Congenital Glioblastoma: Lessons Learned from a Rare Case with Unusual Presentation.

Congenital glioblastomas are uncommon tumors of infancy with unique molecular features and usually better prognosis compared to their pediatric and adult counterparts. A 15-day old newborn harboring a congenital glioblastoma undiagnosed at pregnancy presented to our emergencies with rapid neurological deterioration. Radiological investigations revealed an 8 by 9 cm space occupying and enhancing lesion in the left temporoparietooccipital region with intratumoral hemorrhage. The patient was immediately transferred to the operative theater and was operated in a poor condition. Unfortunately he showed no improvement post-operatively and died within two days in the intensive care unit. As such presentation is unique; we present the case and review the relevant literature concerning the potential pitfalls in diagnosis, management strategies and improvements in outcome.

Simultaneous Occurrence of a Pituitary Adenoma and a Foramen Magnum Meningioma: Case Report.

The coexistence of a pituitary adenoma and a meningioma is a rare event. In all previously reported cases, only 1 of these 2 tumors was symptomatic. We present the case of 61-year-old woman with no significant medical history who was treated for a simultaneously symptomatic pituitary nonfunctioning adenoma and foramen magnum meningioma. Such an association has not been reported previously.

Epidural Venous Angioma Presenting with Spinal Cord Compression in a 42-Year-Old Woman with Previous History of Ovarian Malignancy.

BACKGROUND: Venous angioma is an extremely rare vascular malformation of the epidural space. To the best of our knowledge, only 5 cases have been documented to date and none has been reported in the setting of a previous malignancy. CASE DESCRIPTION: We report the case of a 42-year-old woman with a previous history of ovarian cancer, treated by surgery plus chemotherapy; who presented with signs of spinal cord compression for 3 weeks. Magnetic resonance imaging showed an intensely enhancing epidural mass at the T2-T6 level causing major spinal cord compression, for which urgent surgery was indicated. During surgery, the tumor was extremely hemorrhagic and the hemostasis was hazardous. Blood loss was estimated at 1.5 L, causing hemodynamic instability and requiring intensive resuscitation with fluids and blood transfusions. Gross total resection was achieved and the pathologic examination confirmed the diagnosis of venous angioma. The patient recovered quickly postoperatively and was able to walk independently within 2 weeks of starting intensive rehabilitation. She was symptom free with no clinical or radiologic evidence of recurrence at 1 year follow-up. CONCLUSIONS: Venous angioma should be included in the differential diagnosis of spinal epidural masses even in case of previous malignancy. Subtle imaging features should alert clinicians to this rare yet potentially life-threatening condition. Surgery remains the cornerstone of the treatment and can result in remarkable recovery.

Plagiocephaly due to Frontosphenoidal Suture Synostosis: Report of 2 Cases and Literature Review.

BACKGROUND/AIMS: The most common cause of anterior plagiocephaly is the fusion of the unilateral coronal suture. In some rare cases, however, the fusion of the frontosphenoidal suture may lead to a resembling pattern with specific clinical and radiological features. The aim of the present study is to further enlighten this entity as it is misdiagnosed most of the time. METHODS: A report of 2 cases of plagiocephaly due to frontosphenoidal synostosis with a review of the literature. RESULTS: One female and one male baby were identified. The mean age at presentation was 11.5 months (range: 1-22). Head circumference was normal in each patient. The two fusions were on the left side. Unilateral frontal flattening and recession of the supraorbital rim were the most important physical findings. Computed tomography confirmed the patency of the frontoparietal suture and the closure of the frontosphenoidal suture. Fronto-orbital advancement was performed in both cases with good cosmetic outcome. CONCLUSION: Frontosphenoidal synostosis should be suspected and carefully searched when dealing with plagiocephaly with patent coronal suture. Good outcome requires a prompt diagnosis and early correction.

Intraventricular Glioblastomas.

