[Werner's syndrome associated with meningioma: case report]. 1988

M Kawauchi, and H Gibo, and S Kobayashi, and K Sugita
Department of Neurosurgery, Shinshu University Faculty of Medicine, Matsumoto, Japan.

We present two cases of Werner's syndrome associated with intracranial meningioma. Characteristic clinical features of Werner's syndrome include short stature with slender extremities, premature senility, juvenile cataract, skin changes, a tendency to diabetes mellitus and familial occurrence. A 44-year-old female, who had been treated for diabetes mellitus, was diagnosed as having Werner's syndrome because of various characteristic features. A falx meningioma was incidentally found on CT scan, and was surgically removed. Her diabetes mellitus improved. The second case was a 28-year-old male was diagnosed as having Werner's syndrome, diabetes mellitus, juvenile cataract, together with diabetes insipidus, and liver dysfunction. He developed severe headache, gait disturbance and then became unconscious with right hemiparesis. He was found to have a parasagittal meningioma by CT scan and angiography. After removal of the tumor, diabetes mellitus, diabetes insipidus and liver dysfunction improved. The reported incidence of neoplasms associated with Werner's syndrome is about 10%. The majority of associated tumors were mesenchymal in origin. Ten meningiomas, 1 neurinoma and 2 gliomas are reported as associated tumors in the central nervous system. Most of the associated meningiomas were asymptomatic and found incidentally at autopsies or CT scans. Diabetes mellitus associated with Werner's syndrome is generally mild with high immunoreactive insulin value and is controllable by diet therapy and oral antidiabetic drugs. Daily profile of blood sugar improved after the removal of tumor in our cases. In 50 gm glucose tolerance test, tendency of delayed appearance of peak value, which is common in Werner's syndrome, was not altered in our cases. Discussion is made as to the association of Werner's syndrome with meningioma and diabetes mellitus.

