Sylvian Fissure Meningioma in Childhood: Report of 3 Cases and Review of the Literature.

OBJECTIVE: Sylvian fissure-based meningioma is a rare occurrence during childhood. We report a small series including 3 such cases, all attached to the arachnoid leaflets of the Sylvian fissure. We present a review of the literature and discuss the impact of possibly effective clinical, imaging, and surgical variables on the outcome. METHODS: In a retrospective chart review of children with intracranial meningioma, we encountered 3 cases with Sylvian fissure meningioma operated within the past 32 years. RESULTS: Two girls, 5 and 7 years old, and 1 boy, 7 years of age, presented with epilepsy, headache, diplopia, and hemiparesis. The duration of illness was 3, 4, and 2 months, respectively. All three tumors were attached to the arachnoid layer of the adjacent Sylvian fissure, and they were not dural based. Gross total resection could be achieved, and no recurrences were encountered after 13, 7, and 2 years. CONCLUSION: We report 3 very rare cases of Sylvian arachnoid-based meningiomas. To our knowledge, this is the study with the longest follow-up period (mean 7.3 years, median 7 years). Even though the overall prognosis of pediatric meningiomas may be worse than that of adult illness, we would like to emphasize that the "rare location" of the Sylvian fissure was not an important factor contributing to the outcome in our children.

Stab Wounds to the Head; Case Series, Review of Literature, and Proposed Management Algorithm.

BACKGROUND AND OBJECTIVES: Stabbing the head is a rare event. It may occur following an assault or self-inflicted injury. We intend to report our experience with four such cases and review narratively the relevant literature. A treatment algorithm will be delivered. MATERIALS AND METHODS: We have reviewed four of our cases; three were stabbed to the orbit and one to the head. RESULTS: Enucleation was performed in one case. Vision could be preserved in the other two and brain abscess developed in the other cases due to a retained piece of knife. We made a comparison between our cases and those limited reports in the literature to reach an algorithm. CONCLUSION: The temporal region and the orbit are the targets mostly attacked in the craniofacial stabs. Since knives have a sharp and thin edge, they may break when penetrating the skull. The damage to the brain tissue and the retained piece of knife may be missed easily by overlooking the small injury to the scalp. The suggested algorithm would be a contrast-enhanced computed tomography scan of the skull and brain and a kind of brain vascular study are necessary, considering the location of the knife in the skull and brain passing the area with crowded vasculature. The best treatment protocol is surgery accompanied by antibiotic therapy.

Calcified Mass on Brain CT in a Teenager with Refractory Seizures.

Cerebral echinococcosis is very rare, representing 2% of all cystic echinococcosis (CE) cases. Primary echinococcal cysts of the brain are extremely rare in pediatric patients. We report on a 16-year-old boy referred to our tertiary center with intractable epilepsy for the previous three years despite receiving full doses of three antiepileptic medications. Brain computed tomography (CT) showed a left frontal calcified mass. Magnetic resonance imaging (MRI) of the brain revealed a well-defined spherical mass in the left frontal lobe, slightly hypointense on T1-weighted and heterogeneous hyperintense on T2-weighted images with no contrast enhancement. With a broad differential list in mind, a surgical intervention was planned. During surgery, a primary calcified cerebral echinococcal cyst with severe adhesion to the adjacent dura of the frontal region was discovered and removed intact. Histopathology examination confirmed the diagnosis. Only phenobarbital was continued and no medical therapy for CE was administered. Two years after surgery, the patient remained free of seizures. In areas endemic for CE, cerebral echinococcal cyst should be included in the differential list of patients with intractable seizures. Though rare, this entity can present itself as a calcified mass on neuroimaging. Surgical removal of the calcified cyst is necessary for control and treatment of the epilepsy.

Fatal intratumoral hemorrhage in tectal plate glioblastoma multiforme following ventriculoperitoneal shunt.

