary neoplasms in von Recklinghausen's disease. 251 Mar 33

Retroperitoneal neural sheath tumors are a rare clinical entity with a variable and nonspecific presentation, whose accurate preoperative diagnosis often can be difficult. Since July 1984, 9 retroperitoneal neural sheath tumors, including 3 benign schwannomas, 3 malignant schwannomas and 3 neurofibromas, were evaluated at our institution. Preoperative evaluation included a computerized tomography scan in all patients and magnetic resonance imaging in 4. Magnetic resonance imaging offered better resolution and anatomical definition in certain cases. Preoperative computerized tomography-guided needle biopsy, performed in 3 patients, yielded inaccurate or inconclusive results. The 6 patients with surgically resected benign schwannomas and neurofibromas had no local recurrences and all 6 had no evidence of disease (mean followup 17.3 and 14 months, respectively). Malignant tumors, especially when associated with von Recklinghausen's disease, offered a poor prognosis. Surgical considerations include complete tumor excision with free margins of resection and proper pathological evaluation to determine biological potential.
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PMID:Retroperitoneal neural sheath tumors: Cleveland Clinic experience. 251 39

A case of schwannoma of the larynx in a 29-year-old woman with Recklinghausen's disease is reported. The tumor was located in the arytenoid region. The patient complained of disturbed swallowing, aphonia, and dyspnea. The tumor was endoscopically removed. The light-microscopic and immunohistochemical findings are presented.
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PMID:[Schwannoma of the larynx in Recklinghausen disease. Differential diagnostic considerations and immunohistochemical detection]. 251 15

Neurofibromatosis may involve every organ. Manifestations vary greatly and different kind of neoplasia may be associated with excessive frequency; it's the case of pheochromocytoma that, as a benign tumor, coexists in 10%. Description of coexistency as a malignant tumor is a rarity; therefore, we think it's interesting to publish our experience. Nevertheless the association of Von Recklinghausen's disease with hypertension is not always dependent of that kind of association.
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PMID:[Von Recklinghausen neurofibromatosis and malignant pheochromocytoma: presentation of 3 cases]. 251 55

From 1975 to 1988 seventeen patients with neurofibromatosis type 1 and a disfiguring facial plexiform neurofibroma (FPN) were investigated. The FPN was left-sided in 13 patients. It was orbital/periorbital in 4, lower facial in 7 and involved the whole face in 6 subjects. Neuroimaging (n = 13) revealed a tumor (of optic pathways or basal ganglia) in 8, an ipsilateral middle cranial fossa arachnoid cyst in 2, multiple areas of high signal intensity (in T2-weighted magnetic resonance imaging) in 1, and normal findings in 2 patients. In NF-1 patients with FPN there seems to be a high incidence of intracranial tumors and possibly of arachnoid cysts. Our observation has to be confirmed in a larger patient series.
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PMID:Intracranial abnormalities associated with facial plexiform neurofibromas in neurofibromatosis type 1. 251 8

We report the immunohistochemical and ultrastructural features of a case of duodenal carcinoid (somatostatinoma) combined with cutaneous-type von Recklinghausen's disease in a 65-year-old woman. The duodenal tumor located at the 2nd portion was composed of tumor cells arranged in a trabecular, glandular or nest-like pattern, occasionally associated with eosinophilic globules in the glandular structures. The tumor cells mostly showed strong immunoreactivity with anti-somatostatin antibody. Electron microscopy revealed that every tumor cell contained intracytoplasmic granules with electron-dense cores, 100-300 nm in size, in varying numbers, and intracytoplasmic lumina were rarely detected. The present case including identical cases reported so far suggest that the disease entity is likely to be a combination of duodenal somatostatinoma and von Recklinghausen's disease and/or phaeochromocytoma. This is the tenth case of duodenal carcinoid associated with von Recklinghausen's disease and/or phaeochromocytoma in the world literature and the third case reported as duodenal somatostatinoma.
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PMID:Duodenal carcinoid (somatostatinoma) combined with von Recklinghausen's disease. A case report and review of the literature. 254 98

A 36-year-old man was admitted with cough and sputum. He had cafe-au-lait spots and multiple subcutaneous neurofibromas. Chest X-ray revealed multiple emphysematous bullae in bilateral upper lobes and a tumor in the bulla of the right upper lobe. Needle aspiration biopsy of the tumor showed small cell carcinoma. Although chemotherapy and radiation resulted in decrease in tumor size, it subsequently increased in size and he died 11 months after admission. Including this case there have been 7 reports of Recklinghausen's disease associated with multiple lung cysts and 8 reports with lung cancer in Japan. However, cases with lung cysts and cancer are very rare. The cancer of this case was considered to be associated with emphysematous bulla rather than Recklinghausen's diseases.
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PMID:[A case of Recklinghausen's disease associated with multiple emphysematous bullae and lung cancer]. 255 28


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