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Central nervous system medulloepithelioma is a rare, highly malignant childhood tumor. It might be confused with medulloblastoma or other primitive neuroectodermal tumors, but it is quite particular by its clinical, radiological, and pathological features. The mean survival varies depending on whether or not a gross-total resection is possible. Adjuvant radiochemotherapy is often indicated. Only two reported cases in the literature survived beyond 4 years after treatment by gross total resection and radiotherapy without chemotherapy. We report three cases of supratentorial medulloepithelioma occurring in three children aged 11-17 years. Two patients underwent a gross-total resection followed by radiotherapy and survived more than 4 years after treatment. The third case had, however, recurred twice within the 1st postoperative month despite a complete resection each time and metastasis to the lung developed. Chemotherapy was then carried out after the third procedure and the patient died 7 months later., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Central neurocytoma is a rare benign neoplasm of the central nervous system. The intraventricular location close to the Monro foramina and the attachment to the septum pellucidum are characteristic for the diagnosis. However, atypical appearances may be encountered and confused with other neoplasms. The authors report the radiological findings of 32 surgically treated and pathologically confirmed neurocytomas., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Background and Purpose: Intramedullary spinal cord tumors (IMSCT) are relatively rare neoplasms, accounting for less than 5% of all central nervous system tumors. The optimum management of these tumors still remains controversial. Many decades ago, partial surgical resection followed by radiotherapy was the conventional management for IMSCT. Nowadays, maximal surgical resection of IMSCT without adjuvant therapy is the rule. We discuss the management of our cohort of 45 patients and review retrospectively the surgical outcome and survival., Methods and Results: We reviewed the charts of 45 patients who underwent surgery for IMSCT in our institution since 1990. The study included 23 female and 22 male with a mean age of 28.7 years (range: 18 months-64 years). In 40 patients, the final diagnosis was based on the results of MR imaging. The cervical location of the tumor was the most common (20 cases). Surgical procedures included a gross-total resection in 31 cases, subtotal resection in six cases, partial resection in five cases and a biopsy in three cases. The large majority of patients had histologically-proven low-grade tumors composed essentially of astrocytomas (44,4%) and ependymomas (28,8%). There was no mortality related to surgery. Concerning the functional outcome at six months, we noted that 22.2% of our patients deteriorated, 47.3% stayed the same and 30.5% improved. We found that patients with mild or no preoperative deficits were exceptionally damaged by the surgical procedure., Conclusions: The gold-standard treatment of IMSCT remains maximal microsurgical resection without adjuvant therapy. For malignant or rapidly recurrent IMSCT, the optimum management is still controversial. Determinant predictors for a good outcome after surgery of IMSCT are histological type of lesion, total removal of the tumor and a satisfactory neurological status before surgery.

Non tumoral stenosis of the foramen of Monro is rare; pathogenic mechanisms remain a subject of debate. The narrowing can be unilateral causing monoventicular hydrocephalus, and exceptionally bilateral causing biventricular hydrocephalus. We present two cases of monoventricular hydrocephalus and two other cases of biventricular hydrocephalus. Clinically, all patients had the same signs as in common hydrocephalus. The CT scan and mainly the MRI allowed us to confirm the diagnosis and to avoid tumor obstruction of the foramen of Monro. With neuroendoscopy we were able to describe the foramen of Monro and perform a fenestration of the septum pellucidum. The prognosis is usually good.

Most hemangiopericytomas (HPC) are located in the musculoskeletal system and the skin, while the location in the central nervous system (CNS) is rare. The latter represents 2 to 4% in large series of meningeal tumors, thus accounting for less than 1% of all CNS tumors. In the central nervous system, tumors with a hemangiopericytomatous histolopathological pattern can be either hemangiopericytomas or solitary fibrous tumors. CNS-HPCs have a relentless tendency for local recurrence and metastases outside the CNS. Metastasis can also appear many years after adequate treatment of the primary tumor. We present a pathological study of eight patients with CNS-HPC and compare our results with corresponding published data. The CNS-HPC group consisted of three males and five females with a mean age of 36.75 years. The tumors were supratentorial in four cases, infratentorial in two cases, tentorial in one case and located in the spinal cord in the last one. Histologically, CNS-HPCs were similar to their soft tissue counterparts. One case demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism with infiltration of the cerebellum. All patients underwent surgery with gross-total resection in all cases. No patients received postoperative radiation therapy. Only four patients recurred locally after six, seven and eight months, and five years. Our study presents the pathological features of CNS-HPC as a distinct entity from both meningioma and solitary fibrous tumors. A comparative review of literature with our results is discussed.

Hydatid cyst is rarely observed in the brain (0.5-4.5%). The frequency of calcified cyst is less than 1%. We present a case of a 15-year-old girl with a 5-year follow-up for grand mal seizures that became resistant to three-drug therapy. The CT scan revealed a calcified parieto-occipital lesion. MRI disclosed a suggestive detached membrane. At surgery, the cyst wall was calcified with typical hydatid sand contents. Since surgery, seizure control has been achieved with one drug. Calcification of a cerebral hydatid cyst is exceptional. MRI enabled the diagnosis in this patient.

We report a case of an unusual epidermoid cyst (EC) of the cerebellopontine angle that appeared hyperdense on computed tomography (CT) scanning, hyperintense on T1-weighted MR images and hypointense on T2-weighted magnetic resonance (MR) images. Diffusion-weighted imaging showed a hypointense lesion. We discuss imaging characteristics of ECs, explain the atypical findings in our case and confirm that the signal seen on diffusion-weighted images in the EC is related to a T2 effect.

