Neurofibromatosis type 2: growth stimulation of mixed acoustic schwannoma by concurrent adjacent meningioma: possible role of growth factors

1998 ◽  
Vol 89 (1) ◽  
pp. 149-154 ◽  
Author(s):  
Roberto Pallini ◽  
Angelo Tancredi ◽  
Patrizia Casalbore ◽  
Delio Mercanti ◽  
Luigi M. Larocca ◽  
...  
Keyword(s):  
Neurofibromatosis Type ◽  
Growth Stimulation ◽  
Neurofibromatosis Type 2 ◽  
Annual Growth Rate ◽  
Content Type ◽  
Acoustic Schwannoma ◽  
Epidermal Growth ◽  
The One

✓ The authors report the case of a young man suffering from neurofibromatosis type 2 (NF2) who harbored bilateral acoustic schwannomas and a parasellar meningioma. Neuroimaging studies performed during a 4-year follow-up period showed that the bilateral schwannomas had grown very little and at similar rates. However, after the meningioma had infiltrated the tentorium and approached the ipsilateral schwannoma at the incisura, both Schwann cell tumors started to grow rapidly, particularly the one adjacent to the meningioma, of which the percentage of annual growth rate increased by approximately a factor of 102. At the same time, magnetic resonance imaging showed that this tumor also changed its features. During surgery, the acoustic schwannoma was firmly adherent to both meningioma and tentorium. Histological examination revealed meningotheliomatous cells in the schwannoma adjacent to the meningioma. Antiphosphotyrosine immunoblotting of PC12 cells was compatible with the presence of an epidermal growth factor (EGF)—like molecule in the cerebrospinal fluid (CSF) of the patient. This factor was not detected in the CSF of five other NF2 patients, two of whom bore associated bilateral acoustic schwannomas and meningioma in remote locations. It is hypothesized that the meningotheliomatous cells infiltrating the schwannoma triggered an autocrine/paracrine growth—stimulatory mechanism that involved an EGF-like factor.

scholarly journals Neurofibromatosis type 2: growth stimulation of mixed acoustic schwannoma by concurrent adjacent meningioma: possible role of growth factors

1998 ◽  
Vol 4 (4) ◽  
pp. E11
Author(s):  
Roberto Pallini ◽  
Angelo Tancredi ◽  
Patrizia Casalbore ◽  
Delio Mercanti ◽  
Luigi M. Larocca ◽  
...  
Keyword(s):  
Neurofibromatosis Type ◽  
Growth Stimulation ◽  
Neurofibromatosis Type 2 ◽  
Annual Growth Rate ◽  
Acoustic Schwannoma ◽  
Epidermal Growth ◽  
The One

The authors report the case of a young man suffering from neurofibromatosis type 2 (NF2) who harbored bilateral acoustic schwannomas and a parasellar meningioma. Neuroimaging studies performed during a 4-year follow-up period showed that the bilateral schwannomas had grown very little and at similar rates. However, after the meningioma had infiltrated the tentorium and approached the ipsilateral schwannoma at the incisura, both Schwann cell tumors started to grow rapidly, particularly the one adjacent to the meningioma, of which the percentage of annual growth rate increased by approximately a factor of 102. At the same time, magnetic resonance imaging showed that this tumor also changed its features. During surgery, the acoustic schwannoma was firmly adherent to both meningioma and tentorium. Histological examination revealed meningotheliomatous cells in the schwannoma adjacent to the meningioma. Antiphosphotyrosine immunoblotting of PC12 cells was compatible with the presence of an epidermal growth factor (EGF)-like molecule in the cerebrospinal fluid (CSF) of the patient. This factor was not detected in the CSF of five other NF2 patients, two of whom bore associated bilateral acoustic schwannomas and meningioma in remote locations. It is hypothesized that the meningotheliomatous cells infiltrating the schwannoma triggered an autocrine/paracrine growth-stimulatory mechanism that involved an EGF-like factor.

