1/50. Trigeminal ganglioneuroma.We present the case of an 8-year-old girl with a ganglioneuroma in the left cerebellopontine angle region. The tumor originated from the sensory root of the trigeminal nerve. Histopathologically, it was composed of neoplastic ganglion cells and schwann cells, leading us to the diagnosis of ganglioneuroma. Intracranial ganglioneuroma is very rare. To our knowledge, this is the first report of a trigeminal ganglioneuroma. The nature and origin of this tumor are discussed and the literature reviewed.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/50. Mild gait abnormality and leg discomfort in a child secondary to extradural ganglioneuroma.Ganglioneuromas are benign and slow-growing tumors that most commonly originate from the sympathetic trunk. Ganglioneuromas often decrease in size and rarely require reoperation. Changes in gait or the onset of limb pain without a discernible local cause are indications for investigation of patients for possible intraspinal pathology. We report the case of a 5-year-old boy who presented with seemingly static symptoms, while the slow-growing tumor had enveloped nerve roots and caused bone destruction of the vertebrae.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
3/50. March 2000: A 16 year old female with a cerebellar mass.The March COM: A 16 year old female presented with headaches and cerebellar dysfunction. MR images showed a mass lesion of the right cerebellar hemisphere with mass effect on the medulla. The mass exhibited a striated pattern of alternating isointense and hypointense zones on T1-weighted images that did not contrast enhance. The lesion was hyperintense on T2-weighted images, and also showed a striated appearance. A suboccipital craniotomy and resection of the lesion was performed. Microscopically, the specimen consisted of widened folia and a disorganized cerebellar architectonic pattern in which the internal granular cell layer was occupied by a population of large dysmorphic nerve cell bodies. Patient's diagnosed with Lhermitte-Duclos disease must be adequately evaluated for Cowden's syndrome.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/50. Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum).OBJECTIVE: Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a rare hamartomatous lesion of the cerebellar cortex. The pathogenesis of the disease is still poorly understood. Lhermitte-Duclos disease was recently considered to be part of a multiple hamartoma-neoplasia syndrome (Cowden disease). We add two further cases to this rare entity. patients: A 24-year old woman presented with occipital headaches, blurred vision, diplopia and ataxia of gait. physical examination revealed turricephaly. The second patient was a 37-year old woman, who presented with progressive occipital headache with nausea and vomiting. physical examination revealed congenital facial asymmetry. Computed tomography and NMR-imaging, respectively demonstrated a space occupying mass of a cerebellar hemisphere in both cases. RESULTS: Suboccipital craniotomy and complete removal of the infratentorial tumour were performed in both patients. Histopathological findings clinched the diagnosis of Lhermitte-Duclos disease. Postoperative course was uneventful in the first and complicated by progressive occlusive hydrocephalus in the second patient, necessitating permanent surgical shunt drainage. Both patients were discharged free of complaints. CONCLUSIONS: Dysplastic cerebellar gangliocytoma is commonly associated with progressive mass effects in the posterior fossa and typically presents with headaches, cerebellar dysfunction, occlusive hydrocephalus and cranial nerve palsies. The disease usually manifests in young adults, but the age at presentation ranges from birth to the sixth decade. There is no sex predilection. NMR-imaging became a useful clue to the diagnosis within the last decade. Therapy consists of decompression of the posterior fossa by total surgical removal of the tumour mass.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
5/50. multiple endocrine neoplasia type two B. A case report with light and electron microscopic and immunohistochemical correlation.PURPOSE: To describe a case of multiple endocrine neoplasia type two B (men IIB) where ocular and systemic clinicopathological findings are correlated, in association with light and electron microscopic and immunohistochemical findings. methods: A 23-year-old man presented with mucosal neuromas of the lips, tongue and eyelids, a Marfanoid habitus and prominent corneal nerves. These findings led to the diagnosis of multiple endocrine neoplasia type two B. The patient subsequently developed phaeochromocytoma and metastatic medullary thyroid carcinoma (MTC) which led to his demise. Correlation of light and electron microscopic and immunohistochemical findings with the systemic and ocular findings is presented to emphasise the aggressiveness of MTC in men IIB. Clinicopathological correlation was obtained by examining the eyes post mortem. RESULTS AND CONCLUSIONS: Three new findings in men IIB have been established by this study. The enlarged corneal nerves can now be regarded as ganglioneuromas. Medullary thyroid carcinoma metastases were found in the choroid. light and electron microscopic examination of the eye showed ganglioneuromas of the nerves in the limbus, trabecular meshwork, uveal tract and posterior ciliary nerves; this finding may account for the glaucoma occasionally seen in patients with men IIB.- - - - - - - - - - ranking = 4keywords = nerve (Clic here for more details about this article) |
