An unusual pterygopalatine meningocele associated with neurofibromatosis Type 1

2000 ◽  
Vol 93 (3) ◽  
pp. 480-483 ◽  
Author(s):  
Paul H. Chapman ◽  
Hugh D. Curtin ◽  
Michael J. Cunningham
Keyword(s):  
Cavernous Sinus ◽  
Neurofibromatosis Type 1 ◽  
Lateral Wall ◽  
Neurofibromatosis Type ◽  
Middle Fossa ◽  
Recurrent Meningitis ◽  
Superior Orbital Fissure ◽  
Content Type ◽  
Middle Fossa Approach

✓ The authors describe an unusual meningocele of the lateral wall of the cavernous sinus and the anterior skull base in a young patient with typical stigmata of neurofibromatosis Type 1 (NF1). This lesion was discovered during evaluation for recurrent meningitis. It represented an anterior continuation of Meckel's cave into a large cerebrospinal fluid space within the lateral wall of the cavernous sinus, extending extracranially through an enlarged superior orbital fissure into the pterygopalatine fossa adjacent to the nasal cavity. It was successfully obliterated, via an intradural middle fossa approach, with fat packing and fenestration into the subarachnoid space. This meningocele most likely represents a variant of cranial nerve dural ectasia occasionally seen in individuals with NF1. It has as its basis the same mesodermal defect responsible for the more common sphenoid wing dysplasia and spinal dural ectasias identified with this condition. Involvement of the trigeminal nerve with expansion of the lateral wall of cavernous sinus has not been reported previously. The authors surmise, however, that it may be present in some cases of orbital meningocele associated with sphenoid wing dysplasia.

Establishment and characterization of a novel malignant astrocytoma cell line derived from a tumor removed in a patient with neurofibromatosis Type 1

2001 ◽  
Vol 94 (2) ◽  
pp. 301-308 ◽  
Author(s):  
Masanori Kurimoto ◽  
Yutaka Hirashima ◽  
Tsuneaki Ogiichi ◽  
Hideo Hamada ◽  
Hironaga Kamiyama ◽  
...  
Keyword(s):  
Cell Line ◽  
Neurofibromatosis Type 1 ◽  
Proliferative Activity ◽  
Neurofibromatosis Type ◽  
Malignant Astrocytoma ◽  
Content Type ◽  
Astrocytoma Cell Line ◽  
Astrocytoma Cell ◽  
Fine Print

Object. Patients with neurofibromatosis Type 1 (NF1) have a predisposition to development of a variety of benign and malignant tumors including neurofibromas, astrocytomas, pheochromocytomas, and malignant peripheral nerve sheath tumors. The availability of an astrocytoma cell line derived from NF1 would be useful in studies in which sporadic astrocytomas could be compared with NF1-derived astrocytomas. In this article the authors describe a novel astrocytoma cell line, TM-31, that they established from a tumor removed in a 42-year-old woman with NF1. Methods. The TM-31 cell line was prepared from a surgical specimen of malignant astrocytoma and was serially subcultured over 250 times throughout a 6-year period without showing any sign of cell senescence. Immunocytochemical analyses demonstrated that TM-31 cells are negative for glial fibrillary acidic protein but positive for vimentin and S-100 protein. The TM-31 cells display little neurofibromin expression when subjected to immunoblotting, indicating that there is an NF1 gene mutation. Polymerase chain reaction—single-strand conformational polymorphism analysis revealed that TM-31 cells harbor a p53 point mutation in exon 7, codon 238. Chemosensitivity testing of TM-31 cells revealed a resistance to 1-(4-amino-2-methyl-5-pyrimidinyl)methyl-3-(2-chloroethyl)-3-nitrosourea, although they are sensitive to cisplatin and etoposide. In addition, TM-31 cells displayed no morphological differentiation after all-transretinoic acid and dibutyryl cyclic adenosine monophosphate treatments. Pharmacological inhibition of farnesyltransferase of the Ras oncoprotein led to decreased proliferative activity and inhibition of anchorage-independent growth of TM-31 cells in soft agar. Conclusions. The TM-31 cell line is an immortalized astrocytoma cell line derived from a tumor obtained in a patient with NF1. Ras activation may be the major event of proliferative activity and of the transformed phenotype of TM-31 cells, and the farnesyltransferase inhibitor may be potentially important as a novel antiproliferative therapy for NF1-derived astrocytomas.

