[A case of schwannomatosis--clinical, pathological and biochemical studies]. 1991

A Matsuo, and I Tooyama, and I Akiguchi, and J Kimura, and M Kameyama
Department of Neurology, Faculty of Medicine, Kyoto University.

A case of schwannomatosis is described, including clinical, pathological and biochemical features. A 16-year-old male patient was admitted because of multiple subcutaneous tumors without family history. No café au lait spots were found. Magnetic resonance images (MRI) revealed multiple tumors of cranial and spinal nerves. The tumors in the scalp, right forearm and left spinal nerve at the level of C5 were excised out surgically. The pathological study of all tumor specimens showed a typical appearance of schwannoma with Antoni A and B tissues but not that of neurofibroma. Electrophoretic study of the extract from the tumor detected a basic protein at a molecular weight of 18.5 KD, which has been reported to be a tumor marker protein of benign schwannoma. From these findings, this patient had the features of schwannomatosis, clearly distinguished from those of neurofibromatosis.

UI MeSH Term Description Entries
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D008970 Molecular Weight The sum of the weight of all the atoms in a molecule. Molecular Weights,Weight, Molecular,Weights, Molecular
D009442 Neurilemmoma A neoplasm that arises from SCHWANN CELLS of the cranial, peripheral, and autonomic nerves. Clinically, these tumors may present as a cranial neuropathy, abdominal or soft tissue mass, intracranial lesion, or with spinal cord compression. Histologically, these tumors are encapsulated, highly vascular, and composed of a homogenous pattern of biphasic fusiform-shaped cells that may have a palisaded appearance. (From DeVita Jr et al., Cancer: Principles and Practice of Oncology, 5th ed, pp964-5) Neurinoma,Schwannoma,Schwannomatosis, Plexiform,Neurilemoma,Neurilemmomas,Neurilemomas,Neurinomas,Plexiform Schwannomatoses,Plexiform Schwannomatosis,Schwannomas
D009455 Neurofibroma A moderately firm, benign, encapsulated tumor resulting from proliferation of SCHWANN CELLS and FIBROBLASTS that includes portions of nerve fibers. The tumors usually develop along peripheral or cranial nerves and are a central feature of NEUROFIBROMATOSIS 1, where they may occur intracranially or involve spinal roots. Pathologic features include fusiform enlargement of the involved nerve. Microscopic examination reveals a disorganized and loose cellular pattern with elongated nuclei intermixed with fibrous strands. (From Adams et al., Principles of Neurology, 6th ed, p1016) Neurofibromas
D003390 Cranial Nerve Neoplasms Benign and malignant neoplasms that arise from one or more of the twelve cranial nerves. Cranial Neuroma, Benign,Benign Cranial Nerve Neoplasms,Benign Cranial Nerve Tumors,Cranial Nerve Neoplasms, Benign,Cranial Nerve Neoplasms, Malignant,Cranial Nerve Tumors, Benign,Cranial Nerve Tumors, Malignant,Malignant Cranial Nerve Neoplasms,Malignant Cranial Nerve Tumors,Neoplasms, Cranial Nerve,Neoplasms, Cranial Nerve, Benign,Neoplasms, Cranial Nerve, Malignant,Tumors, Cranial Nerve, Benign,Tumors, Cranial Nerve, Malignant,Benign Cranial Neuroma,Benign Cranial Neuromas,Cranial Nerve Neoplasm,Cranial Neuromas, Benign,Neoplasm, Cranial Nerve,Neuroma, Benign Cranial,Neuromas, Benign Cranial
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D004586 Electrophoresis An electrochemical process in which macromolecules or colloidal particles with a net electric charge migrate in a solution under the influence of an electric current. Electrophoreses
D004676 Myelin Basic Protein An abundant cytosolic protein that plays a critical role in the structure of multilamellar myelin. Myelin basic protein binds to the cytosolic sides of myelin cell membranes and causes a tight adhesion between opposing cell membranes. Golli-MBP1 Protein,Golli-MBP2 Protein,HOG5 Protein,HOG7 Protein,MBP1 Protein,MBP2 Protein,MBP3 Protein,MBP4 Protein,Myelin Basic Protein, 17.2 kDa Isoform,Myelin Basic Protein, 18.5 kDa Isoform,Myelin Basic Protein, 20.2 kDa Isoform,Myelin Basic Protein, 21.5 kDa Isoform,Myelin Basic Protein, Isoform 1,Myelin Basic Protein, Isoform 2,Myelin Basic Protein, Isoform 3,Myelin Basic Protein, Isoform 4,Myelin Basic Protein, Isoform 5,Myelin Basic Protein, Isoform 6,Myelin Basic Protein, Isoform 7,Golli MBP1 Protein,Golli MBP2 Protein
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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