Ventriculo-peritoneal (V-P) shunting is an accepted procedure before a direct approach to large tumors producing obstructive hydrocephalus. However, it has been associated with some complications. Intratumoral hemorrhage is an uncommon but fatal complication of V-P shunt insertion. The exact mechanism of this complication is unknown, but several mechanisms have been proposed including rapid lowering of intracranial pressure following V-P shunt placement or cerebrospinal fluid (CSF) drainage and sudden decrease in CSF volume and pressure after V-P shunting. We report an 8-year-old girl who presented with severe headache, double vision, and bilateral papilledema. Brain magnetic resonance imaging with and without gadolinium administration revealed a large tectal plate tumor compressing the aqueduct which caused hydrocephalus. Due to progressive decline in consciousness level, an emergency CSF diversion was planned and she underwent left side posterior parietal V-P shunt placement. Eight hours after V-P shunt insertion, she suddenly developed tonic-clonic seizures and collapsed into deep coma with dilated pupils and stopped breathing and died 1-day later. Histological examination of the autopsy specimen showed the tumor was a glioblastoma multiforme with massive intratumoral hemorrhage. The possible pathophysiological mechanisms of this fatal complication are discussed here and the pertinent literature is reviewed. It seems that endoscopic third ventriculostomy is the procedure of choice for CSF diversion in patients with tectal plate gliomas and decreases the occurrence of intratumoral hemorrhage.

Gradenigo's syndrome as first presentation of solitary osseous plasmacytoma of the petrous apex.

Plasma cell tumors of the skull base are rare in neurosurgical practice. True solitary osseous plasmacytoma of the skull base without development of multiple myeloma is extremely rare. We report a case of typical Gradenigo's syndrome, including left abducens nerve palsy, left facial pain and paresthesia in V1 and V2 distribution of trigeminal nerve caused by solitary osseous plasmacytoma of the left petrous apex. The patient was a 46-year-old man who presented with diplopia for two days. Magnetic resonance imaging (MRI) of the brain showed a hyperintense mass on T1-weighted images and slightly hypointense mass on T2-weighted images in the left petrous apex and left parasellar area. Through a left subtemporal middle fossa approach, subtotal resection of the lesion was performed. Histopathological examination of the lesion revealed plasmacytoma. The patient received 54 Gy radiation for the local tumor. Four months after radiation, the abducens palsy improved. Four years after treatment, the patient remained well with no symptoms or signs of local recurrence or progression to multiple myeloma.

Brown tumor of the spine in patients with primary hyperparathyroidism.

STUDY DESIGN: Retrospective case series and literature review. OBJECTIVE: To describe our experience in diagnosis and management of patients with spine brown tumor (osteitis fibrosa cystica) as the initial manifestation of primary hyperparathyroidism and also to review the pertinent literature. SUMMARY OF BACKGROUND DATA: The spine can be involved through reparative processes such as giant cell reparative granuloma and brown tumor, which lead to formation of lesions that can simulate tumors on neuroimaging. Brown tumor, an uncommon focal giant cell lesion, is a nonneoplastic and reactive process due to bone resorption and localized osseous lesion caused by primary or secondary hyperparathyroidism. METHODS: Among the cases of spine giant cell lesions treated surgically by the authors (2000-2013), there were 4 cases of spine brown tumor in patients with primary hyperparathyroidism. Clinical, radiological, histopathologic, and surgical data of these 4 cases were collected, and the patients were followed from 5 to 7 years after the surgical intervention. RESULTS: There were 2 male and 2 female patients with age range of 16 to 52 years. The lesions were located in cervical (1 case), thoracic (1 case), and lumbar (2 cases) spine regions. Clinical presentations included neck and low back pain, radicular pain, paraparesis, and sphincter dysfunction. Surgical removal of the spine lesions was achieved in all cases. Spine fusion and instrumentation was done in 3 cases. Parathyroidectomy was performed in all 4 cases. CONCLUSION: Spine involvement with brown tumor in patients with primary hyperparathyroidism is rare and may be the first manifestation of hyperparathyroidism. Brown tumor should be differentiated from other giant cell lesions involving the spine. Long-term surgical outcome was satisfactory with no recurrence. LEVEL OF EVIDENCE: 4.

