Neurilemmoma (Schwannoma)

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)

Also Known As:

Networked: 1555 relevant articles (31 outcomes, 106 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Meningioma (Meningiomas)
3.	Lymphoma (Lymphomas)
4.	Neurofibroma
5.	Glioma (Gliomas)

Experts

1.	Hanemann, C Oliver: 17 articles (03/2013 - 06/2003)
2.	Giovannini, Marco: 12 articles (07/2015 - 08/2002)
3.	Plotkin, Scott R: 11 articles (11/2015 - 01/2009)
4.	Ammoun, Sylwia: 11 articles (01/2015 - 07/2008)
5.	Stemmer-Rachamimov, Anat O: 10 articles (11/2015 - 10/2008)
6.	Hanemann, C O: 10 articles (01/2014 - 08/2000)
7.	Gutmann, David H: 10 articles (10/2008 - 12/2002)
8.	Stemmer-Rachamimov, Anat: 8 articles (01/2015 - 10/2008)
9.	Welling, D Bradley: 8 articles (10/2013 - 09/2002)
10.	Chang, Long-Sheng: 8 articles (10/2013 - 09/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neurilemmoma:

1.	Neurofibromin 2 (Merlin)IBA 01/01/2002 - "Using two-parameter FACS analysis we show that expression of wild-type merlin in NF2 cells led to significant reduction of proliferation and G0/G1 arrest in transduced schwannoma cells. " 09/29/2010 - "Consistent with the observation that over-expression of merlin arrested cell growth at G1-phase, the current study indicates that merlin exerts its antiproliferative effect, at least in part, by maintaining p21 expression, and loss of p21 is a prominent feature of merlin deficient schwannomas." 02/08/2007 - "In the present study, we show that increased expression of wild-type merlin in Tr6BC1 schwannoma cells inhibits HA binding to CD44. " 09/01/2015 - "To date, only merlin has been identified as a hallmark or pathogenesis of both sporadic and NF2-related schwannomas. " 04/01/2014 - "Merlin has multiple functions, including within the nucleus and at the cell membrane, and this review summarizes our current understanding of the mechanisms by which merlin loss is involved in schwannoma pathogenesis, highlighting potential areas for therapeutic intervention. "
2.	nestin (nestin protein)IBA 06/01/2002 - "Nestin is confirmed to be consistently expressed in GISTs but it is also present in most GI schwannomas; GFAP is helpful when separating GISTs and schwannomas, since only the latter are positive. " 03/01/2006 - "Nestin is a recently identified intermediate filament protein that is expressed in neuroectodermal stem cells and skeletal muscle progenitor cells and has been shown to be expressed in gastrointestinal stromal tumours (GISTs) and GI schwannomas. " 07/01/2012 - "Our results suggest that: (1) nestin is a useful marker for diagnosis of high-grade gliomas, (2) nestin is helpful in diagnosis of schwannomas, and (3) nestin expression is related to poor prognosis in high-grade gliomas." 02/01/2005 - "Because of the weak and heterogeneous expression of nestin in neurinomas, phaeochromocytomas, and carcinoids, nestin detection in these lesions is of little practical use." 03/01/2001 - "In contrast, three leiomyomas and a schwannoma that developed in the gastrointestinal tract showed no apparent immunoreactivity for nestin. "
3.	AntibodiesIBA 09/01/1993 - "An immunohistochemical study using a comprehensive panel of antibodies to Schwann cell markers, intermediate filaments and extracellular matrix components has been performed on three cases of plexiform schwannoma. " 01/01/2012 - "Immunohistochemical analysis of the schwannoma was performed with p63 and p73 antibodies and clinical patient characteristics were correlated with the immunoreactivity results. " 03/01/2004 - "Schwannomas exhibiting a monolayered EL are as strong in immunoreaction as those displaying reduplicated/thickened EL, indicating that a single layer of EL is thick enough to be identified by both antibodies with sufficient sensitivity. " 12/01/2003 - "None of the 12 solitary schwannomas reacted with anti-axon neurofilament antibodies. " 10/15/1999 - "Neurinoma AChE reacted with the antibodies HR2 and AE1 raised against AChE from human brain or erythrocyte, whereas BuChE bound to a sheep antiserum."