Suboccipital meningocele presenting as a huge retropharyngeal mass in a patient with neurofibromatosis Type 1

1999 ◽  
Vol 91 (3) ◽  
pp. 503-505 ◽  
Author(s):  
Masanori Kurimoto ◽  
Yutaka Hirashima ◽  
Nakamasa Hayashi ◽  
Shunro Endo ◽  
Masayoshi Ohi ◽  
...  
Keyword(s):  
Bone Defect ◽  
Neurofibromatosis Type 1 ◽  
Contrast Material ◽  
Neurofibromatosis Type ◽  
Occipital Bone ◽  
Cutaneous Manifestations ◽  
Content Type ◽  
The Right ◽  
Occipital Bone Defect

✓ The authors report an extremely rare case of neurofibromatosis Type 1 (NF1) with a suboccipital meningocele presenting as a huge retropharyngeal mass. A 73-year-old woman with typical cutaneous manifestations of NF1 presented with nasal obstruction and dysphagia due to a retropharyngeal mass. Magnetic resonance imaging revealed a huge mass lesion extending from the right occipital bone defect to the retropharynx through the right paravertebral region. Computerized tomography scanning after intrathecal administration of contrast material confirmed that the mass was a meningocele protruding through a right occipital bone defect. The authors attempted to ligate this meningocele, most of which was excised via a suboccipital approach, but a second transcervical operation was required. Finally, the meningocele resolved and the patient was discharged without symptoms.

Successful surgical repair of progressive exophthalmos caused by a meningocele in a patient with neurofibromatosis Type 1

1998 ◽  
Vol 89 (6) ◽  
pp. 1032-1035 ◽  
Author(s):  
Joost de Vries ◽  
Hans Peter M. Freihofer ◽  
Tomas Menovsky ◽  
Johannes R. M. Cruysberg
Keyword(s):  
Visual Acuity ◽  
Neurofibromatosis Type 1 ◽  
Surgical Repair ◽  
Visual Fields ◽  
Neurofibromatosis Type ◽  
Content Type ◽  
Ocular Movements ◽  
Orbital Wall ◽  
The Right

✓ A case of surgical repair of progressive exophthalmos of the right eye in a 43-year-old woman with neurofibromatosis Type 1 (NF1) is presented. Preoperatively, the patient's ocular movements and visual fields were intact. Visual acuity was 20/30 on the right side and 20/20 on the left. Computerized tomography scanning demonstrated complete absence of the superolateral orbital wall on the right side with a large meningocele protruding into the right orbit. Intraoperatively, a new superolateral wall was constructed using the inner table of the left frontal bone as a bone transplant. A free galeoperiosteum flap was used for water-tight dural reconstruction. A few weeks postoperatively the patient's exophthalmos showed remarkable resolution. Her ocular movements, visual acuity, and visual fields remained unchanged. In conclusion, reconstruction of the superolateral wall and repair of a meningocele in a patient with NF1 is worthwhile and can be followed by excellent cosmetic results. More important, the patient's visual functions remain preserved.

Benign spinal nerve sheath tumors: their occurrence sporadically and in neurofibromatosis types 1 and 2

1991 ◽  
Vol 74 (2) ◽  
pp. 248-253 ◽  
Author(s):  
Andrea L. Halliday ◽  
Raymond A. Sobel ◽  
Robert L. Martuza
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Spinal Nerve ◽  
Neurofibromatosis Type 2 ◽  
Content Type ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Fine Print

✓ Benign spinal nerve sheath tumors (neurofibromas and schwannomas) often occur on dorsal nerve roots sporadically or in neurofibromatosis types 1 and 2. These are histologically benign tumors, and distinction between them is frequently not made by clinicians. To determine if there is a correlation between the histological pattern of benign spinal nerve sheath tumors and the type of neurofibromatosis, the clinical and pathological features of these tumors (86 surgical specimens and five autopsies) in 68 patients were reviewed. The patients were classified into one of four categories: neurofibromatosis type 1, neurofibromatosis type 2, uncertain, or sporadic. The diagnostic criteria used for neurofibromatosis types 1 and 2 were established by the National Institutes of Health. Patients who did not fulfill criteria for either neurofibromatosis type 1 or 2 but who had multiple nervous system tumors or other stigmata of neurofibromatosis were designated “uncertain.” Spinal nerve sheath tumors were considered sporadic in 42 cases (40 schwannomas and two neurofibromas). In the 14 patients with neurofibromatosis type 1, all spinal nerve sheath tumors were neurofibromas. In six of the seven patients with neurofibromatosis type 2, all spinal nerve sheath tumors were schwannomas. One patient with neurofibromatosis type 2 had a spinal nerve sheath schwannoma and a tumor with features of both tumor types. The authors conclude that spinal nerve sheath tumors in patients with neurofibromatosis type 1 are neurofibromas. In contrast, spinal nerve sheath tumors occurring in neurofibromatosis type 2 or sporadically are most frequently schwannomas. The distinct histological features of these tumors may reflect different pathogenetic mechanisms even though they arise at identical sites in neurofibromatosis types 1 and 2.