Primary cerebral myxopapillary ependymoma presenting with intratumoral hemorrhage.

Myxopapillary ependymoma (MPE), a benign histological variant of ependymoma, is found most commonly in the cauda equina region. Primary intracranial MPE is very rare, and most cases are a metastatic deposit from a spinal lesion. Primary cerebral MPEs are usually well-defined solid or cystic lesions without hemorrhage. We report the first case of primary cerebral MPE with intratumoral hemorrhage.

Myxopapillary ependymoma of the conus medullaris presenting with intratumoral hemorrhage during weight lifting in a teenager.

Intratumoral hemorrhage within a myxopapillary ependymoma of the conus medullaris and cauda equina is rare. Most patients with myxopapillary ependymoma present insidiously, but they may present with hemorrhage or cauda equina syndrome. Limited number of case reports available has described this condition only in adult patients. We report our experience with intratumoral hemorrhage of myxopapillary ependymoma of the conus medullaris during weight lifting in a 15-year-old boy.

Acute presentation of solitary spinal epidural cavernous angioma in a child.

Solitary spinal epidural cavernous angiomas are rare lesions, especially in paediatric age group. They are infrequently considered in the differential diagnosis of spinal epidural masses in children. We report a case of solitary epidural cavernous angioma of the thoracic spine in a child presenting with acute onset of back pain and myelopathy. Magnetic resonance imaging of the thoracic spine demonstrated a posterior epidural mass at T6-T8 levels with compression of the spinal cord. Using microsurgical technique and bipolar coagulation, total excision of the lesion was achieved. Histopathological examination confirmed the diagnosis of cavernous angioma. At the five-year follow-up, there was no recurrence of the tumour.

Brown tumors of the anterior skull base as the initial manifestation of true normocalcemic primary hyperparathyroidism: report of three cases and review of the literature.

Brown tumor is a bone lesion secondary to hyperparathyroidism of various etiologies. Skeletal involvement in primary hyperparathyroidism secondary to parathyroid adenoma is very uncommon and brown tumor has become extremely a rare clinical entity. Hyperparathyroidism is usually associated with high levels of serum calcium. Brown tumor as the only and initial symptom of normocalcemic primary hyperparathyroidism is extremely rare. Moreover, involvement of the skull base and the orbit is exceedingly rare. The authors would report three cases of brown tumor of the anterior skull base that were associated with true normocalcemic primary hyperparathyroidism. Clinical manifestations, neuroimaging findings, pathological findings, diagnosis and treatment of the patients are discussed and the relevant literature is reviewed.

Craniospinal dissemination of filum myxopapillary ependymoma following spinal trauma: case report and literature review.

BACKGROUND: Myxopapillary ependymoma is a rare benign variant of ependymoma, occurring most commonly in the cauda equina/filum terminale of the spinal cord. It is rare in pediatric patients. Dissemination of the myxopapillary ependymoma along the neuraxis is uncommon both in adults and pediatric patients. METHODS: We report a 16-year-old boy with dissemination of the myxopapillary ependymoma along the neuraxis following spinal trauma. The possible mechanisms of tumor dissemination are discussed with a review of the literature.

Solitary spinal epidural cavernous angioma: report of nine surgically treated cases and review of the literature.

