Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dyschondroplasia is a mesodermal dysplasia with typical skeletal malformations of varying extent and severity. The combination with other malformations has led to numerous disease entities (Maffucci's syndrome, M. Kast, M. Ollier) that should be regarded as different forms of the same syndrome. The importance of the rare disease lies in the high risk of sacomatous transformation of the skeletal lesions and furthermore in an increased risk of development of other tumours. Involvement of the central nervous system is possible through lesions of the bones of the skull or through primary intracranial tumours. The present report concerns a patient with dyschondroplasia complicated by the occurrence of a cerebral glioma and is the third reported case verifed by biopsy.
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PMID:[Dyschondroplasia with glioma of the brain. Third histologically verified case (author's transl)]. 42 May 57

We present a 24-year-old patient with multiple chondromas of both hands, the pelvis, the left leg, and an associated brain stem glioma. There was no evidence of hemangioma or dyschromia, and the condition was diagnosed as Ollier's disease, a special type of dyschondroplasia like Maffucci's syndrome and Kast's disease. An increased overall risk for development of malignant skeletal and nonskeletal tumors is associated with Maffucci's syndrome. The risk of malignant degeneration is lower in Ollier's disease. A glioma in the pons and the right lobe of the cerebellum was found in this patient. The literature describes an association with gliomas in only 12 cases of dyschondroplasia and an infratentorial localization in just one case. Signs of malignancy were histologically confirmed in 7 cases without significant preponderance of any one type. Our patient had a low-grade brain stem astrocytoma with fibrillar and gemistocytic components. A stereotactic serial biopsy made it possible to rule out malignant degeneration. Stereotactic brain tumor biopsy as a routine neurosurgical procedure is particularly valuable for deep space-occupying processes and forms the basis for therapy. In the present case, irradiation was not recommended.
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PMID:A case of dyschondroplasia associated with brain stem glioma: diagnosis by stereotactic biopsy. 223 48