4.	S100 Proteins (S 100 Protein)IBA 05/01/2015 - "On the basis of the histopathologic and immunohistochemical staining with S-100 protein, a diagnosis of plexiform schwannoma was made and has been reported in this study. " 01/01/2013 - "Histopathology confirmed these lesions as schwannomas consisting of areas of Antoni A and B, and immunohistochemical study was positive for S-100 protein. " 01/01/2000 - "Histopathologic examination showed intertwined bundles of spindle cells with spiral-like proliferation, and immunohistochemical studies were positive for S-100 protein, whereby a diagnosis of esophageal schwannoma was established. " 06/01/2015 - "Histopathology and immunohistochemistry of this mass and other endocardial masses identified at necropsy for S-100 protein were consistent with a diagnosis of schwannoma. " 06/01/2015 - "The immunohistochemistry reveals that the schwannoma cells test positive for S-100 protein. "
5.	Messenger RNA (mRNA)IBA 09/01/2015 - "These data are in agreement with the model in RT-4 rat schwannoma cells: i.e., overexpression of ARF was associated with accumulation of p21 expression both at protein and mRNA levels. " 02/01/2013 - "Importantly, we show that human schwannoma cells have reduced expression of SOX10 protein and messenger RNA. " 12/01/2006 - "By this method a total of seven genes with increased and seven genes with decreased mRNA levels in schwannoma compared with normal Schwann cells could be identified. " 12/01/2005 - "In contrast, sst2A mRNA and protein was present in 89% of schwannomas. " 08/01/2003 - "In the human sciatic nerve and schwannoma, RT-PCR, Western blotting and immunohistochemistry analysis further confirmed the presence of Tie-2 mRNA and protein in non-autonomic peripheral nervous tissue. "
6.	Nerve Growth Factor (NGF)IBA 03/17/2000 - "Nerve growth factor activation of nuclear factor kappaB through its p75 receptor is an anti-apoptotic signal in RN22 schwannoma cells." 01/01/1994 - "Nerve growth factor (NGF) reduces the development of trigeminal neurinomas in vivo. " 01/01/1994 - "Growth inhibition of synchronized trigeminal neurinoma cells by nerve growth factor." 03/01/1992 - "In an attempt to determine whether NGF alters patterns of gene expression in p75NGFR-bearing Schwann cells, we examined the regulation of three early response genes (NGFI-A, NGFI-B, and c-fos) in JS1 rat schwannoma cells. " 12/15/1989 - "Of the 34 rats in the NGF-treated group, 11 animals were affected with trigeminal nerve neurinomas as compared to 18/34 in the NGF-untreated group (P less than 0.05). "
7.	GadoliniumIBA 08/01/1997 - "In this study we present a case of schwannoma of the trachea investigated with computer tomography (axial, precontrast) and magnetic resonance (1 Tesla, sagittal T1w before and after Gadolinium, sagittal T2w, coronal and axial after Gadolinium). " 01/01/2015 - "Diagnosis of intralabyrinthine schwannoma was made with gadolinium-enhanced magnetic resonance imaging scans. " 10/01/2013 - "It can provide patterns of oculomotor responses suggesting the execution of gadolinium-enhanced MRI, upon the suspicion of schwannoma of the eighth cranial nerve or of a central disease. " 04/01/2013 - "The black geode sign may be fairly specific to extracranial schwannomas on gadolinium-enhanced MR images." 04/01/2013 - "Forty-three patients with pathologically confirmed extracranial schwannomas underwent preoperative gadolinium-enhanced MRI. "
8.	Ethylnitrosourea (N-Ethyl-N-nitrosourea)IBA 05/01/2006 - "A point mutation of the neu/erbB-2 gene diagnostic of N-ethyl-N-nitrosourea-induced rat schwannomas is an early marker of Schwann precursor cells at high risk of subsequent malignant transformation. " 05/01/2006 - "Contrary to rats of the highly sensitive inbred strain BDIX, BDIV rats are resistant to the induction of malignant schwannomas by N-ethyl-N-nitrosourea, arising predominantly in the trigeminal nerves. " 09/01/2000 - "Contrary to the response of rats of the highly sensitive inbred strain BDIX, BDIV rats are resistant to the induction of malignant schwannomas by exposure to the alkylating N-nitroso carcinogen N-ethyl-N-nitrosourea (EtNU). " 09/01/2000 - "Suppression of ethylnitrosourea-induced schwannoma development involves elimination of neu/erbB-2 mutant premalignant cells in the resistant BDIV rat strain." 12/01/1999 - "Activation of neu by missense point mutation in the transmembrane domain in schwannomas induced in C3H/HeNCr mice by transplacental exposure to N-nitrosoethylurea."