Age at symptom onset and long-term survival in patients with neurofibromatosis Type 2

2003 ◽  
Vol 99 (3) ◽  
pp. 480-483 ◽  
Author(s):  
Goro Otsuka ◽  
Kiyoshi Saito ◽  
Tetsuya Nagatani ◽  
Jun Yoshida
Keyword(s):  
Symptom Onset ◽  
Survival Rates ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Content Type ◽  
Fine Print

Object. Neurofibromatosis Type 2 (NF2) is an intractable disorder predisposing to multiple, recurrent tumors of the central nervous system (CNS). To clarify the survival rate and characteristics that predict poor survival, we retrospectively reviewed clinical data in cases of NF2. Methods. From among 283 patients with neurofibromatosis who had been registered in a nationwide study in Japan between 1986 and 1987, 74 patients with bilateral vestibular schwannomas were analyzed. The mean duration of follow up after diagnosis was 121 months (range 2–287 months). Results of a Kaplan—Meier product-limit analysis indicated that overall 5-, 10-, and 20-year patient survival rates following diagnosis of NF2 were 85, 67, and 38%, respectively. Early onset of the initial symptom significantly compromised survival; 5-, 10-, and 20-year survival rates in patients with symptom onset at an age younger than 25 years were 80, 60, and 28%, respectively, whereas in patients with symptom onset at an age of 25 years or older the rates were 100, 87, and 62%, respectively. Patients with small vestibular schwannomas at diagnosis (< 2 cm in diameter) had better rates of survival. Other variables such as sex, additional tumors in the CNS, or dermal abnormalities did not significantly affect survival. Conclusions. This first report of long-term follow-up results concerning the survival of patients with NF2 indicates an adverse effect of early symptom onset.

Mechanisms of edema after gamma knife surgery for meningiomas

2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 1-3 ◽  
Author(s):  
Amr El Shehaby ◽  
Jeremy C. Ganz ◽  
Wael A. Reda ◽  
Ayman Hafez
Keyword(s):  
Gamma Knife ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Gamma Knife Surgery ◽  
Skull Defect ◽  
Brain Swelling ◽  
Content Type ◽  
Multiple Tumors ◽  
Fine Print

✓ The authors describe two patients in whom tumor swelling and brain swelling (and possible tumor swelling), respectively, developed after undergoing gamma knife surgery. One had a skull defect with a palpable parasagittal tumor. One had neurofibromatosis Type 2 with multiple tumors, one of which was parasagittal.

Multiple schwannomas: schwannomatosis or neurofibromatosis type 2?

1998 ◽  
Vol 89 (1) ◽  
pp. 36-41 ◽  
Author(s):  
Matti Tapio Seppälä ◽  
Markku Alarik Sainio ◽  
Matti Jouko Johannes Haltia ◽  
Jaakko Jyri Kinnunen ◽  
Kirsi Hannele Setälä ◽  
...  
Keyword(s):  
Clinical Course ◽  
Positive Family History ◽  
Neurofibromatosis Type ◽  
Magnetic Resonance Images ◽  
Neurofibromatosis Type 2 ◽  
Long Term Outcome ◽  
Content Type ◽  
Fine Print

Object. The aim of this study was to clarify the clinical outcome of schwannomatosis, a rare condition characterized by multiple nonvestibular schwannomas in the absence of meningiomas, intraspinal ependymomas, and other clinical signs of neurofibromatosis type 2 (NF2). Methods. Nine patients with schwannomatosis treated at one institution are presented and their clinical course during a median follow-up time of 9.9 years is discussed. The patients were typically middle-aged at the time of their first operation (median 43.5 years), none had a positive family history of schwannomatosis or NF2, and none showed cutaneous or ocular signs of NF2. On histopathological examination the tumors from the patients with schwannomatosis showed a lobular appearance and frequent Verocay bodies, signs indicating NF2, more often than 20 sporadic schwannomas that were investigated as controls. Two patients died of unrelated causes at 3.2 and 9.9 years, respectively, of follow up. Magnetic resonance images of the head and spine were obtained in seven patients at the end of the follow-up period. New spinal schwannomas were detected in one patient and a residual schwannoma in three. No germline mutations of the NF2 gene were found in these seven patients. Two additional patients originally included in the schwannomatosis group who were 8.6 and 11.7 years old at initial surgery had NF2. One was diagnosed at follow-up review and the other developed a fulminant disease that led to death in 4 years. Conclusions. The clinical course, long-term outcome, and genetic mechanism of schwannomatosis differ from that of NF2.