6/50. Atypical ganglion cell tumor of the sciatic nerve.CONTEXT: Although herniation of a lumbosacral intervertebral disk is a major cause of sciatic distribution pain, relentlessly progressive symptoms or signs should alert one to the possibility of a tumor involving the nerve. OBJECTIVE: To describe the clinical, neurophysiological, and histological features of a pathologically unique tumor involving the sciatic nerve. SETTING: Tertiary referral university hospital. PATIENT: A 36-year-old woman was seen with a 6-year history of increasingly severe symptoms in the distribution of the left sciatic nerve. RESULTS: electromyography indicated a sciatic nerve lesion in the region of the greater sciatic notch. magnetic resonance imaging demonstrated a tumor involving the left sciatic nerve in this area. light microscopy, electron microscopy, and immunohistochemistry results confirmed the presence of an atypical ganglion cell tumor of the sciatic nerve that exhibited prognostically conflicting clinical and histological features. CONCLUSIONS: To our knowledge, this is the first report of an atypical ganglion cell tumor affecting the sciatic nerve, and illustrates the value of detailed neurophysiological examination in localizing the site of peripheral nerve injury to facilitate focused neuroimaging when standard investigations are uninformative. Longer follow-up is required to determine the true biologic potential of this lesion.- - - - - - - - - - ranking = 36.832945223105keywords = peripheral nerve, nerve, peripheral (Clic here for more details about this article) |
7/50. January 2002: 59-year-old woman with an intrasellar lesion.An intrasellar lesion resembling a hormone-inactive pituitary adenoma was resected in a 59-year-old woman. The well-differentiated benign tumor was composed of ganglion-like cells, clusters of adenohypophyseal tissue as well as a GH-expressing adenoma. In addition, small cells exhibited an intermediate neuronal and epithelial immunoreactivity. Surrounding the tumor was a spindle cell component that histologically resembled Antoni A areas of a schwannoma, but showed a mixed immunohistochemical profile. Final diagnosis was intrasellar glioneuronal hamartoma with GH-cell pituitary adenoma.- - - - - - - - - - ranking = 1.3044859756354keywords = schwannoma (Clic here for more details about this article) |
8/50. Contrast enhancement in Lhermitte-Duclos disease of the cerebellum: correlation of imaging with neuropathology in two cases.Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma, is a rare cerebellar lesion. It has long been regarded as avascular. We report two patients with surgically proven LDD in whom contrast enhancement was observed on MRI. Neuropathological examination revealed proliferation of veins. We suggest that peripheral enhancement of LDD probably reflects vascular proliferation of the cerebellar venous draining system, and should be considered part of the imaging features of LDD.- - - - - - - - - - ranking = 0.43674519285833keywords = peripheral (Clic here for more details about this article) |
9/50. Resection of presacral ganglioneurofibroma by laparoscopy.BACKGROUND: Tumors of the retrorectal space are rare. They comprise a heterogeneous group of benign or malignant tumors that cause similar symptoms due to their location in presacral space. If possible, complete surgery excision is the therapy of choice mainly through a sacral, abdominal-sacral, or a pure abdominal or perineal axis. CASE REPORT: A 15-year-old asymptomatic patient was diagnosed with a retrouterine tumor during her first gynecological examination. magnetic resonance imaging of the pelvis showed a 10 x 8.5 x 7-cm encapsulated presacral tumor. Retrorectal ganglioneurofibroma was removed by laparoscopy with preservation of the sympathetic and parasympathetic nerves and the sacral roots of the ischiadic nerves. No sensory or motoric dysfunction of the legs, bladder, or rectum was observed postoperatively. CONCLUSION: This is the first case report of a complete endoscopic removal of large presacral ganglioneurofibroma in an asymptomatic woman.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
10/50. Posterior fossa gangliocytoma with facial nerve invasion: case report.A 5 year-old boy with a cerebellar gangliocytoma with a peripheral right facial paresis and ataxia is presented. His MRI showed a heterogenous, diffuse lesion, isointense on T1 and hyperintense on T2-weigthed sequences, involving the right cerebellar hemisphere with direct extension into the right facial nerve. The present case is the first description of a gangliocytoma with direct facial nerve invasion, as demonstrated for the facial nerve paresis and supported by MRI and surgical inspection.- - - - - - - - - - ranking = 7.4367451928583keywords = nerve, peripheral (Clic here for more details about this article) |
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