myelin-basic-protein and Spinal-Cord-Neoplasms

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.

Topics: Adolescent; Biomarkers, Tumor; Cranial Nerve Neoplasms; Diagnosis, Differential; Electrophoresis; Humans; Magnetic Resonance Imaging; Male; Molecular Weight; Myelin Basic Protein; Neoplasms, Second Primary; Neurilemmoma; Neurofibroma; Spinal Cord Neoplasms

One hundred and two cases of benign nerve sheath tumors (NSTs) were studied with a combined approach using routine light microscopy (LM), immunohistochemistry (IH) for myelin basic protein (MBP) and S-100 protein as well as transmission electron microscopy (TEM) with the aim of obtaining greater insight into the true nature of these neoplasms, and also to establish the importance of IH and TEM in their diagnosis. Myelin basic protein was not identified in any of these tumors, whereas S-100 protein was positive to a variable degree in both schwannomas and neurofibromas. TEM revealed that Schwann cells predominated in tumors which were strongly positive for S-100 protein and appeared as schwannomas by LM. However, neurofibromas showing a variable patchy positivity for S-100 were composed of an admixture of Schwann cells, fibroblast-like cells and intermediate cells considered to be modified Schwann cells. Perineurial cells in typical form were not seen. It is concluded that all NSTs are basically of Schwann cell origin and that the intermediate cells and fibroblast-like cells are variants of Schwann cells. The different morphological appearances and biological behaviour of schwannomas and neurofibromas may be related to some other factors like micro-environment or genetic predisposition. Further, both IH, especially for S-100 protein, and TEM play an important role in establishing their diagnosis.

Topics: Cranial Nerve Neoplasms; Humans; Immunohistochemistry; Microscopy; Microscopy, Electron; Myelin Basic Protein; Neoplasms, Nerve Tissue; Neurilemmoma; Neurofibroma; S100 Proteins; Schwann Cells; Skin Neoplasms; Spinal Cord Neoplasms

Ascending myelopathy developed in a previously irradiated 10-year-old boy after intraventricular methotrexate and cytosine arabinoside were given for central nervous system relapse of acute lymphoblastic leukemia. The course was fatal in 10 weeks. Cerebrospinal fluid myelin basic protein levels, indicating a demyelinative process, rose prior to the onset of clinical symptoms and remained at very high levels until death. Myelin basic protein may be useful as a predictor of chemotherapy-associated neurotoxicity.

Topics: Antineoplastic Combined Chemotherapy Protocols; Asparaginase; Child; Combined Modality Therapy; Cytarabine; Humans; Hydrocortisone; Injections, Spinal; Leukemia, Lymphoid; Male; Methotrexate; Myelin Basic Protein; Prednisone; Radiotherapy Dosage; Recurrence; Spinal Cord Neoplasms; Testicular Neoplasms; Vincristine

Forty three patients, admitted to the department of Neurological Surgery for management of central nervous system tumours, were studied pre-operatively for serum myelin basic protein immunoreactivity as a marker of central nervous system lesion and for circulating immunoglobulins and complement (C3) levels. Myelin basic protein concentration did not appear to correlate with tumour type or grade. Serum immunoglobulin levels were found to be within the normal range but the mean IgM level was significantly higher in the glioma group when compared with meningiomas.

Topics: Adult; Brain Neoplasms; Complement C3; Female; Humans; Immunoglobulins; Male; Myelin Basic Protein; Radioimmunoassay; Spinal Cord Neoplasms