Molecular alterations in the neurofibromatosis Type 2 gene and its protein rarely occurring in meningothelial meningiomas

2001 ◽  
Vol 94 (1) ◽  
pp. 111-117 ◽  
Author(s):  
James J. Evans ◽  
Sin-Soo Jeun ◽  
Joung H. Lee ◽  
Jyoti A. Harwalkar ◽  
Yigal Shoshan ◽  
...  
Keyword(s):  
Protein Expression ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Content Type ◽  
Exact Test ◽  
Fisher's Exact Test ◽  
Single Stranded Conformational Polymorphism ◽  
Fisher’S Exact Test ◽  
Fine Print

Object. The neurofibromatosis Type 2 (NF2) gene is the only tumor suppressor gene that has been clearly implicated in the development of benign meningiomas. Interestingly, previous data obtained by the authors indicate that reduced NF2 protein expression seldom occurs in meningothelial meningiomas, the most common histological type of meningioma. The goal of the current study was to explore further the hypothesis of NF2 gene-independent tumorigenesis of meningothelial meningiomas. Methods. The authors performed a mutational analysis of all 17 exons of the NF2 gene by using single-stranded conformational polymorphism (SSCP). In addition, expression levels of the NF2 protein and (µ-calpain, a protease suggested to inactivate the NF2 protein, were determined by immunoblotting analysis of 27 meningiomas (20 meningothelial and seven nonmeningothelial). Mutations of the NF2 gene were found in only one (5%) of 20 meningothelial meningiomas and three (43%) of seven nonmeningothelial tumors (Fisher's exact test, p = 0.042). The levels of NF2 protein were severely reduced in six (28.5%) of 21 meningothelial meningiomas, in contrast to six (86%) of seven nonmeningothelial meningiomas (Fisher's exact test, p = 0.023). Activation of (µ-calpain did not correlate with the status of NF2 protein expression in the meningiomas analyzed, demonstrating that (µ-calpain activation does not account for the loss of NF2 protein in meningiomas with apparently normal NF2 genes. Conclusions. These results clearly demonstrate that NF2 gene mutations and decreased NF2 protein expression rarely occur in meningothelial meningiomas compared with other histological types of meningiomas. The clinical behavior of meningothelial meningiomas, however, is similar to that of other benign meningiomas. It is likely, therefore, that the tumorigenesis of meningothelial meningiomas is the result of deleterious alterations of genes that have final phenotypical effects similar to inactivation of the NF2 gene.

Mechanisms of edema after gamma knife surgery for meningiomas

2005 ◽  
Vol 102 ◽  
pp. 1-3 ◽  
Author(s):  
Amr El Shehaby ◽  
Jeremy C. Ganz ◽  
Wael A. Reda ◽  
Ayman Hafez
Keyword(s):  
Gamma Knife ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Gamma Knife Surgery ◽  
Skull Defect ◽  
Brain Swelling ◽  
Content Type ◽  
Multiple Tumors ◽  
Fine Print

✓ The authors describe two patients in whom tumor swelling and brain swelling (and possible tumor swelling), respectively, developed after undergoing gamma knife surgery. One had a skull defect with a palpable parasagittal tumor. One had neurofibromatosis Type 2 with multiple tumors, one of which was parasagittal.

