Biomaterial Database

[Multiple brain tumors with von Recklinghausen's disease]. 1983

S Mashiyama, and T Mori, and H Seki, and M Kanno, and J Suzuki

Thirty-three cases of von Recklinghausen's disease, nine of which had multiple brain tumors, were experienced in our clinic. In this report, these nine cases were reported and discussed, especially representative two cases in detail. Nine cases had various combinations of multiple brain tumors: bilateral acoustic neurinomas in two cases, multiple neurinomas in two cases, multiple meningiomas in one case and multiple neurinomas and meningiomas in two cases. In two cases, multiple brain tumors were totally extirpated at one stage, but both died within ten months. In three cases, partial extirpation of multiple brain tumors were performed at one stage, one died thirteen years after the operation and two are alive but dependent on their families. In recent two cases, two stage operations were performed, by which tumors were successfully removed totally. These postoperative courses were good. The other two cases were treated conservatively. One is alive and working, but has severe auditory disturbance. The other died after six years from onset. Autopsy was performed in this case, which revealed multiple neurinomas in the left 3rd and bilateral 5-12th cranial nerves and numerous meningiomas. In the review of the cases with more than three brain tumors in von Recklinghausen's disease were discussed.

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
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D009378	Neoplasms, Multiple Primary	Two or more abnormal growths of tissue occurring simultaneously and presumed to be of separate origin. The neoplasms may be histologically the same or different, and may be found in the same or different sites.	Neoplasms, Synchronous,Neoplasms, Synchronous Multiple Primary,Multiple Primary Neoplasms,Multiple Primary Neoplasms, Synchronous,Synchronous Multiple Primary Neoplasms,Synchronous Neoplasms,Multiple Primary Neoplasm,Neoplasm, Multiple Primary,Neoplasm, Synchronous,Primary Neoplasm, Multiple,Primary Neoplasms, Multiple,Synchronous Neoplasm
D009456	Neurofibromatosis 1	An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).	Peripheral Neurofibromatosis,Recklinghausen Disease of Nerve,von Recklinghausen Disease,Cafe-au-Lait Spots with Pulmonic Stenosis,Molluscum Fibrosum,NF1 (Neurofibromatosis 1),Neurofibromatosis I,Neurofibromatosis Type 1,Neurofibromatosis Type I,Neurofibromatosis, Peripheral Type,Neurofibromatosis, Peripheral, NF 1,Neurofibromatosis, Peripheral, NF1,Neurofibromatosis, Type 1,Neurofibromatosis, Type I,Pulmonic Stenosis with Cafe-au-Lait Spots,Recklinghausen Disease, Nerve,Recklinghausen's Disease of Nerve,Recklinghausens Disease of Nerve,Watson Syndrome,von Recklinghausen's Disease,Cafe au Lait Spots with Pulmonic Stenosis,Neurofibromatoses, Peripheral,Neurofibromatoses, Type I,Neurofibromatosis, Peripheral,Peripheral Neurofibromatoses,Pulmonic Stenosis with Cafe au Lait Spots,Syndrome, Watson,Type 1 Neurofibromatosis,Type 1, Neurofibromatosis,Type I Neurofibromatoses,Type I, Neurofibromatosis,von Recklinghausens Disease
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D001932	Brain Neoplasms	Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain.	Brain Cancer,Brain Metastases,Brain Tumors,Cancer of Brain,Malignant Primary Brain Tumors,Neoplasms, Intracranial,Benign Neoplasms, Brain,Brain Neoplasm, Primary,Brain Neoplasms, Benign,Brain Neoplasms, Malignant,Brain Neoplasms, Malignant, Primary,Brain Neoplasms, Primary Malignant,Brain Tumor, Primary,Brain Tumor, Recurrent,Cancer of the Brain,Intracranial Neoplasms,Malignant Neoplasms, Brain,Malignant Primary Brain Neoplasms,Neoplasms, Brain,Neoplasms, Brain, Benign,Neoplasms, Brain, Malignant,Neoplasms, Brain, Primary,Primary Brain Neoplasms,Primary Malignant Brain Neoplasms,Primary Malignant Brain Tumors,Benign Brain Neoplasm,Benign Brain Neoplasms,Benign Neoplasm, Brain,Brain Benign Neoplasm,Brain Benign Neoplasms,Brain Cancers,Brain Malignant Neoplasm,Brain Malignant Neoplasms,Brain Metastase,Brain Neoplasm,Brain Neoplasm, Benign,Brain Neoplasm, Malignant,Brain Neoplasms, Primary,Brain Tumor,Brain Tumors, Recurrent,Cancer, Brain,Intracranial Neoplasm,Malignant Brain Neoplasm,Malignant Brain Neoplasms,Malignant Neoplasm, Brain,Neoplasm, Brain,Neoplasm, Intracranial,Primary Brain Neoplasm,Primary Brain Tumor,Primary Brain Tumors,Recurrent Brain Tumor,Recurrent Brain Tumors,Tumor, Brain
D002531	Cerebellum	The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills.	Cerebella,Corpus Cerebelli,Parencephalon,Cerebellums,Parencephalons
D005260	Female		Females