Extraneural diffusion of glioblastoma is very unusual. Extracranial metastases generally occur after craniotomy or a long survival time. Metastases usually involve the lungs, pleura, lymph nodes and occasionally the bone. We report herein a case of bone dissemination occurring 18 month after the initial diagnosis and treatment of supratentorial glioblastoma.

A series of 25 patients with a clinical diagnosis of pituitary apoplexy (PA) is reviewed. It included 14 men and 11 women aged between 20 to 79 years (mean age: 54 years). Twenty-two patients did not know that they had a pituitary tumor when the apoplexy occurred. A precipitating event was found in 3 cases. Symptoms and signs ranged from isolated ocular paresis to a deep coma. Seventeen patients experienced a decrease in their visual acuity. CTscan and MRI showed a pituitary adenoma in all cases, a hemorrhage was also present in 10 out of the 24 CTscans, and in all the 8 MRI performed. Twenty patients underwent surgery; 18 of them by a transsphenoidal approach. A complete recovery of visual acuity was observed in 75% of patients operated within the week following the onset of symptoms, and in 56% of patients operated later on. There was no case of complete visual recovery among the blind patients. Pituitary apoplexy is a clinical concept. It applies only to symptomatic cases. It is generally a complication of a pituitary adenoma which is in most cases unknown. There are different degrees of severity; PA can even be life-threatening. The principal aim of surgery in the acute phase is the improvement of visual prognosis. In our series, blind patients or those with a history of visual loss for more than a week or with a blindness had a poorer prognosis.

Primary central nervous system hemangiopericytoma is rare, accounting for less than 1% of primary central nervous system tumors. Diagnosis is histological. Treatment is surgical excision, followed by radiotherapy. Long-term follow-up is mandatory for these tumors with a high potential for recurrence and metastasis. The sellar location is very rare, and can be confused with pituitary adenoma. We report the case of a patient presenting a sellar hemangiopericytoma, who underwent surgery via a transsphenoidal approach, then right frontal craniotomy followed by radiotherapy. There was no recurrence at one year follow-up.

Study Design: First published report of a cranial aneurysmal bone cyst (ABC) treated successfully with intralesional injection of calcitonin., Objectives: To describe a safe and effective treatment method for ABCs of the cranial base., Summary of Background Data: ABC is a rare form of dystrophic pseudotumor. Less than 100 cases involving the skull have been reported in the literature, most of them localised in the cranial vault. Cranial base locations are rare and difficult to treat. We selected this treatment after a very rapid recurrence of the lesion following a partial resection. Method. - After a partial resection of a 10 cm petro-occipital ABC that encased the vertebral artery and the lower cranial nerves, an Ommaya reservoir was implanted with a catheter tip inside the ABC. Repeated intralesional injections of calcitonin were performed through the reservoir., Results: Shrinkage of the cyst occurred with disappearance of its heterogeneous cystic content and ossification of its walls. There was no complication and the lesion remains quiescent at a 3 year follow-up., Conclusion: We reviewed the pertinent literature concerning percutaneous treatment of ABC. The percutaneous intralesionnal injection of calcitonin was reported in the literature only in 3 publications reporting 9 cases that did not involve the skull. This treatment seems safe and effective, worthy in cranial base ABCs that are difficult to resect completely.

We report a case of solitary plasmacytoma of the cranial vault. A 58-year-old man presented with a frontal soft tissue mass. X-Ray of the skull showed a lytic lesion of the frontal bone. CT scan showed the lesion extending intra and extracranially and cerebral angiography allowed embolization of afferent arteries. Complete removal of the lesion was performed without additional radiotherapy. Two years after surgery the patient is alive, and asymptomatic. Until 1997, 35 cases of solitary plasmacytoma of the cranial vault are reported, of which only five had frontal localisation. Solitary plasmacytoma of the cranial vault has a good outcome but progression towards a multiple myeloma is possible and deserves clinical and biological follow-up.

Choroid plexus cyst is generally small and a relatively common finding at autopsy. Huge and symptomatic cysts are rare. Few cases are reported in the literature. We report one case of symptomatic choroid plexus cyst of the right lateral ventricle in a six month baby who presented with epilepsy. Cerebral CT scan and MRI showed a large cyst in the right lateral ventricle compressing the adjacent structures. Total removal of the cyst has been performed by a parieto-temporal approach. The course was uneventful.

The authors report a case of aspergillus granuloma of the brain, in a 28 year old woman, simulating a meningioma. Preoperative diagnosis of aspergilloma is difficult. However, it may be suspected in a patient who has associated pulmonary and paranasal sinus fungal infection. Peroperatively it may be confused with a brain tumour. The lesions usually are in the frontal lobes. Diagnosis can be made only by surgical biopsy with identification of fungal elements. Granuloma induce a good host response, and a high capacity to elaborate antibodies.

The clinical, follow up and diagnostic features of a case of chemodectoma (paraganglioma) of the orbit are reviewed. 30 cases are reported in the literature. Chemodectoma may arise in any area of the body where non chromaffin paraganglion structures are situated. These are localized in the orbit of the chimpanzee but not in the orbit of man. Malignant behavior of chemodectoma has been a controversial subject, with most authors considering them to be benign tumors. Some authors have diagnosed locally recurrent and infiltrative tumors as malignant. The authors report one case of chemodectoma of the orbit. A 5 year old child has had right exophthalmos. She was treated by tumorectomy. The follow-up is marked by recurrence of the tumor and infiltration of the right hemi-face.