BACKGROUND AND IMPORTANCE: Although glioblastoma is the most common primary brain tumor, primary intraventricular locations are extremely rare; only 21 cases have been reported to date. METHODS: A retrospectively acquired database of all intracranial glioblastomas treated in 2 different neurosurgical departments during the last 10 years was queried. Patients with histologically proven intraventricular glioblastomas were included in the study. RESULTS: Eight patients were identified as having a histologically confirmed intraventricular glioblastoma. Patient age at diagnosis ranged from 6 to 74 years (mean 29.6 years) and the male/female ratio was 5:3. Increased intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. The tumor was located within the lateral ventricle in 6 cases and the anterior third ventricle in 2 others. Gross total tumor excision was achieved in 3 patients, whereas the surgical resection was subtotal in 4 cases and a surgical biopsy was performed in 1 patient. Postoperative adjuvant therapies were administered in 5 patients. Median survival time was 32.1 months, and 3 patients were alive at the end of study. All of them had isocitrate dehydrogenase-mutated tumors. CONCLUSIONS: Intraventricular glioblastoma is extremely rare and can affect younger individuals including children. This malignant tumor should be included in the differential diagnosis of intraventricular lesions, especially in the lateral ventricles. Radical surgical resection can be associated with remarkable disease-free survival, especially in isocitrate dehydrogenase-mutated tumors. Because recurrence virtually is unavoidable, long-term follow-up is mandatory.

Metastatic sacrococcygeal yolk sac tumor: A misleading diagnosis.

Sacrococcygeal yolk sac tumor (YST) is an extremely rare malignant extra-gonadal germ-cell tumor, which usually succeeds to the degeneration of more common benign teratoma. We describe here an unprecedented case of conus medullaris compression by a spinal metastasis from a pure sacrococcygeal YST in a 1½ years old girl, which was misdiagnosed initially as an anal fissure and stress the need of a meticulous clinical examination and further screening in young patients presenting with sphincter disturbances.

Primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle: a case report.

Central nervous system germ cell tumors (GCTs) account for less than 5% of primary brain tumors in children and adolescents but continue to attract much attention. To the best of our knowledge, a primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle has never been previously reported. A 15-year-old boy presented with signs of increased intracranial pressure for the past 2 weeks complicated by 2 episodes of generalized tonic-clonic seizures 1 day before admission. Neurological examination was normal, and funduscopic examination disclosed a grade II papilledema bilaterally. CT and MRI revealed a well-demarcated and enhancing mass within the anterior third ventricle associated with a left lateral ventricle hydrocephalus. There was no evidence of tumor within the pineal or suprasellar region, and systemic and cerebrospinal fluid evaluation demonstrated normal levels of α-fetoprotein and human chorionic gonadotropin. Radical surgery was advised, and total tumor resection was achieved via a transcallosal transforaminal approach. The postoperative course was uneventful, and the final histological diagnosis was a pure embryonal carcinoma. Further screening showed no other location, and adjunctive high-dose chemotherapy was administered. The patient has been symptom free with no clinical or radiological sign of progression at the most recent follow-up examination 2 years after surgery. Primary pure and nonsecreting embryonal carcinoma can develop within the anterior third ventricle and should be considered in the differential diagnosis of anterior third ventricular masses especially in young patients. Accurate identification, radical surgery and high-dose chemotherapy can result in better tumor control and improve the postoperative outcome.

Uncommon progression of an extradural spinal meningioma.

Extradural spinal meningiomas are rare. Our understanding of purely extradural spinal meningiomas is still incomplete and they may be easily confused with malignant neoplasms, much more common in this location. We report a rare case of a purely extradural thoracic spine meningioma in a 70-year-old man, with an unusual progression. In addition we discuss the pathogenesis of these tumors and the potential pitfalls in differential diagnosis and review the relevant literature concerning their treatment and outcome.

Dural metastasis of Ewing's sarcoma.