Symptomatic hamartoma of the spinal cord associated with neurofibromatosis type 1

1998 ◽  
Vol 88 (6) ◽  
pp. 1099-1103 ◽  
Author(s):  
Richard D. Brownlee ◽  
Arthur W. Clark ◽  
Robert J. Sevick ◽  
S. Terence Myles
Keyword(s):  
Spinal Cord ◽  
Mr Imaging ◽  
Neurofibromatosis Type 1 ◽  
Ganglion Cells ◽  
Neurofibromatosis Type ◽  
Conus Medullaris ◽  
Thoracic Spinal Cord ◽  
Content Type ◽  
Thoracic Spinal

✓ The authors present a case in which a symptomatic hamartoma was found in the spinal cord of a patient with neurofibromatosis type 1 (NF-1). This 52-year-old woman presented with painful urinary incontinence. Magnetic resonance (MR) imaging revealed an intramedullary lesion within the lower thoracic spinal cord and conus medullaris, which was surgically removed. Pathological investigation showed a hamartomatous lesion consisting of glial cells, ganglion cells, abundant disoriented axons, and thin-walled vessels. This case provides a pathological correlate to the hamartomatous lesions demonstrated on MR imaging in patients with NF-1 and illustrates that these benign lesions may become symptomatic and require neurosurgical intervention.

Multicentric pleomorphic xanthoastrocytoma in a patient with neurofibromatosis Type 1

2005 ◽  
Vol 102 (2) ◽  
pp. 376-381 ◽  
Author(s):  
Stephan Saikali ◽  
Anne Le Strat ◽  
Anne Heckly ◽  
Nathalie Stock ◽  
Jean-Marie Scarabin ◽  
...  
Keyword(s):  
Malignant Transformation ◽  
Neurofibromatosis Type 1 ◽  
Unusual Case ◽  
Neurofibromatosis Type ◽  
Pleomorphic Xanthoastrocytoma ◽  
Precursor Cells ◽  
Cerebellar Tumor ◽  
Content Type ◽  
Fine Print

✓ The authors report an unusual case of multicentric pleomorphic xanthoastrocytoma (PXA) in a 36-year-old woman with neurofibromatosis Type 1 (NF1). Both lesions were diagnosed as PXA but demonstrated different neuroimaging features and very different outcomes. Although the occipital lesion was cured surgically, the cerebellar tumor recurred three times and underwent malignant transformation into an anaplastic oligodendroglioma. The authors discuss the causes of PXA and suggest that it could originate from common bipotential precursor cells with two phenotypes.

Ossifying fibroma of the skull in a patient with neurofibromatosis type 1

1996 ◽  
Vol 85 (5) ◽  
pp. 941-944 ◽  
Author(s):  
Martino Ruggieri ◽  
Vito Pavone ◽  
Alessandra Tiné ◽  
Agata Polizzi ◽  
Gaetano Magro ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Ossifying Fibroma ◽  
Skeletal System ◽  
Content Type ◽  
Primary Bone Tumor ◽  
Primary Bone ◽  
Bone Abnormalities ◽  
Fine Print

✓ Ossifying fibroma is a rare, benign, primary bone tumor that occurs most commonly in the mandible; a cranial vault location is extremely rare. In this report a case of symptomatic frontoparietotemporal ossifying fibroma with intracranial growth and cerebral displacement in a 12-year-old boy with neurofibromatosis type 1 (NF1) is described. Once excised the lesion did not recur. The skeletal system is frequently affected in NF1, and bone abnormalities are present in 50% to 70% of patients with this condition. The etiology of such lesions in NF1 is still controversal. To the authors' knowledge, ossifying fibromas of calvarial bones have not been described in NF1.

Psychological disturbance and sleep disorders in children with neurofibromatosis type 1

2005 ◽  
Vol 47 (4) ◽  
pp. 237-242 ◽  
Author(s):  
Hilary Johnson ◽  
Luci Wiggs ◽  
Gregory Stores ◽  
Susan M Huson
Keyword(s):  
Sleep Disorders ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Psychological Disturbance
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