9.	Carbon MonoxideIBA 01/01/2011 - "We report a rare patient of a simultaneous extradural and intradural compression of the cervical spinal cord due to co-existent intervertebral disc herniation and an intradural schwannoma at the same level. " 08/01/2009 - "Although it seems that the co-occurrence of CMT1A and schwannomas in a family would be the result of independent events, we could not completely ignore the possibility that the coincidence of two diseases might be due to a shared genetic background." 05/01/2002 - "Clinical features, es pecially computed tomography (CT) scan, were completely reviewed to find the co-existence of orbital neurilemmoma and pneumosinus dilatans. " 02/27/1998 - "Moreover, all of the schwannoma cases co-expressed the HGF gene. " 01/01/1984 - "This report relates to the 50 neurinomas over 2 cm in diameter, these requiring multidisciplinary co-operation during surgical removal. "
10.	Somatostatin Receptors (Somatostatin Receptor)IBA 05/01/1997 - "Somatostatin receptors in schwannomas." 12/01/2005 - "Furthermore, our findings suggest a potential clinical role for somatostatin receptor agonists in tumor imaging and/or treatment of schwannomas and MPNSTs." 11/01/1987 - "Tumors originating from neuroblasts, i.e., ganglioneuroblastoma and medulloblastoma, contained a high density of somatostatin receptors, whereas neurinomas and neurofibromas as well as the ependymomas, one teratoma, and one plexus papilloma were lacking such receptors. " 11/01/1987 - "Fifty-two brain tumors, consisting of 17 astrocytomas, 4 oligodendrogliomas, 20 glioblastomas, 3 neurinomas, 2 ependymomas, 1 neurofibroma, 1 ganglioneuroblastoma, 1 medulloblastoma, 1 plexus papilloma, 1 teratoma, and 1 germinoma, were tested for their content of specific somatostatin receptors using autoradiographic techniques or in vitro binding assays with membrane homogenates. " 04/15/1996 - "Somatostatin receptors were identified in bone and vascular/perivascular tumors (3 of 3 osteosarcomas, 1 of 1 giant cell tumor, 2 of 2 angiosarcomas, and 4 of 4 hemangiopericytomas), in 2 of 2 synovial sarcomas, in 2 of 5 histiocytomas, and in several muscle cell tumors (1 of 2 leiomyomas, 2 of 4 leiomyosarcomas, and 3 of 5 rhabdomyosarcomas) but were absent in 4 liposarcomas, 3 mesotheliomas, 3 chondrosarcomas, 10 Ewing sarcomas, 11 schwannomas, and 5 Wilms' tumors. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 07/01/2010 - "The recognition of intranodal schwannoma is important because it is cured with excision, whereas some of the other diagnostic considerations for a spindle cell lesion within a lymph node may require radiation or chemotherapy." 04/01/2007 - "Malignant schwannomas are insensitive to chemotherapy and radiation, resulting in poor prognosis." 01/01/1992 - "The prognosis is poor in the malignant forms and chemotherapy may be useful in malignant schwannomas." 06/01/2001 - "A benign retroperitoneal schwannoma from a patient without prior exposure to radiotherapy or chemotherapy was analyzed by chromosome banding after short-term culture. " 11/01/2012 - "Schwannoma is radioresistant, and chemotherapy is not effective, so surgical resection is the only curative treatment for this tumor. "