BACKGROUND: Metastatic Ewing's sarcoma to the central nervous system is an uncommon condition and debate concerning the true origin of its metastases is still up to date. To the best of our knowledge, only two cases of dural metastatic Ewing's sarcoma have been published in the English medical literature. We present an additional case in a 24-year-old female and discuss the pathogenesis of these unusual tumors with review of the relevant literature concerning their treatment and outcome. CASE DESCRIPTION: A 24-year-old female with previous history of pelvis Ewing's sarcoma and recently discovered lung metastases, presented with moderate headache for the past 2 weeks and weakness in her left leg for the past 2 days. Computed tomography scan and magnetic resonance imaging revealed an extra-axial right frontoparietal mass invading the superior sagittal sinus but with clear delineation with brain parenchyma. Imaging features were suggestive of a meningioma as no abnormalities in the skull abutting to the tumor were noted. The patient underwent surgical removal of her tumor. Near total resection was achieved and histological examination showed evidence of metastatic Ewing's sarcoma. Postoperative adjuvant radiation and chemotherapy were administered. The patient improved well postoperatively with full recovery of her motor weakness. She is symptom free with no signs of progression, at most recent follow-up, 8 months after surgery. CONCLUSION: Despite its rarity, metastatic Ewing's sarcoma must be considered in the differential diagnosis of extra-axial dural masses particularly meningiomas.

Epilepsy surgery program in Tunisia: an example of a Tunisian French collaboration.

OBJECTIVE: To describe the first Tunisian epilepsy surgery program establishment and to emphasize on its originality that is an exchange and surgery decision taken by two Mediterranean neurophysiological teams, via the Internet. METHODS: Patients with mesial temporal lobe epilepsy (MTLE) and refractory to antiepileptic drugs were included. A noninvasive protocol evaluation including a detailed history, neurological evaluation, brain imaging, scalp video-EEG monitoring and neuropsychological evaluation were performed. The different findings were discussed between the Neurophysiological Department of Charles Nicolle Hospital of Tunis and Rouen through the EUMEDCONNECT Internet network project. If cases of concordance of clinical, neuropsychological, neuroimaging data and video-EEG recordings, surgery was indicated. RESULTS: 15 patients (7 women and 8 men) with mean age of 30 years were included. 10 patients had right hippocampal sclerosis (HS) and 5 had left HS. MRI findings were concordant with the ictal EEG in 12 patients. One patient had bitemporal ictal EEG abnormalities and right HS on MRI. One patient had contralateral ictal clinical and EEG patterns to the side of HS. One patient had temporal 'plus' epilepsy. Surgery was performed in 10 cases. After surgery, all patients are seizure free, with no operative mortality or major surgery complications. CONCLUSION: Our model of twin affiliations between advanced epilepsy surgery programs in a developed country and starting programs in a developing country, using Internet technology, can be a model for collaboration in other countries.

Epilepsy surgery program in Tunisia, the Charles Nicolle Hospital experience.

BACKGROUND: Epilepsy surgery is the best treatment in case of intractable epilepsy. However, it is underused in developing countries, creating an enormous treatment gap. On 2006, an epilepsy surgery program was set up in the Neurological Department of Charles Nicolle Hospital, Tunis, Tunisia. AIM: To describe its establishment and to emphasize on its originality that is an exchange and surgery decision taken by two Neurological teams via the internet. METHODS: Patients underwent a phase 1 presurgical evaluation at the Neurological Department of Charles Nicolle Hospital of Tunis. It included a detailed clinical history, a neurological evaluation, brain MRI, a continuous video EEG scalp monitoring and a neuropsychological evaluation. RESULTS: According to the different electro clinical and radiological findings, the surgery indication was taken between the Neurological department of Charles Nicolle Hospital of Tunis and the Neurophysiological Department of Charles Nicolle Hospital in Rouen through the EUMEDCONNECT network project. ATL was performed and the four patients are actually seizure free. CONCLUSION: Epilepsy surgery is actually a possible treatment option in Tunisia and should be indicated in patients with intractable partial seizures.