UI MeSH Term Description Entries
D008297 Male Males
D008577 Meningeal Neoplasms Benign and malignant neoplastic processes that arise from or secondarily involve the meningeal coverings of the brain and spinal cord. Intracranial Meningeal Neoplasms,Spinal Meningeal Neoplasms,Benign Meningeal Neoplasms,Leptomeningeal Neoplasms,Malignant Meningeal Neoplasms,Meningeal Cancer,Meningeal Neoplasms, Benign,Meningeal Neoplasms, Intracranial,Meningeal Neoplasms, Malignant,Meningeal Tumors,Neoplasms, Leptomeningeal,Neoplasms, Meningeal,Benign Meningeal Neoplasm,Cancer, Meningeal,Cancers, Meningeal,Intracranial Meningeal Neoplasm,Leptomeningeal Neoplasm,Malignant Meningeal Neoplasm,Meningeal Cancers,Meningeal Neoplasm,Meningeal Neoplasm, Benign,Meningeal Neoplasm, Intracranial,Meningeal Neoplasm, Malignant,Meningeal Neoplasm, Spinal,Meningeal Neoplasms, Spinal,Meningeal Tumor,Neoplasm, Benign Meningeal,Neoplasm, Intracranial Meningeal,Neoplasm, Leptomeningeal,Neoplasm, Malignant Meningeal,Neoplasm, Meningeal,Neoplasm, Spinal Meningeal,Neoplasms, Benign Meningeal,Neoplasms, Intracranial Meningeal,Neoplasms, Malignant Meningeal,Neoplasms, Spinal Meningeal,Spinal Meningeal Neoplasm,Tumor, Meningeal,Tumors, Meningeal
D008579 Meningioma A relatively common neoplasm of the CENTRAL NERVOUS SYSTEM that arises from arachnoidal cells. The majority are well differentiated vascular tumors which grow slowly and have a low potential to be invasive, although malignant subtypes occur. Meningiomas have a predilection to arise from the parasagittal region, cerebral convexity, sphenoidal ridge, olfactory groove, and SPINAL CANAL. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2056-7) Benign Meningioma,Malignant Meningioma,Meningiomas, Multiple,Meningiomatosis,Angioblastic Meningioma,Angiomatous Meningioma,Cerebral Convexity Meningioma,Clear Cell Meningioma,Fibrous Meningioma,Hemangioblastic Meningioma,Hemangiopericytic Meningioma,Intracranial Meningioma,Intraorbital Meningioma,Intraventricular Meningioma,Meningotheliomatous Meningioma,Microcystic Meningioma,Olfactory Groove Meningioma,Papillary Meningioma,Parasagittal Meningioma,Posterior Fossa Meningioma,Psammomatous Meningioma,Secretory Meningioma,Sphenoid Wing Meningioma,Spinal Meningioma,Transitional Meningioma,Xanthomatous Meningioma,Angioblastic Meningiomas,Angiomatous Meningiomas,Benign Meningiomas,Cerebral Convexity Meningiomas,Clear Cell Meningiomas,Convexity Meningioma, Cerebral,Convexity Meningiomas, Cerebral,Fibrous Meningiomas,Groove Meningiomas, Olfactory,Hemangioblastic Meningiomas,Hemangiopericytic Meningiomas,Intracranial Meningiomas,Intraorbital Meningiomas,Intraventricular Meningiomas,Malignant Meningiomas,Meningioma, Angioblastic,Meningioma, Angiomatous,Meningioma, Benign,Meningioma, Cerebral Convexity,Meningioma, Clear Cell,Meningioma, Fibrous,Meningioma, Hemangioblastic,Meningioma, Hemangiopericytic,Meningioma, Intracranial,Meningioma, Intraorbital,Meningioma, Intraventricular,Meningioma, Malignant,Meningioma, Meningotheliomatous,Meningioma, Microcystic,Meningioma, Multiple,Meningioma, Olfactory Groove,Meningioma, Papillary,Meningioma, Parasagittal,Meningioma, Posterior Fossa,Meningioma, Psammomatous,Meningioma, Secretory,Meningioma, Sphenoid Wing,Meningioma, Spinal,Meningioma, Transitional,Meningioma, Xanthomatous,Meningiomas,Meningiomas, Angioblastic,Meningiomas, Angiomatous,Meningiomas, Benign,Meningiomas, Cerebral Convexity,Meningiomas, Clear Cell,Meningiomas, Fibrous,Meningiomas, Hemangioblastic,Meningiomas, Hemangiopericytic,Meningiomas, Intracranial,Meningiomas, Intraorbital,Meningiomas, Intraventricular,Meningiomas, Malignant,Meningiomas, Meningotheliomatous,Meningiomas, Microcystic,Meningiomas, Olfactory Groove,Meningiomas, Papillary,Meningiomas, Parasagittal,Meningiomas, Posterior Fossa,Meningiomas, Psammomatous,Meningiomas, Secretory,Meningiomas, Sphenoid Wing,Meningiomas, Spinal,Meningiomas, Transitional,Meningiomas, Xanthomatous,Meningiomatoses,Meningotheliomatous Meningiomas,Microcystic Meningiomas,Multiple Meningioma,Multiple Meningiomas,Olfactory Groove Meningiomas,Papillary Meningiomas,Parasagittal Meningiomas,Posterior Fossa Meningiomas,Psammomatous Meningiomas,Secretory Meningiomas,Sphenoid Wing Meningiomas,Spinal Meningiomas,Transitional Meningiomas,Wing Meningioma, Sphenoid,Wing Meningiomas, Sphenoid,Xanthomatous Meningiomas
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D014898 Werner Syndrome An autosomal recessive disorder that causes premature aging in adults, characterized by sclerodermal skin changes, cataracts, subcutaneous calcification, muscular atrophy, a tendency to diabetes mellitus, aged appearance of the face, baldness, and a high incidence of neoplastic disease. Progeria, Adult,Adult Premature Aging Syndrome,Adult Progeria,Werner's Syndrome,Werners Syndrome,Syndrome, Werner,Syndrome, Werner's,Syndrome, Werners

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