2.	Heterologous Transplantation (Xenotransplantation) 09/01/2011 - "The in vivo efficacy of AR42 was investigated using schwannoma xenografts. " 06/15/2009 - "Lack of robust xenograft or transgenic models of schwannomas prevents extension of these studies in vivo. " 09/01/2011 - "In vivo treatment with AR42 inhibited the growth of schwannoma xenografts, induced apoptosis, and decreased Akt activation. " 01/01/2009 - "We have successfully established an intracranial schwannoma xenograft model that can be followed with noninvasive BLI. " 01/01/2012 - "AR42 was dosed orally in murine schwannoma allografts and human VS xenografts. "
3.	Radiotherapy 12/01/2014 - "Results We present a case with > 10 years follow-up of radiation following skull base SFT, initially misdiagnosed as schwannoma, where radiotherapy did not improve recurrence or metastatic behavior and led to complications during subsequent surgical resection. " 04/01/2014 - "We describe an unusual case of malignant transformation of benign cranial nerves schwannoma eleven months after surgery and five months post radiotherapy. " 12/01/2013 - "Multiple-fraction delivery is better termed "stereotactic radiotherapy." There are compelling radiobiological principles supporting the biological superiority of single-fraction radiation for achieving an optimal therapeutic response for the slowly proliferating, late-responding, tissue of a schwannoma. " 09/01/2013 - "A schwannoma of the greater petrosal nerve located within the petrous apex and treated with stereotactic radiotherapy." 08/01/2012 - "Data on radiotherapy for trigeminal schwannomas (TSs) and comparison of stereotactic radiosurgery (SRS) with fractionated stereotactic radiotherapy (FSRT) are limited. "
4.	Laminectomy 06/01/2012 - "Hemi-semi laminectomy is a safe and effective method for resection of spinal schwannomas." 12/01/2015 - "This study comprised 49 consecutive patients with intradural extramedullary schwannoma who underwent surgical resection: 31 patients via MIS approach (MIS group; 6 patients via a muscle-splitting approach using a tubular retractor and 25 patients via unilateral hemilaminectomy preserving the contralateral paraspinal muscle) and 18 patients via total laminectomy (TL group). " 01/01/2015 - "Here, we report a successful removal of a lumbar dumbbell-shaped schwannoma using a combined laminoplastic laminotomy with Wiltse's paraspinal surgical approach. " 01/01/2015 - "Posterior Laminoplastic Laminotomy Combined with a Paraspinal Transmuscular Approach for Removing a Lumbar Dumbbell-shaped Schwannoma: A Technical Note." 01/01/2015 - "We present the case of a woman with a preoperative radiological diagnosis of schwannoma at D10 level in whom a laminectomy was performed to remove the tumour. "
5.	Aftercare (After-Treatment) 03/01/2005 - "The purpose of this study is to examine the presenting signs and symptoms, location, nerve of origin, and outcome after treatment of patients with nonvestibular schwannomas of the head and neck. " 03/01/2008 - "We report here a case of masticatory disturbance evoked by trigeminal schwannoma, in which we have evaluated the changes in occlusal force and masticatory sensation before and after treatment for the tumour. " 01/01/2006 - "In the case of subcutaneous schwannoma xenografts, reduction in size after infection occurred more slowly, with a mean reduction of onethird by 42 days after treatment. " 01/01/2007 - "Complications developed in only one patient: the onset of facial palsy immediately after treatment, which subsequently recovered to House-Brackmann Grade III. In summary, radiosurgery was found to be a very useful method of treating facial schwannoma, for both tumor control and functional control. "