Vestibular schwannoma growth in patients with neurofibromatosis Type 2: a longitudinal study

2002 ◽  
Vol 96 (2) ◽  
pp. 223-228 ◽  
Author(s):  
Victor-Felix Mautner ◽  
Michael E. Baser ◽  
Sarang D. Thakkar ◽  
Urs M. Feigen ◽  
J. M. Friedman ◽  
...  
Keyword(s):  
Growth Rates ◽  
Age At Onset ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Spinal Tumors ◽  
Content Type ◽  
Gradient Gel Electrophoresis ◽  
Temperature Gradient Gel Electrophoresis ◽  
Fine Print

Object. The factors that determine the growth rates of vestibular schwannomas (VSs) in patients with neurofibromatosis Type 2 (NF2) are unknown. The authors undertook this study to determine if clinical factors or type of constitutional NF2 mutation were associated with VS growth rates in cases of NF2. Methods. The authors reviewed serial gadolinium-enhanced magnetic resonance (MR) images of the head and full spine of 37 patients with sporadic NF2 who had been observed over periods ranging from 0.2 to 8 years (median 3.9 years) at a specialized referral clinic for NF2. A box model was used to calculate VS volumes so that tumor growth rates could be estimated. Temperature-gradient gel electrophoresis was used to screen for constitutional NF2 mutations. The VS growth rates tended to decrease with increasing patient age at onset of signs or symptoms (r2 = 0.23, p = 0.003) and at the time the baseline gadolinium-enhanced MR image was obtained (r2 = 0.38, p < 0.001). The authors did not find significant associations between VS growth rates and the number of non-VS cerebral or spinal tumors or different types of constitutional NF2 mutations. Conclusions. There is considerable variability in growth rates of VSs in patients with NF2, but they tend to be higher in patients who are younger at onset of signs or symptoms.

Hearing restoration with auditory brainstem implants after radiosurgery for neurofibromatosis Type 2

2001 ◽  
Vol 95 (6) ◽  
pp. 1028-1033 ◽  
Author(s):  
Michel Kalamarides ◽  
Alexis Bozorg Grayeli ◽  
Didier Bouccara ◽  
Emmanuelle Ambert Dahan ◽  
Wolf Peter Sollmann ◽  
...  
Keyword(s):  
Radiation Treatment ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Sound Recognition ◽  
Tumor Removal ◽  
Environmental Sound ◽  
Content Type ◽  
Auditory Brainstem Implants

✓ The auditory brainstem implant (ABI) is designed to restore useful auditory sensations in patients with neurofibromatosis Type 2 (NF2). The implantation is usually performed at the time of tumor removal in patients who do not undergo radiation treatment. The authors evaluated the performance of ABIs in three patients with NF2 in whom vestibular schwannoma continued to grow after radiation treatment. These three patients with NF2 received a 21-channel ABI; a translabyrinthine approach was used for both the tumor removal and the ABI placement. The interval between radiosurgery and the tumor removal plus device implantation ranged from 2 to 11 years. In all cases, the tumor was growing and the patients presented with total deafness. The mean number of active electrodes in these three patients was equivalent to the average results reported in other patients who received ABIs. The patients in this study used the ABI regularly for everyday life and obtained useful levels of environmental sound recognition. It is concluded that hearing function can be rehabilitated using ABIs in patients with NF2, even if radiosurgery fails to control the tumor growth.

Spinal cord ganglioglioma in a child with neurofibromatosis Type 2

1999 ◽  
Vol 90 (2) ◽  
pp. 231-233 ◽  
Author(s):  
Paul D. Sawin ◽  
Nicholas Theodore ◽  
Harold L. Rekate
Keyword(s):  
Spinal Cord ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Histopathological Analysis ◽  
Unique Case ◽  
Content Type ◽  
Complete Surgical Resection ◽  
Disease Entities ◽  
Glial Lineage

✓ Gangliogliomas of the spinal cord are rare disease entities that occur in early childhood. Their occurrence in association with neurofibromatosis Type 2 (NF2) has not been described. The authors describe the unique case of a 2-year-old child with stigmata of NF2 who harbored a spinal cord ganglioglioma that presented as a rapidly growing, exophytic intramedullary mass lesion at the cervicomedullary junction. Treatment consisted of complete surgical resection. Histopathological analysis of the lesion demonstrated a mixed population of neoplastic cells, of both neuronal and glial lineage, that supported the diagnosis of ganglioglioma.

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