PURPOSE: We intend to report the largest series of spinal epidural cavernomas (SEC), discuss their clinical features, imaging characteristics, surgical findings, outcome analysis and compare them with similar reports in the literature. METHODS: Among the cases of spinal tumors treated surgically by the authors, there have been nine cases of SEC's. All the data were collected prospectively and the cases have been followed after surgery up to the time of this analysis. RESULTS: There were six female and three male patients with the ages ranging between 13 and 74 years. The lesions were located in the thoracic spine (4 cases), lumbar spine (4 cases) and one at the sacral level. Clinical presentations included acute spinal pain and paraparesis in two, low back pain and radiculopathy in five, and slowly progressive myelopathy in the other two cases. The lesion was iso-intense with the spinal cord in T1W images and hyperintense in T2W images and showed strong homogeneous enhancement after contrast medium injection in most of our cases. In the presence of hemorrhage inside the lesion, it was hyperintense in both T1W and T2W MR sequences as in our case 6. In the single case presenting with acute hemorrhage, epidural hematoma was the only finding, our case 1. Complete surgical removal was achieved in all our cases, and confirmed by postoperative MRI. CONCLUSION: SEC is hard to be differentiated from other epidural spinal lesions before intervention but should be considered in the list of differential diagnosis regarding its favorable outcome.

Discal cyst in pediatric patients: case report and review of the literature.

Discal (intraspinal extradural) cysts which communicate with intervertebral disk are uncommon cause of lumbar radiculopathy, especially in pediatric patients. Only three pediatric cases have been reported in the literature. A 15-year-old boy presented with acute onset left-sided S1 radiculopathy. Magnetic resonance imaging of the lumbar spine revealed a well-defined round extradural mass seated in the left ventrolateral epidural space of the spinal canal behind the S1 vertebral body. He did not respond to the conservative therapies. Because of persistent left S1 radiculopathy, left laminotomy was performed at L5-S1 level with coagulation of the posterior longitudinal ligament surrounding the anular tear; however, discectomy was not done. The symptoms resolved and no recurrence was found at 7-year follow-up. The clinical manifestations, imaging findings, possible pathogeneses, and treatment options of these lesions are discussed and the relevant literature is reviewed.

Intramedullary neurenteric cyst of the conus medullaris without associated spinal malformation: a case report and review of the literature.

Spinal neurenteric (NE) cysts are rare congenital anomalies that may occur either alone or in the context of a complex malformative disorder. They are usually intradural-extramedullary lesions. Intramedullary NE cysts not associated with other congenital anomalies are very rare and only a few cases have been reported in the conus medullaris region. Intramedullary neurenteric cysts not associated with other spinal anomalies are very rare especially in the conus medullaris region. MRI is useful to define the cyst and the osseous anomalies associated with this lesion. The goal of treatment of an intramedullary neurenteric cyst is total excision at the first operation, if possible. Life-long follow-up with annual MRI is recommended due to the risk of cyst recurrence. We report an intramedullary NE cyst of the conus medullaris without associated malformation and the relevant literature is briefly reviewed.

Primary spinal epidural rhabdomyosarcoma: a case report and review of the literature.

BACKGROUND: Primary intraspinal sarcomas are very rare in children. Rhabdomyosarcoma (RMS) is a highly aggressive and rapidly growing sarcoma with skeletal origin that occasionally appears in the spinal epidural space. METHOD: We report a 13-year-old girl who presented with back pain, progressive paraparesis, and urinary retention. She had muscular weakness in her lower extremities and absent deep tendon reflex. An epidural dumbbell-shape mass at T11-T12 level was observed on MRI. The patient underwent T12 hemilaminectomy, partial T11 hemilaminectomy, and right facetectomy. A large, firm, dark-red vascular epidural tumor was found compressing the cord. The tumor which extended to intracanal and foraminal parts was removed completely. DISCUSSION: Histopathological examination revealed undifferentiated small round and oval tumoral cells. Immunohistochemical staining was positive for vimentin, desmin, and myogenin. The final diagnosis was alveolar RMS. She received radiotherapy and chemotherapy by vincristine, actinomycin-D, doxorubicin, and ifosfamide. The patient was in good condition with no tumor recurrence or metastasis at the 1-year follow-up.

