Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Analysis of 7 child cases with neurinoma of the acoustic nerve, tumors which are extremely rare in children, showed that they have characteristic clinical features. The disease was manifested at the age of 12--15 years, sometimes against the background of diffuse neurofibromatosis. The mean duration of the disease varied from 5 months to one year. The tumor was marked by rapid growth, medial variant of localization with extension in the oro-caudal direction, and rich blood supply. Hearing diminished rapidly to total deafness, and the loss was usually bilateral. The facial nerve suffered less frequently. The hypertensive-occlusive syndrome was observed with marked stem-cerebellar symptoms and protein-cellular dissociation in the cerebrospinal fluid, which suggests tumor of the cerebellum. Ventriculography with maiodil emulsion and vertebral angiography helped in making a precise diagnosis. Radical removal of the tumor proved very difficult because it was large and intimately connected with the brain stem.
 ...
PMID:[Neurinomas of the auditory nerve in children]. 9 32

A 60-year-old white woman with generalized neurofibromatosis and multiple melanocytic hamartomas of the iris developed an unusual choroidal mass, with secondary sensory retinal separation in the left eye. Ophthalmoscopically the tumor had a peculiar donut configuration that was caused by a large focus of central necrosis within a spindle B melanoma.
 ...
PMID:Malignant melanoma of the choroid in neurofibromatosis. 10 2

Neurofibromatosis associated with secondary neoplasms is discussed. Neurosarcomas are the most common and most malignant secondary tumors in neurofibromatosis. Any sudden growth in a peripheral tumor in this disease should alert the physician to the possibility of malignant change and the need for biopsy. Only by early diagnosis and adequate excision can some of these patients be saved.
 ...
PMID:Neoplastic complications of neurofibromatosis. 10 88

Endocrine disturbances have been studied in 4 children with neurofibromatosis von Recklinghausen, aged 5.0-10.5 years. Hypothalamic precocious puberty was seen in three boys; growth hormone deficiency was diagnosed in a girl. After TRH stimulation one boy and the girl showed a diminished TSH-response. Another boy showed a relatively high basal level of TSH and an elevated TSH-response to TRH. All children were euthyroid. Two boys had a hyperprolactinemia even under basal conditions; the one with elevated TSH-response showed an excessive response of prolactin as well. In all our patients a suprasellar tumor caused the endocrine disorders described.
 ...
PMID:[Endocrine disorders in children with neurofibromatosis von Recklinghausen (author's transl)]. 10 38

The classical abnormalities of von Recklinghausen disease (VRD) or neurofibromatosis are well recognized. However, vertebral scalloping and gauge defects of the anterior and lateral aspects have not been reviewed, to the authors' knowledge. Case reports of 3 patients with vertebral scalloping are presented. The classical posterior scalloping from dural ectasia is also discussed. These concavities can occur either in isolation, or contiguous to a tumor. Recognition of them can aid in the diagnosis and evaluation of patients with suspected VRD.
 ...
PMID:Vertebral scalloping in neurofibromatosis. 10 40

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.
 ...
PMID:Neurofibromatosis and optic glioma: clinical and morphological correlations. 11 90

A case report with simultaneous occurrence of neurofibromatosis, incomplete branchial arch syndromes, a branchial cleft cyst and a pseudocyst in connection with a vascular hamartoma anterior to the right ear of a 38-year-old woman is presented. A possible common pathogenesis of the vascular hamartoma and the incomplete branchial arch syndromes as well as that of the neurofibromatosis is suggested. The pseudocyst is interpreted as a branchial cleft cyst showing inflammatory changes due to a pharyngitis shortly before the preauricular tumor appeared.
 ...
PMID:Incomplete branchial arch syndromes, branchial cleft cyst and vascular hamartoma in a patient with multiple neurofibromatosis. 12 Aug 62

A 6-year-old girl had neurofibromatosis and hepatocellular carcinoma (hepatoma). Although neurofibromatosis is a common disorder, hepatic cancers are rare in childhood. Hepatic cancer, Wilms' tumor, and adrenocortical neoplasia are all seen with increased frequency in association with certain congenital anomalies, particularly with hemihypertrophy and various hamartomas. Neurofibromatosis, a hamartomatous disorder, is also seen with these congenital anomalies and it is reported with an unusually high incidence in patients with Wilms' tumor. We therefore suggest that these disorders may be related.
 ...
PMID:Hepatoma in a child with neurofibromatosis. 21 87

The diagnosis and treatment of tumors of the skeleton of the hand are reviewed. Benign enchondromas were found to be the most common. Primary osteogenetic sarcoma was rare as was malignant degeneration in initially benign tumors and in the bony manifestations of systemic disease such as neurofibromatosis or hyperparathyroidism. Tumors were more common in phalanges and metacarpals than in the carpus and showed their highest incidence in the 4th decade. Diagnosis was aided by angiography and bone-scanning. Some difficulties were encountered in pathological examination. Treatment involved curettage and cancellous grafting in small tumors, en bloc resection followed by osteosynthetic stabilisation in larger instances and radical ablation followed by chemotherapy in malignancy.
 ...
PMID:[Diagnostic and therapeutic problems in tumors of the bones of the hand]. 29 52

Acoustic neuroma has always been reported to accompany neurofibromatosis in patients with enlargement of the internal auditory canal. The authors describe a case in which the internal auditory canal was enlarged but there was no tumor. This may represent a normal variant or a manifestation of the bony dysplasia of neurofibromatosis.
 ...
PMID:Internal auditory canal enlargement in neurofibromatosis without acoustic neuroma. 40 81


1 2 3 4 5 6 7 8 9 10 Next >>