Isolated primary craniopharyngioma in the cerebellopontine angle.

Between January 2000 and January 2011, we diagnosed three patients with isolated craniopharyngioma in the cerebellopontine angle (CPA). Brain MRI revealed cystic lesions with various imaging characteristics, including hypointensity on T1-weighted (T1W) images and hyperintensity on T2-weighted (T2W) images. The first patient's lesion showed rim enhancement after gadolinium administration. The second patient's lesion showed mixed signal intensity on both T1W and T2W images. The third patient's MRI showed a well-defined cystic lesion in the right CPA that compressed the brainstem. This lesion was hyperintense on T1W images and hypointense relative to cerebrospinal fluid on T2W images, and was peripherally enhanced after gadolinium administration. All three patients underwent surgical intervention through a suboccipital retrosigmoid craniotomy/craniectomy and lesions that did not adhere to adjacent tissues were removed completely. Histopathological examination confirmed the tumors to be adamantinomatous craniopharyngioma. The post-operative course was uneventful for all patients uneventful and no tumor recurrences were detected at the last follow-up. Primary CPA craniopharyngioma can be completely removed surgically, provided it does not densely adhere to vital structures.

Chondromyxoid fibroma of the anterior skull base invading the orbit in a pediatric patient: case report and review of the literature.

Chondromyxoid fibroma (CMF) is a rare bone tumor of cartilaginous derivation representing less than 1% of all benign bone neoplasms. It is a slow-growing, locally destructive tumor that usually affects the metaphyseal region of long bones of patients in their second or third decade of life. Involvement of the skull base and orbit is extremely rare. We present the first case of histopathology-proven CMF originating in the greater sphenoid wing and extending into the orbit in a 14-year-old boy. He complained of pain and swelling of his left lateral orbit for 4 months and had 3-mm nonaxial proptosis. Magnetic resonance imaging of the orbit revealed a well-defined lesion in the lateral wall of the left orbit, hypointense on T1-weighted images, and heterogenous hyperintense on T2-weighted images and enhanced after contrast media injection. Through a left semicoronal skin incision, the mass was removed totally and the lateral orbital wall was reconstructed. The clinical manifestations, imaging findings, and surgical treatment of the lesion are discussed with a review of the literature.

Intracranial hemorrhage following lumbar spine surgery.

BACKGROUND: Intracranial hemorrhage is a serious but rare complication of spinal surgery, which can occur in the intracerebral, cerebellar, epidural, or subdural compartment. PURPOSE: To describe patients with intracranial hemorrhage after lumbar spinal surgery and present clinical and diagnostic imaging findings. METHODS: In this retrospective study, medical records of 1,077 patients who underwent lumbar spinal surgery in our tertiary referral neurosurgery center between January 2003 and September 2010 were studied. The original presentations of the patients before the surgical intervention were herniated lumbar disc, spinal canal stenosis, spondylolisthesis, lumbar spinal trauma, and lumbar spine and epidural tumor. The operations performed consisted of discectomy, multiple level laminectomy, stabilization and fusion, lumbar instrumentation, and lumbar spinal and epidural tumor resection. RESULTS: Four cases developed intracranial hemorrhage including acute subdural hematoma (one case), epidural hematoma (one case), and remote cerebellar hemorrhage (two cases). The clinical and diagnostic imaging characteristics along with treatments performed and outcomes of these four patients are described and the pertinent literature regarding post-lumbar spinal surgery intracranial hemorrhages is reviewed. CONCLUSION: Though rare, intracranial hemorrhage can occur following lumbar spinal surgery. This complication may be asymptomatic or manifest with intense headache at early stages any time during the first week after surgery. Dural tear, bloody CSF leakage, focal neurologic symptoms, and headache are indicators of potential intracranial hemorrhage, which should be considered during or following surgery and necessitate diagnostic imaging.